Level of endogenous formaldehyde in maple syrup as determined by spectrofluorimetry.

PubMed

Lagacé, Luc; Guay, Stéphane; Martin, Nathalie

2003-01-01

The level of endogenous formaldehyde in maple syrup was established from a large number (n = 300) of authentic maple syrup samples collected during 2000 and 2001 in the province of Quebec, Canada. The average level of formaldehyde from these authentic samples was measured at 0.18 mg/kg in 2000 and 0.28 mg/kg in 2001, which is lower than previously published. These average values can be attributed to the improved spectrofluorimetric method used for the determination. However, the formaldehyde values obtained demonstrate a relatively large distribution with maximums observed at 1.04 and 1.54 mg/kg. These values are still under the maximum tolerance level of 2.0 mg/kg paraformaldehyde pesticide residue. Extensive heat treatment of maple syrup samples greatly enhanced the formaldehyde concentration of the samples, suggesting that extensive heat degradation of the sap constituents during evaporation could be responsible for the highest formaldehyde values in maple syrup.

The effect of the tariff on the maple industry

Treesearch

Paul E. Sendak

1972-01-01

The U. S. maple tariff is a tax on maple syrup and sugar imports from Canada into the United States. An analysis of the maple tariff indicates that it was never very effective in protecting the domestic maple industry from foreign competition. The tariff has been especially ineffective since World War II. Its removal will not hurt the U. S. maple syrup industry.

Chemical compositional, biological, and safety studies of a novel maple syrup derived extract for nutraceutical applications.

PubMed

Zhang, Yan; Yuan, Tao; Li, Liya; Nahar, Pragati; Slitt, Angela; Seeram, Navindra P

2014-07-16

Maple syrup has nutraceutical potential given the macronutrients (carbohydrates, primarily sucrose), micronutrients (minerals and vitamins), and phytochemicals (primarily phenolics) found in this natural sweetener. We conducted compositional (ash, fiber, carbohydrates, minerals, amino acids, organic acids, vitamins, phytochemicals), in vitro biological, and in vivo safety (animal toxicity) studies on maple syrup extracts (MSX-1 and MSX-2) derived from two declassified maple syrup samples. Along with macronutrient and micronutrient quantification, thirty-three phytochemicals were identified (by HPLC-DAD), and nine phytochemicals, including two new compounds, were isolated and identified (by NMR) from MSX. At doses of up to 1000 mg/kg/day, MSX was well tolerated with no signs of overt toxicity in rats. MSX showed antioxidant (2,2-diphenyl-1-picrylhydrazyl (DPPH) assay) and anti-inflammatory (in RAW 264.7 macrophages) effects and inhibited glucose consumption (by HepG2 cells) in vitro. Thus, MSX should be further investigated for potential nutraceutical applications given its similarity in chemical composition to pure maple syrup.

Chemical Compositional, Biological, and Safety Studies of a Novel Maple Syrup Derived Extract for Nutraceutical Applications

PubMed Central

2015-01-01

Maple syrup has nutraceutical potential given the macronutrients (carbohydrates, primarily sucrose), micronutrients (minerals and vitamins), and phytochemicals (primarily phenolics) found in this natural sweetener. We conducted compositional (ash, fiber, carbohydrates, minerals, amino acids, organic acids, vitamins, phytochemicals), in vitro biological, and in vivo safety (animal toxicity) studies on maple syrup extracts (MSX-1 and MSX-2) derived from two declassified maple syrup samples. Along with macronutrient and micronutrient quantification, thirty-three phytochemicals were identified (by HPLC-DAD), and nine phytochemicals, including two new compounds, were isolated and identified (by NMR) from MSX. At doses of up to 1000 mg/kg/day, MSX was well tolerated with no signs of overt toxicity in rats. MSX showed antioxidant (2,2-diphenyl-1-picrylhydrazyl (DPPH) assay) and anti-inflammatory (in RAW 264.7 macrophages) effects and inhibited glucose consumption (by HepG2 cells) in vitro. Thus, MSX should be further investigated for potential nutraceutical applications given its similarity in chemical composition to pure maple syrup. PMID:24983789

Improved amino acid, bioenergetic metabolite and neurotransmitter profiles following human amnion epithelial cell transplant in intermediate maple syrup urine disease mice.

PubMed

Skvorak, Kristen J; Dorko, Kenneth; Marongiu, Fabio; Tahan, Veysel; Hansel, Marc C; Gramignoli, Roberto; Arning, Erland; Bottiglieri, Teodoro; Gibson, K Michael; Strom, Stephen C

2013-06-01

Orthotopic liver transplant (OLT) significantly improves patient outcomes in maple syrup urine disease (MSUD; OMIM: 248600), yet organ shortages point to the need for alternative therapies. Hepatocyte transplantation has shown both clinical and preclinical efficacy as an intervention for metabolic liver diseases, yet the availability of suitable livers for hepatocyte isolation is also limited. Conversely, human amnion epithelial cells (hAEC) may have utility as a hepatocyte substitute, and they share many of the characteristics of pluripotent embryonic stem cells while lacking their safety and ethical concerns. We reported that like hepatocytes, transplantation of hAEC significantly improved survival and lifespan, normalized body weight, and significantly improved branched-chain amino acid (BCAA) levels in sera and brain in a transgenic murine model of intermediate maple syrup urine disease (imsud). In the current report, we detail the neural and peripheral metabolic improvements associated with hAEC transplant in imsud mice, including amino acids associated with bioenergetics, the urea cycle, as well as the neurotransmitter systems for serotonin, dopamine, and gamma-aminobutyric acid (GABA). This stem cell therapy results in significant global correction of the metabolic profile that characterizes the disease, both in the periphery and the central nervous system, the target organ for toxicity in iMSUD. The significant correction of the disease phenotype, coupled with the theoretical benefits of hAEC, particularly their lack of immunogenicity and tumorigenicity, suggests that human amnion epithelial cells deserve serious consideration for clinical application to treat metabolic liver diseases. Copyright © 2013. Published by Elsevier Inc.

Managing for delicious ecosystem service under climate change: can United States sugar maple (Acer saccharum) syrup production be maintained in a warming climate?

Treesearch

Stephen N. Matthews; Louis R. Iverson

2017-01-01

Sugar maple (Acer saccharum) is a highly valued tree in United States (US) and Canada, and its sap when collected from taps and concentrated, makes a delicious syrup. Understanding how this resource may be impacted by climate change and other threats is essential to continue management for maple syrup into the future. Here, we evaluate the current...

Improved detection of sugar addition to maple syrup using malic acid as internal standard and in 13C isotope ratio mass spectrometry (IRMS).

PubMed

Tremblay, Patrice; Paquin, Réal

2007-01-24

Stable carbon isotope ratio mass spectrometry (delta13C IRMS) was used to detect maple syrup adulteration by exogenous sugar addition (beet and cane sugar). Malic acid present in maple syrup is proposed as an isotopic internal standard to improve actual adulteration detection levels. A lead precipitation method has been modified to isolate quantitatively malic acid from maple syrup using preparative reversed-phase liquid chromatography. The stable carbon isotopic ratio of malic acid isolated from this procedure shows an excellent accuracy and repeatability of 0.01 and 0.1 per thousand respectively, confirming that the modified lead precipitation method is an isotopic fractionation-free process. A new approach is proposed to detect adulteration based on the correlation existing between the delta13Cmalic acid and the delta13Csugars-delta13Cmalic acid (r = 0.704). This technique has been tested on a set of 56 authentic maple syrup samples. Additionally, authentic samples were spiked with exogeneous sugars. The mean theoretical detection level was statistically lowered using this technique in comparison with the usual two-standard deviation approach, especially when maple syrup is adulterated with beet sugar : 24 +/- 12% of adulteration detection versus 48 +/- 20% (t-test, p = 7.3 x 10-15). The method was also applied to published data for pineapple juices and honey with the same improvement.

Interannual and spatial variability of maple syrup yield as related to climatic factors

PubMed Central

Houle, Daniel

2014-01-01

Sugar maple syrup production is an important economic activity for eastern Canada and the northeastern United States. Since annual variations in syrup yield have been related to climate, there are concerns about the impacts of climatic change on the industry in the upcoming decades. Although the temporal variability of syrup yield has been studied for specific sites on different time scales or for large regions, a model capable of accounting for both temporal and regional differences in yield is still lacking. In the present study, we studied the factors responsible for interregional and interannual variability in maple syrup yield over the 2001–2012 period, by combining the data from 8 Quebec regions (Canada) and 10 U.S. states. The resulting model explained 44.5% of the variability in yield. It includes the effect of climatic conditions that precede the sapflow season (variables from the previous growing season and winter), the effect of climatic conditions during the current sapflow season, and terms accounting for intercountry and temporal variability. Optimal conditions for maple syrup production appear to be spatially restricted by less favourable climate conditions occurring during the growing season in the north, and in the south, by the warmer winter and earlier spring conditions. This suggests that climate change may favor maple syrup production northwards, while southern regions are more likely to be negatively affected by adverse spring conditions. PMID:24949244

Potential of fluorescence spectroscopy for the characterisation of maple syrup flavours.

PubMed

Panneton, Bernard; Clément, Alain; Lagacé, Luc

2013-10-01

Maple syrup has high maket value. It is produced in North East America from the heat-evaporated sap of Acer saccharum Marshall. For marketing purposes, there is interest in defining its flavour profile in a consistent and repeatable manner. An experiment was undertaken to explore the potential of autofluorescence of maple syrup induced at 275 and 360 nm to characterise flavours. A mixed data factor analysis revealed two independent groups of variables. One represents early season woody and late season empyreumatic flavours. The other is related to off-flavour, confectionery and maple flavours. Maple and confectionery flavours are subtle, difficult to distinguish and opposed to off-flavour. There were clear relationships among the two groups and fluorescence profiles. For each of the five basic flavours, discriminant models based on partial least squares regressions were developed. For each sample of syrup, flavours combined to form flavour profiles, and the results from the five discriminant models were aggregated to reproduce these profiles. For excitation at 275 nm, the woody/off-flavour and confectionery/empyreumatic/maple flavour profiles were classified correctly 86 and 78% of the time (cross-validation) respectively. Induced autofluorescence spectra were shown to contain information related to maple syrup flavours. This fluorescence-flavour relationship is not considered quantitative yet, and further research avenues are proposed. © 2013 Society of Chemical Industry.

Maple syrup-production, composition, chemistry, and sensory characteristics.

PubMed

Perkins, Timothy D; van den Berg, Abby K

2009-01-01

Maple syrup is made from sap exuded from stems of the genus Acer during the springtime. Sap is a dilute solution of primarily water and sucrose, with varying amounts of amino and organic acids and phenolic substances. When concentrated, usually by heating, a series of complex reactions produce a wide variety of flavor compounds that vary due to processing and other management factors, seasonal changes in sap chemistry, and microbial contamination. Color also forms during thermal evaporation. Flavor and color together are the primary factors determining maple syrup grade, and syrup can range from very light-colored and delicate-flavored to very dark-colored and strong-flavored.

Influence of demographic characteristics on production practices within the Ohio maple syrup industry

Treesearch

Gary W. Graham; P. Charles Goebel; Randall B. Heiligmann; Matthew S. Bumgardner

2007-01-01

Maple syrup production contributes approximately $5 million annually to Ohio's economy and provides supplemental nontimber forest product income for forestland owners. To better understand the factors that influence this important nontimber forest industry in Ohio, including producer heritage, producer age, sap collection methods, size of maple operation, and...

Artificial Sweeteners and Other Sugar Substitutes

MedlinePlus

... Lactitol Honey Sucralose (Splenda) Maltitol Maple syrup Mannitol Molasses Sorbitol Xylitol Advantame The topic of sugar substitutes ... for consumption are fruit juices and nectars, honey, molasses, and maple syrup. Natural sweeteners have a variety ...

Increasing the Efficiency of Maple Sap Evaporators with Heat Exchangers

Treesearch

Lawrence D. Garrett; Howard Duchacek; Mariafranca Morselli; Frederick M. Laing; Neil K. Huyler; James W. Marvin

1977-01-01

A study of the engineering and economic effects of heat exchangers in conventional maple syrup evaporators indicated that: (1) Efficiency was increased by 15 to 17 percent with heat exchangers; (2) Syrup produced in evaporators with heat exchangers was similar to syrup produced in conventional systems in flavor and in chemical and physical composition; and (3) Heat...

Movement Disorders in Adult Surviving Patients with Maple Syrup Urine Disease

PubMed Central

Carecchio, Miryam; Schneider, Susanne A.; Chan, Heidi; Lachmann, Robin; Lee, Philip J.; Murphy, Elaine; Bhatia, Kailash P.

2014-01-01

Maple syrup urine disease is a rare metabolic disorder caused by mutations in the branched-chain α-keto acid dehydrogenase complex gene. Patients generally present early in life with a toxic encephalopathy because of the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and the corresponding ketoacids. Movement disorders in maple syrup urine disease have typically been described during decompensation episodes or at presentation in the context of a toxic encephalopathy, with complete resolution after appropriate dietary treatment. Movement disorders in patients surviving childhood are not well documented. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed. Pyramidal signs were present in 11 patients (64.7%), and a spastic-dystonic gait was observed in 6 patients (35.2%). In summary, movement disorders are common in treated adult patients with maple syrup urine disease, and careful neurological examination is advisable to identify those who may benefit from specific therapy. PMID:21484869

Movement disorders in adult surviving patients with maple syrup urine disease.

PubMed

Carecchio, Miryam; Schneider, Susanne A; Chan, Heidi; Lachmann, Robin; Lee, Philip J; Murphy, Elaine; Bhatia, Kailash P

2011-06-01

Maple syrup urine disease is a rare metabolic disorder caused by mutations in the branched-chain α-keto acid dehydrogenase complex gene. Patients generally present early in life with a toxic encephalopathy because of the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and the corresponding ketoacids. Movement disorders in maple syrup urine disease have typically been described during decompensation episodes or at presentation in the context of a toxic encephalopathy, with complete resolution after appropriate dietary treatment. Movement disorders in patients surviving childhood are not well documented. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed. Pyramidal signs were present in 11 patients (64.7%), and a spastic-dystonic gait was observed in 6 patients (35.2%). In summary, movement disorders are common in treated adult patients with maple syrup urine disease, and careful neurological examination is advisable to identify those who may benefit from specific therapy. © 2011 Movement Disorder Society. Copyright © 2011 Movement Disorder Society.

The cost of maple sugaring in Vermont

Treesearch

Paul E. Sendak; John P. Bennink; John P. Bennink

1985-01-01

An annual maple syrup production cost series was developed for the period 1972 to 1984. Comparisons were made between plastic tubing and metal bucket technology and wood- and oil-fired evaporation. The effects of sap- sugar content and size of operation were examined together with the relationships among major cost items. The information will be useful to maple syrup...

Analysis of plastic residues in maple sap and syrup collected from tubing systems sanitized with isopropyl alcohol.

PubMed

Lagacé, Luc; Charron, Carmen; Sadiki, Mustapha

2017-05-01

A plastic tubing system operated under vacuum is usually used to collect sap from maple trees during spring time to produce maple syrup. This system is commonly sanitized with isopropyl alcohol (IPA) to remove microbial contamination colonizing the system during the sugar season. Questions have been raised whether IPA would contribute to the leaching of plastic residues in maple sap and syrup coming from sanitized systems. First, an extraction experiment was performed in the lab on commercial plastic tubing materials that were submitted to IPA under harsh conditions. The results of the GC-MS analysis revealed the presence of many compounds that served has target for further tests. Secondly, tests were done on early and mid-season maple sap and syrup coming from many sugarbushes using IPA or not to determine potential concentrations of plastic residues. Results obtained from sap and syrup samples showed that no quantifiable (< 1-75 μg/L) concentration of any plastic molecules tested was determined in all samples coming from IPA treated or not treated systems. However, some samples of first sap run used as a rinse solution to be discarded before the season start and that were coming from non sanitized or IPA sanitized systems, showed quantifiable concentrations of chemical residue such as ultraviolet protector (octabenzone). These results show that IPA can be safely used to sanitize maple sap collection system in regards to the leaching of plastic residues in maple sap and syrup and reinforced the need to thoroughly rinse the tubing system at the beginning of the season for both sanitized and non sanitized systems.

[Anaesthesia in patients with maple syrup urine disease. Case report and perioperative anaesthetic management].

PubMed

Haberstich, P; Kindler, C H; Schürch, M

2010-10-01

Maple syrup urine disease is a rare autosomal-recessive metabolic disorder caused by a deficit of oxidative decarboxylation of branched-chain amino acids. First symptoms appear in the neonatal period. Without treatment the disease is characterized by rapid progression of neurological symptoms. During stressful situations, such as infection or surgery, patients may experience severe ketoacidosis, rapid neurological deterioration and hypoglycemia. The perioperative management of a 26-year-old man with maple syrup urine disease is described, a review of the disease is given and anaesthesia-related implications are discussed.

Isoleucine Deficiency in a Neonate Treated for Maple Syrup Urine Disease Masquerading as Acrodermatitis Enteropathica.

PubMed

Ross, Benjamin; Kumar, Manish; Srinivasan, Hema; Ekbote, Alka V

2016-08-08

Special diet with restricted branched-chain-amino-acids used for treating maple syrup urine disease can lead to specific amino acid deficiencies. We report a neonate who developed skin lesions due to isoleucine deficiency while using specialised formula. Feeds were supplemented with expressed breast milk. This caused biochemical and clinical improvement with resolution of skin lesions. Breast milk is a valuable and necessary adjunct to specialized formula in maple syrup urine disease to prevent specific amino acid deficiency in the neonatal period.

29 CFR 780.800 - Scope and significance of interpretative bulletin.

Code of Federal Regulations, 2014 CFR

2014-07-01

..., or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15..., sugar-beet molasses, sugarcane or maple sap, into sugar (other than refined sugar) or syrup. The limited...

29 CFR 780.800 - Scope and significance of interpretative bulletin.

Code of Federal Regulations, 2012 CFR

2012-07-01

..., or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15..., sugar-beet molasses, sugarcane or maple sap, into sugar (other than refined sugar) or syrup. The limited...

29 CFR 780.800 - Scope and significance of interpretative bulletin.

Code of Federal Regulations, 2013 CFR

2013-07-01

..., or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15..., sugar-beet molasses, sugarcane or maple sap, into sugar (other than refined sugar) or syrup. The limited...

A cost analysis: processing maple syrup products

Treesearch

Neil K. Huyler; Lawrence D. Garrett

1979-01-01

A cost analysis of processing maple sap to syrup for three fuel types, oil-, wood-, and LP gas-fired evaporators, indicates that: (1) fuel, capital, and labor are the major cost components of processing sap to syrup; (2) wood-fired evaporators show a slight cost advantage over oil- and LP gas-fired evaporators; however, as the cost of wood approaches $50 per cord, wood...

Isolation, Identification, and Biological Evaluation of Phenolic Compounds from a Traditional North American Confectionery, Maple Sugar.

PubMed

Liu, Yongqiang; Rose, Kenneth N; DaSilva, Nicholas A; Johnson, Shelby L; Seeram, Navindra P

2017-05-31

Maple sap, collected from the sugar maple (Acer saccharum) tree, is boiled to produce the popular plant-derived sweetener, maple syrup, which can then be further evaporated to yield a traditional North American confectionery, maple sugar. Although maple sap and maple syrup have been previously studied, the phytochemical constituents of maple sugar are unknown. Herein, 30 phenolic compounds, 1-30, primarily lignans, were isolated and identified (by HRESIMS and NMR) from maple sugar. The isolates included the phenylpropanoid-based lignan tetramers (erythro,erythro)-4″,4‴-dihydroxy-3,3',3″,3‴,5,5'-hexamethoxy-7,9';7',9-diepoxy-4,8″;4',8‴-bisoxy-8,8'-dineolignan-7″,7‴,9″,9‴-tetraol, 29, and (threo,erythro)-4″,4‴-dihydroxy-3,3',3″,3‴,5,5'-hexamethoxy-7,9';7',9-diepoxy-4,8″;4',8‴-bisoxy-8,8'-dineolignan-7″,7‴,9″,9‴-tetraol, 30, neither of which have been identified from maple sap or maple syrup before. Twenty of the isolates (selected on the basis of sample quantity available) were evaluated for their potential biological effects against lipopolysaccharide-induced inflammation in BV-2 microglia in vitro and juglone-induced oxidative stress in Caenorhabditis elegans in vivo. The current study increases scientific knowledge of possible bioactive compounds present in maple-derived foods including maple sugar.

Self-Interest and Volunteerism: Analysis of a Statewide Association.

ERIC Educational Resources Information Center

Passewitz, Gregory R.; Donnermeyer, Joseph F.

1989-01-01

A survey of all known Ohio maple syrup producers indicated that economic self-interest is an important factor explaining the levels of participation in the statewide association. Although maple syrup is an important source of income for many Amish, none was involved in the association. (JOW)

Gas chromatographic determination of formaldehyde in maple syrup as 2,4-dinitrophenylhydrazone derivative.

PubMed

Baraniak, Z; Nagpal, D S; Neidert, E

1988-01-01

A procedure is presented for the quantitative extraction and determination of formaldehyde in maple sap and syrup. The method is based on the reaction between formaldehyde and 2,4-dinitrophenylhydrazine and determination of the derivative by gas chromatography. The procedure was used to evaluate formaldehyde in saps and syrups of paraformaldehyde implanted trees. Average recoveries were 101.5 +/- 5.7%. The detection limit was 0.078 mg/kg.

Ophthalmoplegia in Maple Syrup Urine Disease

ERIC Educational Resources Information Center

Zee, David S.; And Others

1974-01-01

Reported is the case of a female infant whose early symptom of ophthalmoplegia (paralysis of one or more motor nerves in the eye) led to eventual diagnosis and treatment for maple syrup urine disease, a condition in which early dietary restrictions can prevent severe mental retardation and neurologic disability. (DB)

Development and UFLC-MS/MS Characterization of a Product-Specific Standard for Phenolic Quantification of Maple-Derived Foods.

PubMed

Liu, Yongqiang; Ma, Hang; Seeram, Navindra P

2016-05-04

The phenolic contents of plant foods are commonly quantified by the Folin-Ciocalteu assay based on gallic acid equivalents (GAEs). However, this may lead to inaccuracies because gallic acid is not always representative of the structural heterogeneity of plant phenolics. Therefore, product-specific standards have been developed for the phenolic quantification of several foods. Currently, maple-derived foods (syrup, sugar, sap/water, and extracts) are quantified for phenolic contents based on GAEs. Because lignans are the predominant phenolics present in maple, herein, a maple phenolic lignan-enriched standard (MaPLES) was purified (by chromatography) and characterized (by UFLC-MS/MS with lignans previously isolated from maple syrup). Using MaPLES and secoisolariciresinol (a commercially available lignan), the phenolic contents of the maple-derived foods increased 3-fold compared to GAEs. Therefore, lignan-based standards are more appropriate for phenolic quantification of maple-derived foods versus GAEs. Also, MaPLES can be utilized for the authentication and detection of fake label claims on maple products.

Dual Mechanism of Brain Injury and Novel Treatment Strategy in Maple Syrup Urine Disease

ERIC Educational Resources Information Center

Zinnanti, William J.; Lazovic, Jelena; Griffin, Kathleen; Skvorak, Kristen J.; Paul, Harbhajan S.; Homanics, Gregg E.; Bewley, Maria C.; Cheng, Keith C.; LaNoue, Kathryn F.; Flanagan, John M.

2009-01-01

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and dysmyelination in affected individuals. Treatment requires life-long dietary restriction and monitoring of branched-chain amino acids to avoid brain injury. Despite careful management, children…

Plastic Tubing and Maple Syrup Quality

Treesearch

Russell S. Walters; Harry W. Yawney

1978-01-01

Maple syrup made from sap collected using improperly or carelessly installed plastic pipelines varied more in color from day to day, and was more often darker in color, than sap collected from either the property installed pipeline or clean, frequently emptied galvanized buckets. Use of both properly installed tubing and buckets, following recommended procedures,...

Hepatocyte Transplantation Improves Phenotype and Extends Survival in a Murine Model of Intermediate Maple Syrup Urine Disease

PubMed Central

Skvorak, Kristen J; Paul, Harbhajan S; Dorko, Kenneth; Marongiu, Fabio; Ellis, Ewa; Chace, Donald; Ferguson, Carolyn; Gibson, K Michael; Homanics, Gregg E; Strom, Stephen C

2009-01-01

Maple syrup urine disease (MSUD; OMIM 248600) is an inborn error of metabolism of the branched chain α-ketoacid dehydrogenase (BCKDH) complex that is treated primarily by dietary manipulation of branched-chain amino acids (BCAA). Dietary restriction is lifelong and compliance is difficult. Liver transplantation significantly improves outcomes; however, alternative therapies are needed. To test novel therapies such as hepatocyte transplantation (HTx), we previously created a murine model of intermediate MSUD (iMSUD), which closely mimics human iMSUD. LacZ-positive murine donor hepatocytes were harvested and directly injected (105 cells/50 µl) into liver of iMSUD mice (two injections at 1–10 days of age). Donor hepatocytes engrafted into iMSUD recipient liver, increased liver BCKDH activity, improved blood total BCAA/alanine ratio, increased body weight at weaning, and extended the lifespan of HTx-treated iMSUD mice compared to phosphate-buffered saline (PBS)–treated and untreated iMSUD mice. Based on these data demonstrating partial metabolic correction of iMSUD in a murine model, coupled to the fact that multiple transplants are possible to enhance these results, we suggest that HTx represents a promising therapeutic intervention for MSUD that warrants further investigation. PMID:19436271

Fungi in Ontario maple syrup & some factors that determine the presence of mold damage.

PubMed

Frasz, Samantha L; Miller, J David

2015-08-17

Maple syrup is a high value artisanal product produced mainly in Canada and a number of States primarily in the northeast USA. Mold growth (Wallemia sebi) on commercial product was first reported in syrup in 1908. Since then, few data have been published. We conducted a systematic examination for fungi in maple syrup from 68 producers from all of the syrup-producing areas of Ontario, Canada. The mean pH of the samples was pH 6.82, sugar content averaged 68.0±0.89 °Brix and aw averaged 0.841±0.011. Some 23 species of fungi were isolated based on morphology and molecular techniques. The most common fungus in the maple syrup samples was Eurotium herbariorum, followed by Penicillium chrysogenum, Aspergillus penicillioides, Aspergillus restrictus, Aspergillus versicolor and two species of Wallemia. Cladosporium cladosporioides was also common but only recovered when fungi known from high sugar substrates were also present in the mold damaged sample. The rarely reported yeast Citeromyces matrinsis was found in samples from three producers. There appear to be three potential causes for mold damage observed. High aw was associated with about one third of the mold damage. Independently, cold packing (bottling at ~25 °C) was a risk factor. However, syrup of good quality and quite low aw values was contaminated. We hypothesize that sanitation in the bottling line and other aspects of the bottling process may be partial explanations. Clarifying this requires further study. Copyright © 2015 Elsevier B.V. All rights reserved.

Processing Maple Syrup with a Vapor Compression Distiller: An Economic Analysis

Treesearch

Lawrence D. Garrett

1977-01-01

A test of vapor compression distillers for processing maple syrup revealed that: (1) vapor compression equipment tested evaporated 1 pound of water with .047 pounds of steam equivalent (electrical energy); open-pan evaporators of similar capacity required 1.5 pounds of steam equivalent (oil energy) to produce 1 pound of water; (2) vapor compression evaporation produced...

Maple Syrup Decreases TDP-43 Proteotoxicity in a Caenorhabditis elegans Model of Amyotrophic Lateral Sclerosis (ALS).

PubMed

Aaron, Catherine; Beaudry, Gabrielle; Parker, J Alex; Therrien, Martine

2016-05-04

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease causing death of the motor neurons. Proteotoxicity caused by TDP-43 protein is an important aspect of ALS pathogenesis, with TDP-43 being the main constituent of the aggregates found in patients. We have previously tested the effect of different sugars on the proteotoxicity caused by the expression of mutant TDP-43 in Caenorhabditis elegans. Here we tested maple syrup, a natural compound containing many active molecules including sugars and phenols, for neuroprotective activity. Maple syrup decreased several age-dependent phenotypes caused by the expression of TDP-43(A315T) in C. elegans motor neurons and requires the FOXO transcription factor DAF-16 to be effective.

Total antioxidant content of alternatives to refined sugar.

PubMed

Phillips, Katherine M; Carlsen, Monica H; Blomhoff, Rune

2009-01-01

Oxidative damage is implicated in the etiology of cancer, cardiovascular disease, and other degenerative disorders. Recent nutritional research has focused on the antioxidant potential of foods, while current dietary recommendations are to increase the intake of antioxidant-rich foods rather than supplement specific nutrients. Many alternatives to refined sugar are available, including raw cane sugar, plant saps/syrups (eg, maple syrup, agave nectar), molasses, honey, and fruit sugars (eg, date sugar). Unrefined sweeteners were hypothesized to contain higher levels of antioxidants, similar to the contrast between whole and refined grain products. To compare the total antioxidant content of natural sweeteners as alternatives to refined sugar. The ferric-reducing ability of plasma (FRAP) assay was used to estimate total antioxidant capacity. Major brands of 12 types of sweeteners as well as refined white sugar and corn syrup were sampled from retail outlets in the United States. Substantial differences in total antioxidant content of different sweeteners were found. Refined sugar, corn syrup, and agave nectar contained minimal antioxidant activity (<0.01 mmol FRAP/100 g); raw cane sugar had a higher FRAP (0.1 mmol/100 g). Dark and blackstrap molasses had the highest FRAP (4.6 to 4.9 mmol/100 g), while maple syrup, brown sugar, and honey showed intermediate antioxidant capacity (0.2 to 0.7 mmol FRAP/100 g). Based on an average intake of 130 g/day refined sugars and the antioxidant activity measured in typical diets, substituting alternative sweeteners could increase antioxidant intake an average of 2.6 mmol/day, similar to the amount found in a serving of berries or nuts. Many readily available alternatives to refined sugar offer the potential benefit of antioxidant activity.

Acute Pancreatitis in a Patient with Maple Syrup Urine Disease: A Management Paradox.

PubMed

Gold, Nina B; Blumenthal, Jennifer A; Wessel, Ann E; Stein, Deborah R; Scott, Adam; Fox, Victor L; Turner, Amy; Kritzer, Amy; Rajabi, Farrah; Peeler, Katherine; Tan, Wen-Hann

2018-04-19

Maple syrup urine disease (MSUD) is an inborn error of metabolism that causes elevated leucine in the setting of acute illnesses. We describe an 8-year-old boy with MSUD who developed acute pancreatitis and subsequent leucinosis. This case highlights the complexities of fluid management in patients with MSUD. Copyright © 2018 Elsevier Inc. All rights reserved.

Coniferous Understory Influences Sugar Maple (Acer Saccharum Marsh.) Sap Production

Treesearch

Russell S. Walters

1978-01-01

Sap and maple syrup equivalent production increased after a coniferous understory was removed from a sugarbush in northwestern Vermont. These increases, which became apparent the sixth year after treatment, were 14 and 17 percent for sap and syrup respectively, relative to the yields from an adjacent open sugarbush. The open sugarbush yields were used as the control in...

The Maple Products: An Outdoor Education Unit.

ERIC Educational Resources Information Center

Yaple, Charles; And Others

Designed to take advantage of the spring season, this resource packet on maple products centers upon a field lesson in harvesting and making maple syrup. The resources in this packet include: a narrative on the origins of maple sugar; an illustrated description of "old time maple sugarin'"; suggestions for pre-trip activities (history of…

The Anaesthetic Management of a Patient with Maple Syrup Urine Disease

PubMed Central

Karahan, Mahmut Alp; Sert, Hüseyin; Havlioğlu, İnanç; Yüce, Hasan Hüsnü

2014-01-01

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficit of oxidative decarboxylation of branched-chain aminoacids. It leads to a build-up of leucine, isoleucine, valine, and toxic metabolites in blood and urine, progressing to acute and chronic brain dysfunction. The first symptoms appear in early childhood and are characterized by sweet-smelling urine, with an odor similar to that of maple syrup. At birth, infants seem healthy, but if untreated, they may suffer from neurological deterioration, seizures, hypertonia, or ataxia. During stressful situations, such as infection or surgery, patients may experience severe ketoacidosis, rapid neurological deterioration, and hypoglycemia. We report the anaesthetic management in a child patient with MSUD, admitted for peritonal dialysis catheter insertion with general anaesthesia. PMID:27366451

The Anaesthetic Management of a Patient with Maple Syrup Urine Disease.

PubMed

Karahan, Mahmut Alp; Sert, Hüseyin; Havlioğlu, İnanç; Yüce, Hasan Hüsnü

2014-12-01

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficit of oxidative decarboxylation of branched-chain aminoacids. It leads to a build-up of leucine, isoleucine, valine, and toxic metabolites in blood and urine, progressing to acute and chronic brain dysfunction. The first symptoms appear in early childhood and are characterized by sweet-smelling urine, with an odor similar to that of maple syrup. At birth, infants seem healthy, but if untreated, they may suffer from neurological deterioration, seizures, hypertonia, or ataxia. During stressful situations, such as infection or surgery, patients may experience severe ketoacidosis, rapid neurological deterioration, and hypoglycemia. We report the anaesthetic management in a child patient with MSUD, admitted for peritonal dialysis catheter insertion with general anaesthesia.

Wernicke-like encephalopathy during classic maple syrup urine disease decompensation.

PubMed

Manara, R; Del Rizzo, M; Burlina, A P; Bordugo, A; Citton, V; Rodriguez-Pombo, P; Ugarte, M; Burlina, A B

2012-05-01

We describe a new neuroradiologic picture observed during metabolic decompensation in two maple syrup urine disease (MSUD) patients that resembles Wernicke encephalopathy (WE). Clinical observations and the review of the literature regarding WE and MSUD pathophysiology prompted us to hypothesize a pathogenic link between these two disorders. Based on these findings, clinicians and neuroradiologists should be aware of MSUD as a possible predisposing factor of WE in children.

Tapping the Sugar Maple--Learning and Appreciating

ERIC Educational Resources Information Center

Malone, Charles

1976-01-01

The article discusses how to tap a maple tree. Tapping a maple tree to produce maple syrup can: (1) lead to better understanding in many subject areas, (2) develop skills through participation in a rewarding activity, and (3) help students appreciate the many important roles that trees play in our environment and daily lives. (NQ)

Branched-Chain Amino and Keto Acid Biochemistry and Cellular Biology in Central Nervous System Diseases

DTIC Science & Technology

2009-05-21

pyruvate dehydrogenase complex (PDC) and 2-oxo- glutarate dehydrogenase complex. These dehydrogenase complexes share the same basic structure, perform the...Science 312 (2006) 927-930. [20] J. Dancis, M. Levitz, R.G. Westall, Maple syrup urine disease: branched- chain keto- aciduria , Pediatrics 25 (1960...2127 2128 Dancis J, Levitz M, Westall RG. 1960. Maple syrup urine disease: branched-chain keto- aciduria . Pediatrics 25:72-9. Danner DJ, Lemmon

Sensory-motor polyneuropathy occurring in variant maple syrup urine disease.

PubMed

Harty, S; King, M D; McCoy, B; Costigan, D; Treacy, E P

2008-12-01

Maple syrup urine disease (MSUD; OMIM 248600) results from an inherited deficiency of the branched-chain ketoacid dehydrogenase (BCKD) complex. Approximately 20% of patients with BCKD deficiency are non-classic variants of MSUD with differing clinical severity. Outcomes for this cohort are generally favourable; episodes of metabolic decompensation do not appear to correlate with adverse events if acute management is promptly provided. A case of predominantly axonal sensory-motor neuropathy following metabolic decompensation which persisted for a number of months is presented in an adolescent girl with variant (intermediate type) MSUD. EMG and nerve conduction studies suggested a pre-existent asymptomatic chronic neuropathy, exacerbated by the acute decompensation. Peak leucine concentration at decompensation was 1083 μmol/L. The patient had laboratory signs of secondary mitochondrial respiratory chain dysfunction at presentation. She had been on a moderate dose of thiamine prior to decompensation; thiamine and pyridoxine blood concentrations were normal. This, to our knowledge, is the first report of a neuropathy presenting in a patient with a decompensation of variant MSUD. We propose that this presentation resembles the intermittent neuropathy observed in pyruvate dehydrogenase deficiency and may reflect secondary inhibition of pyruvate dehydrogenase activity by MSUD metabolites.

Identification of a founder mutation for maple syrup urine disease in Hutterites.

PubMed

Mroch, Amelia; Davis-Keppen, Laura; Matthes, Cindy; Stein, Quinn

2014-04-01

Maple syrup urine disease (MSUD) is an organic acidemia detected on newborn screening. The condition has been reported with increased frequency in certain founder populations including Hutterites. We present a case of MSUD in a Hutterite boy. Mutation analysis was completed and identified a candidate founder mutation in the BCKDHB gene, specifically c.595_596delAG. Further testing of other Hutterites with MSUD is needed to determine whether additional mutations may exist.

Haemodialysis is an effective treatment in acute metabolic decompensation of maple syrup urine disease.

PubMed

Atwal, P S; Macmurdo, C; Grimm, P C

2015-09-01

Acute metabolic decompensation in maple syrup urine disease can occur during intercurrent illness and is a medical emergency. A handful of reports in the medical literature describe the use of peritoneal dialysis and haemodialysis as therapeutic inventions. We report the only patient from our centre to have haemodialysis performed in this setting. Combined with dietary BCAA restriction and calorific support, haemodialysis allows rapid reduction in plasma leucine concentrations considerably faster than conservative methods.

Placental stem cell correction of murine intermediate maple syrup urine disease.

PubMed

Skvorak, Kristen J; Dorko, Kenneth; Marongiu, Fabio; Tahan, Veysel; Hansel, Marc C; Gramignoli, Roberto; Gibson, K Michael; Strom, Stephen C

2013-03-01

There is improved survival and partial metabolic correction of a mouse intermediate maple syrup urine disease (iMSUD) model after allogenic hepatocyte transplantation, confirming that a small number of enzyme-proficient liver-engrafted cells can improve phenotype. However, clinical shortages of suitable livers for hepatocyte isolation indicate a need for alternative cell sources. Human amnion epithelial cells (hAECs) share stem cell characteristics without the latter's safety and ethical concerns and differentiate to hepatocyte-like cells. Eight direct hepatic hAEC transplantations were performed in iMSUD mice over the first 35 days beginning at birth; animals were provided a normal protein diet and sacrificed at 35 and 100 days. Treatment at the neonatal stage is clinically relevant for MSUD and may offer a donor cell engraftment advantage. Survival was significantly extended and body weight was normalized in iMSUD mice receiving hAEC transplantations compared with untreated iMSUD mice, which were severely cachectic and died ≤28 days after birth. Branched chain α-keto acid dehydrogenase enzyme activity was significantly increased in transplanted livers. The branched chain amino acids leucine, isoleucine, valine, and alloisoleucine were significantly improved in serum and brain, as were other large neutral amino acids. Placental-derived stem cell transplantation lengthened survival and corrected many amino acid imbalances in a mouse model of iMSUD. This highlights the potential for their use as a viable alternative clinical therapy for MSUD and other liver-based metabolic diseases. Copyright © 2012 American Association for the Study of Liver Diseases.

Placental Stem Cell Correction of Murine Intermediate Maple Syrup Urine Disease

PubMed Central

Skvorak, Kristen J.; Dorko, Kenneth; Marongiu, Fabio; Tahan, Veysel; Hansel, Marc C.; Gramignoli, Roberto; Gibson, K. Michael; Strom, Stephen C.

2012-01-01

We previously reported improved survival and partial metabolic correction of a mouse intermediate maple syrup urine disease (iMSUD) model post allogenic hepatocyte transplant, confirming that a small number of enzyme proficient liver-engrafted cells can improve phenotype. However, clinical shortages of suitable livers for hepatocyte isolation indicate a need for alternative cell sources. Human amnion epithelial cells (hAEC) share stem cell characteristics while lacking many safety and ethical concerns, and differentiate to hepatocyte-like cells. Eight direct hepatic hAEC transplants were administered to iMSUD mice over the first 35 days beginning at birth; animals were provided a normal protein diet and sacrificed at days 35 and 100. Treatment at the neonatal stage is clinically relevant for MSUD, and may offer a donor cell engraftment advantage. Survival was significantly extended and body weight was normalized in iMSUD mice receiving hAEC transplants compared to iMSUD (severely cachectic; dead ≤28 days). Branched chain α-keto acid dehydrogenase enzyme activity was significantly increased in transplanted livers. Branched chain amino acids leucine, isoleucine, valine, and alloisoleucine were significantly improved in the sera and brain, as were other large neutral amino acids. Conclusion: Placental-derived stem cell transplantation lengthened survival and corrected many amino acid imbalances in a mouse model of iMSUD. This highlights the potential for their use as a viable alternative clinical therapy for MSUD and other liver-based metabolic diseases. PMID:23175463

Variation in sugar content in a budded sugar maple clone

Treesearch

Frank S., Jr. Santamour; Frank E. Cunningham

1964-01-01

The production of maple sugar and maple syrup from the sap of the sugar maple tree (Acer saccharum Marsh.) is an important industry in the Northeast, particularly in Vermont and New York. Although it has been recognized for a long time that some trees are sweeter than others - have a higher sugar content in their sap - systematic study of ways to...

Production and characterization of murine models of classic and intermediate maple syrup urine disease

PubMed Central

Homanics, Gregg E; Skvorak, Kristen; Ferguson, Carolyn; Watkins, Simon; Paul, Harbhajan S

2006-01-01

Background Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical phenotypes depending on the degree of enzyme deficiency. Current treatments are not satisfactory and require new approaches to combat this disease. A major hurdle in developing new treatments has been the lack of a suitable animal model. Methods To create a murine model of classic MSUD, we used gene targeting and embryonic stem cell technologies to create a mouse line that lacked a functional E2 subunit gene of branched-chain keto acid dehydrogenase. To create a murine model of intermediate MSUD, we used transgenic technology to express a human E2 cDNA on the knockout background. Mice of both models were characterized at the molecular, biochemical, and whole animal levels. Results By disrupting the E2 subunit gene of branched-chain keto acid dehydrogenase, we created a gene knockout mouse model of classic MSUD. The homozygous knockout mice lacked branched-chain keto acid dehydrogenase activity, E2 immunoreactivity, and had a 3-fold increase in circulating branched-chain amino acids. These metabolic derangements resulted in neonatal lethality. Transgenic expression of a human E2 cDNA in the liver of the E2 knockout animals produced a model of intermediate MSUD. Branched-chain keto acid dehydrogenase activity was 5–6% of normal and was sufficient to allow survival, but was insufficient to normalize circulating branched-chain amino acids levels, which were intermediate between wildtype and the classic MSUD mouse model. Conclusion These mice represent important animal models that closely approximate the phenotype of humans with the classic and intermediate forms of MSUD. These animals provide useful models to further characterize the pathogenesis of MSUD, as well as models to test novel therapeutic strategies, such as gene and cellular therapies, to treat this devastating metabolic disease. PMID:16579849

Determination of the 13C/12C ratio of ethanol derived from fruit juices and maple syrup by isotope ratio mass spectrometry: collaborative study.

PubMed

Jamin, Eric; Martin, Frédérique; Martin, Gilles G

2004-01-01

A collaborative study of the carbon-13 isotope ratio mass spectrometry (13C-IRMS) method based on fermentation ethanol for detecting some sugar additions in fruit juices and maple syrup is reported. This method is complementary to the site-specific natural isotope fractionation by nuclear magnetic resonance (SNIF-NMR) method for detecting added beet sugar in the same products (AOAC Official Methods 995.17 and 2000.19), and uses the same initial steps to recover pure ethanol. The fruit juices or maple syrups are completely fermented with yeast, and the alcohol is distilled with a quantitative yield (>96%). The carbon-13 deviation (delta13C) of ethanol is then determined by IRMS. This parameter becomes less negative when exogenous sugar derived from plants exhibiting a C4 metabolism (e.g., corn or cane) is added to a juice obtained from plants exhibiting a C3 metabolism (most common fruits except pineapple) or to maple syrup. Conversely, the delta13C of ethanol becomes more negative when exogenous sugar derived from C3 plants (e.g., beet, wheat, rice) is added to pineapple products. Twelve laboratories analyzed 2 materials (orange juice and pure cane sugar) in blind duplicate and 4 sugar-adulterated materials (orange juice, maple syrup, pineapple juice, and apple juice) as Youden pairs. The precision of that method for measuring delta13C was similar to that of other methods applied to wine ethanol or extracted sugars in juices. The within-laboratory (Sr) values ranged from 0.06 to 0.16%o (r = 0.17 to 0.46 percent per thousand), and the among-laboratories (SR) values ranged from 0.17 to 0.26 percent per thousand (R = 0.49 to 0.73 percent per thousand). The Study Directors recommend that the method be adopted as First Action by AOAC INTERNATIONAL.

29 CFR 780.801 - Statutory provisions.

Code of Federal Regulations, 2010 CFR

2010-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar..., sugar-beet molasses, sugarcane, or maple sap, into sugar (other than refined sugar) or syrup. Section 13...

29 CFR 780.801 - Statutory provisions.

Code of Federal Regulations, 2011 CFR

2011-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar..., sugar-beet molasses, sugarcane, or maple sap, into sugar (other than refined sugar) or syrup. Section 13...

29 CFR 780.801 - Statutory provisions.

Code of Federal Regulations, 2014 CFR

2014-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar..., sugar-beet molasses, sugarcane, or maple sap, into sugar (other than refined sugar) or syrup. Section 13...

29 CFR 780.801 - Statutory provisions.

Code of Federal Regulations, 2012 CFR

2012-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar..., sugar-beet molasses, sugarcane, or maple sap, into sugar (other than refined sugar) or syrup. Section 13...

29 CFR 780.801 - Statutory provisions.

Code of Federal Regulations, 2013 CFR

2013-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar..., sugar-beet molasses, sugarcane, or maple sap, into sugar (other than refined sugar) or syrup. Section 13...

Haemodialysis is an effective treatment in acute metabolic decompensation of maple syrup urine disease

PubMed Central

Atwal, P.S.; Macmurdo, C.; Grimm, P.C.

2015-01-01

Acute metabolic decompensation in maple syrup urine disease can occur during intercurrent illness and is a medical emergency. A handful of reports in the medical literature describe the use of peritoneal dialysis and haemodialysis as therapeutic inventions. We report the only patient from our centre to have haemodialysis performed in this setting. Combined with dietary BCAA restriction and calorific support, haemodialysis allows rapid reduction in plasma leucine concentrations considerably faster than conservative methods. PMID:26937409

Heavy metal, pH, and total solid content of maple sap and syrup produced in eastern Canada.

PubMed

Robinson, A R; MacLean, K S; MacConnell, H M

1989-01-01

Maple sap and syrups in eastern Canada were analyzed for pH, total solids, and the heavy metals Cu, Fe, Pb, and Zn. The levels of heavy metals found were within the range normally contained in food and water samples except for Pb. The concentration factor found in reducing sap to syrup did not reflect the same concentration change for the measured parameters. This indicates removal or conversion of heavy metals and organic acids with the sugar sands. There was no statistical difference among provinces with respect to the heavy metal, pH, and total solids content of sap. The only significant difference in syrup occurred with Cu and this appeared to be the result of the processing procedure. As the season progressed, the Cu, Pb, pH, and total solids content of the sap decreased while Zn increased and Fe showed little change. Syrups reflected a similar change. Statistical differences occurred in sap composition among sites within each province.

Replacement of Sugar Syrup with High-Fructose Syrup in Imitation Maple Syrup

DTIC Science & Technology

1975-01-01

Corn syrupa Liquid sugar1- High - fructose syrupc Corn syrupa High - fructose syrup Corn syrupa TADLE 1. Thin Syrup Formulation...c <&> y &> - — 4ß ^ 11 12 13 14 15 16 17 IB % SOLIDS BASIS ISOSWEET 100 HIGH FRUCTOSE CORN SYRUP (Courtesy of Staley) "’ ■ "MMM—MM...iJjffVllMW! fmmmm,,m*w.mn’i’»l’« mm ■ " "’" ’" W ’ "~ FrnT1ŕ" "■ «»<■■ MBMH

29 CFR 780.819 - Production must be of unrefined sugar or syrup.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 29 Labor 3 2010-07-01 2010-07-01 false Production must be of unrefined sugar or syrup. 780.819... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15...

29 CFR 780.819 - Production must be of unrefined sugar or syrup.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 29 Labor 3 2011-07-01 2011-07-01 false Production must be of unrefined sugar or syrup. 780.819... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15...

29 CFR 780.819 - Production must be of unrefined sugar or syrup.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 3 2013-07-01 2013-07-01 false Production must be of unrefined sugar or syrup. 780.819... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15...

29 CFR 780.819 - Production must be of unrefined sugar or syrup.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 3 2012-07-01 2012-07-01 false Production must be of unrefined sugar or syrup. 780.819... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15...

29 CFR 780.819 - Production must be of unrefined sugar or syrup.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 3 2014-07-01 2014-07-01 false Production must be of unrefined sugar or syrup. 780.819... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15...

Maple syrup urine disease: tailoring a plan for pregnancy.

PubMed

Brown, James; Tchan, Michel; Nayyar, Roshini

2018-06-01

Maple syrup urine disease (MSUD) is an autosomal metabolic condition that can cause significant morbidity in pregnant women and their infants. Advances in nutritional management mean more mothers with congenital metabolic disorders are becoming pregnant, and presenting challenges in obstetric care. In particular, the increase in protein requirements during pregnancy and metabolic stress of childbirth require careful planning and caloric titration. We report a case of a second time mother with MSUD and demonstrate a treatment scaffold to help achieve successful pregnancies for women with this and similar conditions.

Hepatitis C: Diet and Nutrition

MedlinePlus

... Sugary foods Sugar goes by many names: sucrose, corn syrup, honey, maple syrup, and fructose. Sugary foods tend to offer little more than calories. Many of them (such as pastries and desserts) tend to be high in fat, too. There is nothing wrong with ...

Establishing an Alaskan birch syrup industry: Birch Syrup—It’s the Un-maple!TM

Treesearch

Marlene Cameron

2001-01-01

Ten years ago a small group of Alaskans began commercial production of birch syrup from the sap of the paper birch, Betula papyrifera, and established an industry that is expanding in response to demand and has the potential to make a significant contribution to Alaska's economy. There are still many problems to be solved; research and...

Polyphenolic Extract from Maple Syrup Potentiates Antibiotic Susceptibility and Reduces Biofilm Formation of Pathogenic Bacteria

PubMed Central

Maisuria, Vimal B.; Hosseinidoust, Zeinab

2015-01-01

Phenolic compounds are believed to be promising candidates as complementary therapeutics. Maple syrup, prepared by concentrating the sap from the North American maple tree, is a rich source of natural and process-derived phenolic compounds. In this work, we report the antimicrobial activity of a phenolic-rich maple syrup extract (PRMSE). PRMSE exhibited antimicrobial activity as well as strong synergistic interaction with selected antibiotics against Gram-negative clinical strains of Escherichia coli, Proteus mirabilis, and Pseudomonas aeruginosa. Among the phenolic constituents of PRMSE, catechol exhibited strong synergy with antibiotics as well as with other phenolic components of PRMSE against bacterial growth. At sublethal concentrations, PRMSE and catechol efficiently reduced biofilm formation and increased the susceptibility of bacterial biofilms to antibiotics. In an effort to elucidate the mechanism for the observed synergy with antibiotics, PRMSE was found to increase outer membrane permeability of all bacterial strains and effectively inhibit efflux pump activity. Furthermore, transcriptome analysis revealed that PRMSE significantly repressed multiple-drug resistance genes as well as genes associated with motility, adhesion, biofilm formation, and virulence. Overall, this study provides a proof of concept and starting point for investigating the molecular mechanism of the reported increase in bacterial antibiotic susceptibility in the presence of PRMSE. PMID:25819960

Polyphenolic extract from maple syrup potentiates antibiotic susceptibility and reduces biofilm formation of pathogenic bacteria.

PubMed

Maisuria, Vimal B; Hosseinidoust, Zeinab; Tufenkji, Nathalie

2015-06-01

Phenolic compounds are believed to be promising candidates as complementary therapeutics. Maple syrup, prepared by concentrating the sap from the North American maple tree, is a rich source of natural and process-derived phenolic compounds. In this work, we report the antimicrobial activity of a phenolic-rich maple syrup extract (PRMSE). PRMSE exhibited antimicrobial activity as well as strong synergistic interaction with selected antibiotics against Gram-negative clinical strains of Escherichia coli, Proteus mirabilis, and Pseudomonas aeruginosa. Among the phenolic constituents of PRMSE, catechol exhibited strong synergy with antibiotics as well as with other phenolic components of PRMSE against bacterial growth. At sublethal concentrations, PRMSE and catechol efficiently reduced biofilm formation and increased the susceptibility of bacterial biofilms to antibiotics. In an effort to elucidate the mechanism for the observed synergy with antibiotics, PRMSE was found to increase outer membrane permeability of all bacterial strains and effectively inhibit efflux pump activity. Furthermore, transcriptome analysis revealed that PRMSE significantly repressed multiple-drug resistance genes as well as genes associated with motility, adhesion, biofilm formation, and virulence. Overall, this study provides a proof of concept and starting point for investigating the molecular mechanism of the reported increase in bacterial antibiotic susceptibility in the presence of PRMSE. Copyright © 2015, American Society for Microbiology. All Rights Reserved.

Challenges in the management of patients with maple syrup urine disease diagnosed by newborn screening in a developing country.

PubMed

De Castro-Hamoy, Leniza G; Chiong, Mary Anne D; Estrada, Sylvia C; Cordero, Cynthia P

2017-01-01

Maple syrup urine disease (MSUD) is a rare inborn error of metabolism resulting from a deficiency in the branched-chain alpha-ketoacid dehydrogenase complex. MSUD has been reported to be the most common inborn error of metabolism in the Philippines. We described all patients with maple syrup urine disease patients diagnosed through newborn screening during its first 2 years of implementation and the challenges encountered during their medical management. There were 24 patients diagnosed with maple syrup urine disease for the 2-year period. All patients needed hospital admission. The most common complication during hospital admission was infection, needing intravenous antibiotics which were given to 21 of the patients. Out of the 24 diagnosed, 16 patients are alive, while eight have died. Several neurologic and non-neurologic complications have been observed during the follow-up of the patients. The common challenges of MSUD management in a low-resource setting identified in this study were late diagnosis, lack of access to metabolic specialists and medical supplies, nosocomial septicemia, and protein deficiency. Aside from early properly timed collection, improvement in other logistical concerns will also help in earlier diagnosis. Mechanisms of transfer of critically ill patients must be improved. Hospitals in difficult-to-reach areas must be equipped to handle critical metabolic cases when transfers are not possible. Newborn screening has been proven to improve outcome in patients with MSUD but the success of the program in preventing disability is also dependent on improvements in other aspects of healthcare.

Maple syrup urine disease: mechanisms and management.

PubMed

Blackburn, Patrick R; Gass, Jennifer M; Vairo, Filippo Pinto E; Farnham, Kristen M; Atwal, Herjot K; Macklin, Sarah; Klee, Eric W; Atwal, Paldeep S

2017-01-01

Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by defects in the branched-chain α-ketoacid dehydrogenase complex, which results in elevations of the branched-chain amino acids (BCAAs) in plasma, α-ketoacids in urine, and production of the pathognomonic disease marker, alloisoleucine. The disorder varies in severity and the clinical spectrum is quite broad with five recognized clinical variants that have no known association with genotype. The classic presentation occurs in the neonatal period with developmental delay, failure to thrive, feeding difficulties, and maple syrup odor in the cerumen and urine, and can lead to irreversible neurological complications, including stereotypical movements, metabolic decompensation, and death if left untreated. Treatment consists of dietary restriction of BCAAs and close metabolic monitoring. Clinical outcomes are generally good in patients where treatment is initiated early. Newborn screening for MSUD is now commonplace in the United States and is included on the Recommended Uniform Screening Panel (RUSP). We review this disorder including its presentation, screening and clinical diagnosis, treatment, and other relevant aspects pertaining to the care of patients.

Neonatal case of classic maple syrup urine disease: usefulness of (1) H-MRS in early diagnosis.

PubMed

Sato, Takeshi; Muroya, Koji; Hanakawa, Junko; Asakura, Yumi; Aida, Noriko; Tomiyasu, Moyoko; Tajima, Go; Hasegawa, Tomonobu; Adachi, Masanori

2014-02-01

We describe a male neonate with classic maple syrup urine disease (MSUD) in metabolic crisis. On day 7 of life, he was referred to hospital because of coma and metabolic acidosis with maple syrup odor. On day 4 after admission, brain magnetic resonance imaging findings were consistent with encephalopathy due to MSUD. Proton magnetic resonance spectroscopy ((1) H-MRS) showed a large methyl resonance peak at 0.9 p.p.m. The diagnosis of MSUD was confirmed on low branched-chain α-keto acid dehydrogenase complex activity in lymphocyte. (1) H-MR spectra were obtained in 10 min, while it took at least several days to obtain the results of other diagnostic examinations. In convalescence, the peak at 0.9 p.p.m. decreased. The large methyl resonance peak at 0.9 p.p.m. in brain (1) H-MRS would be one of the earliest clues to the diagnosis of classic MSUD in the neonatal period, especially in metabolic crisis. © 2014 The Authors. Pediatrics International © 2014 Japan Pediatric Society.

Maple syrup urine disease: mechanisms and management

PubMed Central

Farnham, Kristen M; Atwal, Herjot K; Macklin, Sarah; Klee, Eric W; Atwal, Paldeep S

2017-01-01

Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by defects in the branched-chain α-ketoacid dehydrogenase complex, which results in elevations of the branched-chain amino acids (BCAAs) in plasma, α-ketoacids in urine, and production of the pathognomonic disease marker, alloisoleucine. The disorder varies in severity and the clinical spectrum is quite broad with five recognized clinical variants that have no known association with genotype. The classic presentation occurs in the neonatal period with developmental delay, failure to thrive, feeding difficulties, and maple syrup odor in the cerumen and urine, and can lead to irreversible neurological complications, including stereotypical movements, metabolic decompensation, and death if left untreated. Treatment consists of dietary restriction of BCAAs and close metabolic monitoring. Clinical outcomes are generally good in patients where treatment is initiated early. Newborn screening for MSUD is now commonplace in the United States and is included on the Recommended Uniform Screening Panel (RUSP). We review this disorder including its presentation, screening and clinical diagnosis, treatment, and other relevant aspects pertaining to the care of patients. PMID:28919799

Management of a Woman With Maple Syrup Urine Disease During Pregnancy, Delivery, and Lactation.

PubMed

Wessel, Ann E; Mogensen, Kris M; Rohr, Frances; Erick, Miriam; Neilan, Edward G; Chopra, Sameer; Levy, Harvey L; Gray, Kathryn J; Wilkins-Haug, Louise; Berry, Gerard T

2015-09-01

Maple syrup urine disease (MSUD) is an inherited disorder of metabolism of the branched-chain amino acids leucine, isoleucine, and valine. Complications of acute elevation in plasma leucine include ketoacidosis and risk of cerebral edema, which can be fatal. Individuals with MSUD are at risk of metabolic crisis throughout life, especially at times of physiological stress. We present a case of successful management of a woman with MSUD through pregnancy, delivery, postpartum, and lactation, including nutrition therapy using modified parenteral nutrition. © 2014 American Society for Parenteral and Enteral Nutrition.

Reexamination of effects of paraformaldehyde on tissues around tapholes in sugar maple trees

Treesearch

David R. Houston; J. Chris Fagan

1997-01-01

Pellets containing paraformaldehyde (PFA) are sometimes placed behind spouts in tapped sugar maples to prolong sapflow and increase the yield of sap used to make maple syrup.The practice is controversial because the chemical has been shown to promote decay and is reported to cause cambial dieback around tapholes.This study reexamined the effects of PFA on tapped sugar...

[Acute encephalopathy due to late-onset maple syrup urine disease in a school boy].

PubMed

Qu, Su-Qing; Yang, Li-Cai; Luan, Zuo; Du, Kan; Yang, Hui

2012-03-01

Maple syrup urine disease is a common amino acids metabolic disease. In most patients, onset occurs in the neonatal period and infancy. In this study, the case of a school boy with acute encephalopathy due to late-onset maple syrup urine disease is summarized. The boy (8.5 years) was admitted because of acute encephalopathy after suffering from infection for two days at the age of eight and a half years. Metabolic acidosis, hyperuricemia and decreased protein level in cerebrospinal fluid were found by general laboratory tests. Magnetic resonance imaging of the brain revealed signal intensity abnormalities in the bilateral cerebellum dentate nucleus, brainstem, thalamus, putamen, caudate nucleus and cortex of the cerebral hemispheres. On T1WI and T2WI scanning, hyperintensive signal was found. Blood leucine and valine were significantly elevated. Urinary 2-hydroxy isovaleric acid, 3-hydroxybutyric acid, 2-keto isovaleric acid, and 2-keto acid also increased. Both the blood amino acid and urine organic acid profiles led to the diagnosis of maple syrup urine disease. In the acute period, the patient was treated with a large dose of vitamin B1, glucose, L-carnitine and a protein-restrict diet. The patient's condition improved significantly after five days of treatment, and he recovered completely two days later. Afterwards, treatment with vitamin B1, L-carnitine and a protein-restrict diet (1 g/kg/day) was continued. One and a half months later, blood amino acids and urine organic acids returned to normal. Magnetic resonance imaging of the brain also indicated a great improvement. It was concluded that inborn metabolic disease should be considered in the patients with an onset similar to acute encephalopathy. Early diagnosis and proper treatment can prevent brain damage and improve prognosis.

Molecular basis of maple syrup urine disease: Novel mutations at the E1[alpha] locus that impair E1([alpha][sub 2][beta][sub 2]) assembly or decrease steady-state E1[alpha] mRNA levels of branched-chain [alpha]-keto acid dehydrogenase complex

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chuang, J.L.; Fisher, C.R.; Chuang, D.T.

1994-08-01

The authors report the occurrence of three novel mutations in the E1[alpha] (BCKDHA) locus of the branched-chain [alpha]-keto acid dehydrogenase (BCKAD) complex that cause maple syrup urine disease (MSUD). An 8-bp deletion in exon 7 is present in one allele of a compound-heterozygous patient (GM-649). A single C nucleotide insertion in exon 2 occurs in one allele of an intermediate-MSUD patient (Lo). The second allele of patient Lo carries an A-to-G transition in exon 9 of the E1[alpha] gene. This missense mutation changes Tyr-368 to Cys (Y368C) in the E1[alpha] subunit. Both the 8-bp deletion and the single C insertionmore » generate a downstream nonsense codon. Both mutations appear to be associated with a low abundance of the mutant E1[alpha] mRNA, as determined by allele-specific oligonucleotide probing. Transfection studies strongly suggest that the Y368C substitution in the E1[alpha] subunit impairs its proper assembly with the normal E1[beta]. Unassembled as well as misassembled E1[alpha] and E1[beta] subunits are degraded in the cell. 32 refs., 8 figs.« less

Implications of Maple Syrup Urine Disease in Newborns.

PubMed

Harris-Haman, Pamela; Brown, Lenora; Massey, Susan; Ramamoorthy, Sivaranjani

Maple syrup urine disease (MSUD) is an inherited metabolic disorder that affects the body's ability to metabolize amino acids. If left untreated, it places newborns at risk for life-threatening health problems, including episodes of illness called metabolic crisis. Newborn screening for MSUD should ideally be done within the first 24 to 48 hours after birth. With proper screening, along with genetic counseling, nutritional counseling, primary care follow-up, and ongoing monitoring, newborns with MSUD can typically go on to live healthful lives. Nurses play a key role in supporting families with a diagnosis of MSUD. © 2017 AWHONN, the Association of Women’s Health, Obstetric and Neonatal Nurses.

Quantitative deviating effects of maple syrup extract supplementation on the hepatic gene expression of mice fed a high-fat diet.

PubMed

Kamei, Asuka; Watanabe, Yuki; Shinozaki, Fumika; Yasuoka, Akihito; Shimada, Kousuke; Kondo, Kaori; Ishijima, Tomoko; Toyoda, Tsudoi; Arai, Soichi; Kondo, Takashi; Abe, Keiko

2017-02-01

Maple syrup contains various polyphenols and we investigated the effects of a polyphenol-rich maple syrup extract (MSXH) on the physiology of mice fed a high-fat diet (HFD). The mice fed a low-fat diet (LFD), an HFD, or an HFD supplemented with 0.02% (002MSXH) or 0.05% MSXH (005MSXH) for 4 weeks. Global gene expression analysis of the liver was performed, and the differentially expressed genes were classified into three expression patterns; pattern A (LFD < HFD > 002MSXH = 005MSXH, LFD > HFD < 002MSXH = 005MSXH), pattern B (LFD < HFD = 002MSXH > 005MSXH, LFD > HFD = 002MSXH < 005MSXH), and pattern C (LFD < HFD > 002MSXH < 005MSXH, LFD > HFD < 002MSXH > 005MSXH). Pattern A was enriched in glycolysis, fatty acid metabolism, and folate metabolism. Pattern B was enriched in tricarboxylic acid cycle while pattern C was enriched in gluconeogenesis, cholesterol metabolism, amino acid metabolism, and endoplasmic reticulum stress-related event. Our study suggested that the effects of MSXH ingestion showed (i) dose-dependent pattern involved in energy metabolisms and (ii) reversely pattern involved in stress responses. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Chlorogenic acid oxidation-induced greening of sunflower butter cookies as a function of different sweeteners and storage conditions.

PubMed

Liang, Sihui; Were, Lilian M

2018-02-15

Sunflower butter use as an allergen-free alternative to tree and legume nut butter in baking is limited by chlorogenic acid induced greening that occurs at alkaline pH. Limited information is available on controlling this greening in a food matrix. This study examined how different liquid sweeteners and relative humidity influenced greening of sunflower butter cookies. Doughs had similar initial pH (7.52-7.66) which increased to 8.44-9.13 after baking as ranked: xylitol>maple syrup>corn syrup>honey>agave syrup. Cookies made with maple syrup had the highest moisture and greening corresponding with lowest free chlorogenic acid. The % greening followed the same trend as greening intensity, and was positively correlated (r=0.9101) with chlorogenic-lysine adduct content. Our findings provide an ingredient solution to controlling greening, as results demonstrate that greening can be promoted with high relative humidity storage, and use of high moisture and pH ingredients. Unwanted greening can be inhibited by simply changing the liquid sweetener. Copyright © 2017 Elsevier Ltd. All rights reserved.

The Minnesota Maple Series: Community-Generated Knowledge Delivered through an Extension Website

ERIC Educational Resources Information Center

Wilsey, David S.; Miedtke, Juile A.; Sagor, Eli

2012-01-01

Extension continuously seeks novel and effective approaches to outreach and education. The recent retirement of a longtime content specialist catalyzed members of University of Minnesota Extension's Forestry team to reflect on our instructional capacity (internal and external) and educational design in the realm of maple syrup production. We…

Imaging in Classic Form of Maple Syrup Urine Disease: A Rare Metabolic Central Nervous System

PubMed Central

Jain, Aditi; Jagdeesh, K.; Mane, Ranoji; Singla, Saurabh

2013-01-01

Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder of branched-chain amino acid metabolism. The condition gets its name from the distinctive sweet odour of affected infants’ urine. MSUD is caused by a deficiency of the branched-chain α-ketoacid dehydrogenase enzyme complex, leading to accumulation of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. Imaging is characterestized by MSUD oedema affecting the myelinated white matter. We present a neonate with classic type of MSUD and its imaging features on computed tomography, conventional magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance spectroscopy. PMID:24049754

Imaging in classic form of maple syrup urine disease: a rare metabolic central nervous system.

PubMed

Jain, Aditi; Jagdeesh, K; Mane, Ranoji; Singla, Saurabh

2013-04-01

Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder of branched-chain amino acid metabolism. The condition gets its name from the distinctive sweet odour of affected infants' urine. MSUD is caused by a deficiency of the branched-chain α-ketoacid dehydrogenase enzyme complex, leading to accumulation of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. Imaging is characterestized by MSUD oedema affecting the myelinated white matter. We present a neonate with classic type of MSUD and its imaging features on computed tomography, conventional magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance spectroscopy.

The use of botulinum toxin and epidural analgesia for the treatment of spasticity and pain in a patient with maple syrup urine disease

PubMed Central

Kaki, Abdullah M.; Arab, Abeer A.

2012-01-01

A 7-year-old boy, weighing 18 kg, was diagnosed with maple syrup urine disease (MSUD). He suffered from spasticity of the lower limbs and pain that did not respond to oral medications. Injections of botulinum toxin A (BTX-A) at 10 sites and epidural analgesia with 0.125% bupivacaine were used to treat spasticity with good results. We conclude that BTX-A combined with epidural analgesia may be a useful treatment option for incapacitating, painful spasticity related to MSUD. This treatment modality allowed a comprehensive rehabilitation program to be completed and it lasted longer than 9 months. PMID:22754448

Bibliography of Technical Publications and Papers, July 1974 - June 1975

DTIC Science & Technology

1975-07-01

N. E., D. E. WESTCOTT, K. R. JOHNSON, and J. A. WESTERLING. Replacement sugar syrup with high -fructoce syrup in imitation maple syrup . 75-72-FEL (FEL...1975). -:::--::----::c:-, J, J, MCMULLEN, D, P, BUTLER, W. K. CALHOUN. The influence of a high corn oil on rat serum and liver lipids... histories of inflow velocity and projected area. University of Minne- sota, Contract No. DAAG17-73-C-0174. 75-10-AHEL, May 1974 (AD A005 271). 74

Further investigation into maple syrup yields 3 new lignans, a new phenylpropanoid, and 26 other phytochemicals.

PubMed

Li, Liya; Seeram, Navindra P

2011-07-27

Maple syrup is made by boiling the sap collected from certain maple ( Acer ) species. During this process, phytochemicals naturally present in tree sap are concentrated in maple syrup. Twenty-three phytochemicals from a butanol extract of Canadian maple syrup (MS-BuOH) had previously been reported; this paper reports the isolation and identification of 30 additional compounds (1-30) from its ethyl acetate extract (MS-EtOAc) not previously reported from MS-BuOH. Of these, 4 compounds are new (1-3, 18) and 20 compounds (4-7, 10-12, 14-17, 19, 20, 22-24, 26, and 28-30) are being reported from maple syrup for the first time. The new compounds include 3 lignans and 1 phenylpropanoid: 5-(3″,4″-dimethoxyphenyl)-3-hydroxy-3-(4'-hydroxy-3'-methoxybenzyl)-4-(hydroxymethyl)dihydrofuran-2-one (1), (erythro,erythro)-1-[4-[2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3,5-dimethoxyphenyl]-1,2,3-propanetriol (2), (erythro,threo)-1-[4-[2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3,5-dimethoxyphenyl]-1,2,3-propanetriol (3), and 2,3-dihydroxy-1-(3,4- dihydroxyphenyl)-1-propanone (18), respectively. In addition, 25 other phenolic compounds were isolated including (threo,erythro)-1-[4-[(2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3-methoxyphenyl]-1,2,3-propanetriol (4), (threo,threo)-1-[4-[(2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3-methoxyphenyl]-1,2,3-propanetriol (5), threo-guaiacylglycerol-β-O-4'-dihydroconiferyl alcohol (6), erythro-1-(4-hydroxy-3-methoxyphenyl)-2-[4-(3-hydroxypropyl)-2,6-dimethoxyphenoxy]-1,3-propanediol (7), 2-[4-[2,3-dihydro-3-(hydroxymethyl)-5-(3-hydroxypropyl)-7-methoxy-2-benzofuranyl]-2,6-dimethoxyphenoxy]-1-(4-hydroxy-3-methoxyphenyl)-1,3-propanediol (8), acernikol (9), leptolepisol D (10), buddlenol E (11), (1S,2R)-2-[2,6-dimethoxy-4-[(1S,3aR,4S,6aR)-tetrahydro-4-(4-hydroxy-3,5-dimethoxyphenyl)-1H,3H-furo[3,4-c]furan-1-yl]phenoxy]-1-(4-hydroxy-3-methoxyphenyl)-1,3-propanediol (12), syringaresinol (13), isolariciresinol (14), icariside E4 (15), sakuraresinol (16), 1,2-diguaiacyl-1,3-propanediol (17), 2,3-dihydroxy-1-(4-hydroxy-3,5-dimethoxyphenyl)-1-propanone (19), 3-hydroxy-1-(4-hydroxy-3,5-dimethoxyphenyl)propan-1-one (20), dihydroconiferyl alcohol (21), 4-acetylcatechol (22), 3',4',5'-trihydroxyacetophenone (23), 3,4-dihydroxy-2-methylbenzaldehyde (24), protocatechuic acid (25), 4-(dimethoxymethyl)pyrocatechol (26), tyrosol (27), isofraxidin (28), and 4-hydroxycatechol (29). One sesquiterpene, phaseic acid (30), which is a known metabolite of the phytohormone abscisic acid, was also isolated from MS-EtOAc. The antioxidant activities of MS-EtOAc (IC(50) = 75.5 μg/mL) and the pure isolates (IC(50) ca. 68-3000 μM) were comparable to that of vitamin C (IC(50) = 40 μM) and the synthetic commercial antioxidant butylated hydroxytoluene (IC(50) = 3000 μM), in the diphenylpicrylhydrazyl radical scavenging assay. The current study advances scientific knowledge of maple syrup constituents and suggests that these diverse phytochemicals may impart potential health benefits to this natural sweetener.

Further Investigation Into Maple Syrup Yields Three New Lignans, a New Phenylpropanoid, and Twenty-Six Other Phytochemicals

PubMed Central

LI, LIYA; SEERAM, NAVINDRA P.

2011-01-01

Maple syrup is made by boiling the sap collected from certain maple (Acer) species. During this process, phytochemicals naturally present in tree sap are concentrated in maple syrup. We previously reported 23 phytochemicals from a butanol extract of Canadian maple syrup (MS-BuOH). Here we report the isolation and identification of 30 additional compounds (1–30) from its ethyl acetate extract (MS-EtOAc) not previously reported from MS-BuOH. Of these, 4 compounds are new (1–3, 18) and 20 compounds (4–7, 10–12, 14–17, 19–20, 22–24, 26, 28–30) are being reported from maple syrup for the first time. The new compounds include 3 lignans and 1 phenylpropanoid: 5-(3″,4″-dimethoxyphenyl)-3-hydroxy-3-(4′-hydroxy-3′-methoxybenzyl)-4-hydroxymethyl-dihydrofuran-2-one (1), (erythro, erythro)-1-[4-[2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3,5-dimethoxyphenyl]-1,2,3-propanetriol (2), (erythro, threo)-1-[4-[2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3,5-dimethoxyphenyl]-1,2,3-propanetriol (3) and 2,3-dihydroxy-1-(3,4-dihydroxyphenyl)-1-propanone (18), respectively. In addition, 25 other phenolic compounds were isolated including (threo, erythro)-1-[4-[(2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3-methoxyphenyl]-1,2,3-propanetriol (4), (threo, threo)-1-[4-[(2-hydroxy-2-(4-hydroxy-3-methoxyphenyl)-1-(hydroxymethyl)ethoxy]-3-methoxyphenyl]-1,2,3-propanetriol (5), threo-guaiacylglycerol-β-O-4′-dihydroconiferyl alcohol (6), erythro-1-(4-hydroxy-3-methoxyphenyl)-2-[4-(3-hydroxypropyl)-2,6-dimethoxyphenoxy]-1,3-propanediol (7), 2-[4-[2,3-dihydro-3-(hydroxymethyl)-5-(3-hydroxypropyl)-7-methoxy-2-benzofuranyl]-2,6-dimethoxyphenoxy]-1-(4-hydroxy-3-methoxyphenyl)-1,3-propanediol (8), acernikol (9), leptolepisol D (10), buddlenol E (11), (1S,2R)-2-[2,6-dimethoxy-4-[(1S,3aR,4S,6aR)-tetrahydro-4-(4-hydroxy-3,5-dimethoxyphenyl)-1H,3H-furo[3,4-c]furan-1-yl]phenoxy]-1-(4-hydroxy-3-methoxyphenyl)-1,3-propanediol (12), syringaresinol (13), isolariciresinol (14), icariside E4 (15), sakuraresinol (16), 1,2-diguaiacyl-1,3-propanediol (17), 2,3-dihydroxy-1-(4-hydroxy-3,5-dimethoxyphenyl)-1-propanone (19), 3-hydroxy-1-(4-hydroxy-3,5-dimethoxyphenyl)propan-1-one (20), dihydroconiferyl alcohol (21), 4-acetylcatechol (22), 3′,4′,5′-trihydroxyacetophenone (23), 3,4-dihydroxy-2-methylbenzaldehyde (24), protocatechuic acid (25), 4-(dimethoxymethyl)-pyrocatechol (26), tyrosol (27), isofraxidin (28) and 4-hydroxycatechol (29). One sesquiterpene, phaseic acid (30), which is a known metabolite of the phytohormone, abscisic acid, was also isolated from MS-EtOAc. The antioxidant activities of MS-EtOAc (IC50 = 75.5 μg/mL), and the pure isolates (IC50 ca. 68–3000 μM) were comparable to vitamin C (IC50 = 40 μM) and the synthetic commercial antioxidant, butylated hydroxytoluene (IC50 = 3000 μM), in the diphenylpicrylhydrazyl radical scavenging assay. The current study advances scientific knowledge of maple syrup constituents and suggest that these diverse phytochemicals may impart potential health benefits to this natural sweetener. PMID:21675726

29 CFR 780.815 - Basic conditions of exemption; second part, processing of sugar beets, sugar-beet molasses...

Code of Federal Regulations, 2010 CFR

2010-07-01

... sugar beets, sugar-beet molasses, sugarcane, or maple sap. 780.815 Section 780.815 Labor Regulations... Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup... Quantities § 780.815 Basic conditions of exemption; second part, processing of sugar beets, sugar-beet...

29 CFR 780.815 - Basic conditions of exemption; second part, processing of sugar beets, sugar-beet molasses...

Code of Federal Regulations, 2011 CFR

2011-07-01

... sugar beets, sugar-beet molasses, sugarcane, or maple sap. 780.815 Section 780.815 Labor Regulations... Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup... Quantities § 780.815 Basic conditions of exemption; second part, processing of sugar beets, sugar-beet...

29 CFR 780.815 - Basic conditions of exemption; second part, processing of sugar beets, sugar-beet molasses...

Code of Federal Regulations, 2014 CFR

2014-07-01

... sugar beets, sugar-beet molasses, sugarcane, or maple sap. 780.815 Section 780.815 Labor Regulations... Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup... Quantities § 780.815 Basic conditions of exemption; second part, processing of sugar beets, sugar-beet...

29 CFR 780.815 - Basic conditions of exemption; second part, processing of sugar beets, sugar-beet molasses...

Code of Federal Regulations, 2013 CFR

2013-07-01

... sugar beets, sugar-beet molasses, sugarcane, or maple sap. 780.815 Section 780.815 Labor Regulations... Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup... Quantities § 780.815 Basic conditions of exemption; second part, processing of sugar beets, sugar-beet...

29 CFR 780.815 - Basic conditions of exemption; second part, processing of sugar beets, sugar-beet molasses...

Code of Federal Regulations, 2012 CFR

2012-07-01

... sugar beets, sugar-beet molasses, sugarcane, or maple sap. 780.815 Section 780.815 Labor Regulations... Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup... Quantities § 780.815 Basic conditions of exemption; second part, processing of sugar beets, sugar-beet...

Tapholes in sugar maples: What happens in the tree.

Treesearch

Russell S. Walters; Alex L. Shigo

1978-01-01

Maple syrup production starts by drilling a taphole in the tree. This process injures the wood, which may become discolored or decayed as a result. If trees are to be tapped, every effort must be made to minimize injury while obtaining the desired amount of sap. Information about tapholes is given here for the benefit of the producer. Some important points discussed...

Some effects of paraformaldehyde on wood surrounding tapholes in sugar maple trees

Treesearch

Alex L. Shigo; Frederick M. Laing; Frederick M. Laing

1970-01-01

Pills of paraformaldehyde (trioxymethylene) are commonly used in tapholes in sugar maple trees (Acer saccharam Marsh.) to increase the yield of sap collected for making syrup and sugar. The explanation offered for this increase in sap yield is that microorganisms in the tapholes cause premature decline and stoppage of the sap flow, but that paraformaldehyde increases...

Molecular characterization of maple syrup urine disease patients from Tunisia.

PubMed

Jaafar, N; Moleirinho, A; Kerkeni, E; Monastiri, K; Seboui, H; Amorim, A; Prata, M J; Quental, S

2013-03-15

Maple syrup urine disease (MSUD) is a rare disorder of branched-chain amino acids (BCAA) metabolism caused by the defective function of branched-chain α-ketoacid dehydrogenase complex (BCKD). The disease causal mutations can occur either in BCKDHA, BCKDHB or DBT genes encoding respectively the E1α, E1β and E2 subunits of the complex. In this study we report the molecular characterization of 3 Tunisian patients with the classic form of MSUD. Two novel putative mutations have been identified: the alteration c.716A>G (p.Glu239Gly) in BCKDHB and a small deletion (c.1333_1336delAATG; p.Asn445X) detected in DBT gene. Copyright © 2012 Elsevier B.V. All rights reserved.

Intermittent maple syrup urine disease: two case reports.

PubMed

Axler, Olof; Holmquist, Peter

2014-02-01

The presenting symptoms and clinical course of 2 cases of intermittent maple syrup urine disease (MSUD) are described. Intermittent MSUD is a potentially life-threatening metabolic disorder caused by a deficiency of branched-chain α-keto acid dehydrogenase, the enzyme complex that decarboxylates the 3 branched-chain amino acids. In contrast to classic MSUD, children with the intermittent form show normal development with normal intelligence and, when asymptomatic, normal levels of branched-chain amino acids. Symptoms usually appear between 5 months and 2 years of age, when a trivial infection such as otitis media or viral gastroenteritis triggers catabolism of muscle protein. Intermittent MSUD should be suspected in cases of common infections with a clinically atypical course, especially in children displaying ataxia or marked drowsiness.

DNA damage in an animal model of maple syrup urine disease.

PubMed

Scaini, Giselli; Jeremias, Isabela C; Morais, Meline O S; Borges, Gabriela D; Munhoz, Bruna P; Leffa, Daniela D; Andrade, Vanessa M; Schuck, Patrícia F; Ferreira, Gustavo C; Streck, Emilio L

2012-06-01

Maple syrup urine disease is an inborn error of metabolism caused by a severe deficiency of the branched chain alpha-ketoacid dehydrogenase complex. Neurological dysfunction is a common finding in patients with maple syrup urine disease. However, the mechanisms underlying the neuropathology of brain damage in this disorder are poorly understood. In this study, we investigated whether acute or chronic administration of a branched chain amino acid pool (leucine, isoleucine and valine) causes transient DNA damage, as determined by the alkaline comet assay, in the brain and blood of rats during development and whether antioxidant treatment prevented the alterations induced by branched chain amino acids. Our results showed that the acute administration of branched chain amino acids increased the DNA damage frequency and damage index in the hippocampus. However, the chronic administration of branched chain amino acids increased the DNA damage frequency and damage index in both the hippocampus and the striatum, and the antioxidant treatment was able to prevent DNA damage in the hippocampus and striatum. The present study demonstrated that metabolite accumulation in MSUD induces DNA damage in the hippocampus and striatum and that it may be implicated in the neuropathology observed in the affected patients. We demonstrated that the effect of antioxidant treatment (N-acetylcysteine plus deferoxamine) prevented DNA damage, suggesting the involvement of oxidative stress in DNA damage. Copyright © 2012 Elsevier Inc. All rights reserved.

Impacts of Climate Change on the Timing of the Production Season of Maple Syrup in Eastern Canada

PubMed Central

Côté, Benoît; Logan, Travis; Power, Hugues; Charron, Isabelle; Duchesne, Louis

2015-01-01

Maple syrup production is an important economic activity in north-eastern North-America. The beginning and length of the production season is linked to daily variation in temperature. There are increasing concerns about the potential impact of climatic change on this industry. Here, we used weekly data of syrup yield for the 1999–2011 period from 121 maple stands in 11 regions of Québec (Canada) to predict how the period of production may be impacted by climate warming. The date at which the production begins is highly variable between years with an average range of 36 days among the regions. However, the average start date for a given region, which ranged from Julian day 65 to 83, was highly predictable (r2 = 0.88) using the average temperature from January to April (TJ-A). A logistic model predicting the weekly presence or absence of production was also developed. Using the inputs of 77 future climate scenarios issued from global models, projections of future production timing were made based on average TJ-A and on the logistic model. The projections of both approaches were in very good agreement and suggest that the sap season will be displaced to occur 15–19 days earlier on average in the 2080–2100 period. The data also show that the displacement in time will not be accompanied by a greater between years variability in the beginning of the season. However, in the southern part of Québec, very short periods of syrup production due to unfavourable conditions in the spring will occur more frequently in the future although their absolute frequencies will remain low. PMID:26682889

Impacts of Climate Change on the Timing of the Production Season of Maple Syrup in Eastern Canada.

PubMed

Houle, Daniel; Paquette, Alain; Côté, Benoît; Logan, Travis; Power, Hugues; Charron, Isabelle; Duchesne, Louis

2015-01-01

Maple syrup production is an important economic activity in north-eastern North-America. The beginning and length of the production season is linked to daily variation in temperature. There are increasing concerns about the potential impact of climatic change on this industry. Here, we used weekly data of syrup yield for the 1999-2011 period from 121 maple stands in 11 regions of Québec (Canada) to predict how the period of production may be impacted by climate warming. The date at which the production begins is highly variable between years with an average range of 36 days among the regions. However, the average start date for a given region, which ranged from Julian day 65 to 83, was highly predictable (r2 = 0.88) using the average temperature from January to April (TJ-A). A logistic model predicting the weekly presence or absence of production was also developed. Using the inputs of 77 future climate scenarios issued from global models, projections of future production timing were made based on average TJ-A and on the logistic model. The projections of both approaches were in very good agreement and suggest that the sap season will be displaced to occur 15-19 days earlier on average in the 2080-2100 period. The data also show that the displacement in time will not be accompanied by a greater between years variability in the beginning of the season. However, in the southern part of Québec, very short periods of syrup production due to unfavourable conditions in the spring will occur more frequently in the future although their absolute frequencies will remain low.

Comparison of nonfried apple snacks with commercially available fried snacks.

PubMed

Joshi, A P K; Rupasinghe, H P V; Pitts, N L

2011-06-01

The study was carried out to evaluate the selected quality attributes of a prototype nonfried apple snack produced by application of vacuum impregnation (VI) of maple syrup and vacuum drying. When maple syrup concentration was adjusted to 20-40% in the VI solution, vacuum-dried apple slices are resulted in the greatest textural attributes, whiteness index, and desirable moisture content and water activity. Comparison of the VI-treated, vacuum-dried apple slices with commercially fried apple and potato snacks revealed that the consumer acceptability was greater for the fried snack products due to their flavor and texture; however, in addition to higher oil content (>30%), commercial fried apple and potato snacks possessed lower antioxidant capacity than nonfried apple snacks. VI process enhanced the calcium content of the nonfried apple snack products.

Imaging findings of anaplastic astrocytoma in a child with maple syrup urine disease: a case report.

PubMed

Aw-Zoretic, Jessie; Wadhwani, Nitin R; Lulla, Rishi R; Rishi, Lulla R; Ryan, Maura E

2015-09-01

Maple syrup urine disease (MSUD) is an inborn error of branched-chain amino acid metabolism, which usually presents in childhood with encephalopathy due to cerebral edema and dysmyelination. Even with treatment, metabolic stressors may precipitate later episodes of acute decompensation. Changes related to cerebral and white matter edema have been described by magnetic resonance imaging (MRI), and imaging can aid in both initial diagnosis and evaluation of decompensation. To date, there are no published known reports of cancer in patients with MSUD. Here, we present the first case report of an anaplastic astrocytoma in a teenager with MSUD, with a discussion of imaging findings and the use of magnetic resonance spectroscopy (MRS) to help distinguish between tumor and metabolic changes.

Limitations in the use of ozone to disinfect maple sap.

PubMed

Labbe, R G; Kinsley, M; Wu, J

2001-01-01

The sap of the maple sugar tree (Acer saccharum) contains 2 to 3% sucrose and is traditionally collected early in the year and concentrated by boiling to produce maple syrup. High levels of microorganisms in the sap occur during holding, leading to a darker syrup with lower economic value. We investigated the use of dissolved ozone as a method to reduce the microbial population in sap. After 40 min of ozone treatment, concentrations of up to 0.30 mg/liter were achieved but were ineffective in reducing the aerobic plate count. Three predominant colonies on nutrient agar were selected for isolation and identification from sap. These included one mucoid and one nonmucoid yeast, both identified as Candida, and Pseudomonas fluorescens. When suspended in buffer, each was readily inactivated by ozone. Addition of 3% sucrose to the buffer markedly reduced the effectiveness of ozone. With the use of an ozone generator with a larger ozone output, saturating ozone concentrations (1 mg/liter) were achieved within 5 min but were accompanied by only a 1-log reduction in aerobic plate count of maple sap. After 40 min of ozone treatment, a less than 3-log reduction occurred. The results indicate that, because of the presence of sucrose, ozone may be of limited use in reducing the microbial population in sap.

Phenylbutyrate therapy for maple syrup urine disease

PubMed Central

Brunetti-Pierri, Nicola; Lanpher, Brendan; Erez, Ayelet; Ananieva, Elitsa A.; Islam, Mohammad; Marini, Juan C.; Sun, Qin; Yu, Chunli; Hegde, Madhuri; Li, Jun; Wynn, R. Max; Chuang, David T.; Hutson, Susan; Lee, Brendan

2011-01-01

Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been associated with a selective reduction in branched-chain amino acids (BCAA) in spite of adequate dietary protein intake. Based on this clinical observation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresponding α-keto acids (BCKA) in patients with classic and variant late-onset forms of maple syrup urine disease (MSUD). We also performed in vitro and in vivo experiments to elucidate the mechanism for this effect. We found that BCAA and BCKA are both significantly reduced following phenylbutyrate therapy in control subjects and in patients with late-onset, intermediate MSUD. In vitro treatment with phenylbutyrate of control fibroblasts and lymphoblasts resulted in an increase in the residual enzyme activity, while treatment of MSUD cells resulted in the variable response which did not simply predict the biochemical response in the patients. In vivo phenylbutyrate increases the proportion of active hepatic enzyme and unphosphorylated form over the inactive phosphorylated form of the E1α subunit of the branched-chain α-keto acid dehydrogenase complex (BCKDC). Using recombinant enzymes, we show that phenylbutyrate prevents phosphorylation of E1α by inhibition of the BCKDC kinase to activate BCKDC overall activity, providing a molecular explanation for the effect of phenylbutyrate in a subset of MSUD patients. Phenylbutyrate treatment may be a valuable treatment for reducing the plasma levels of neurotoxic BCAA and their corresponding BCKA in a subset of MSUD patients and studies of its long-term efficacy are indicated. PMID:21098507

Phenylbutyrate therapy for maple syrup urine disease.

PubMed

Brunetti-Pierri, Nicola; Lanpher, Brendan; Erez, Ayelet; Ananieva, Elitsa A; Islam, Mohammad; Marini, Juan C; Sun, Qin; Yu, Chunli; Hegde, Madhuri; Li, Jun; Wynn, R Max; Chuang, David T; Hutson, Susan; Lee, Brendan

2011-02-15

Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been associated with a selective reduction in branched-chain amino acids (BCAA) in spite of adequate dietary protein intake. Based on this clinical observation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresponding α-keto acids (BCKA) in patients with classic and variant late-onset forms of maple syrup urine disease (MSUD). We also performed in vitro and in vivo experiments to elucidate the mechanism for this effect. We found that BCAA and BCKA are both significantly reduced following phenylbutyrate therapy in control subjects and in patients with late-onset, intermediate MSUD. In vitro treatment with phenylbutyrate of control fibroblasts and lymphoblasts resulted in an increase in the residual enzyme activity, while treatment of MSUD cells resulted in the variable response which did not simply predict the biochemical response in the patients. In vivo phenylbutyrate increases the proportion of active hepatic enzyme and unphosphorylated form over the inactive phosphorylated form of the E1α subunit of the branched-chain α-keto acid dehydrogenase complex (BCKDC). Using recombinant enzymes, we show that phenylbutyrate prevents phosphorylation of E1α by inhibition of the BCKDC kinase to activate BCKDC overall activity, providing a molecular explanation for the effect of phenylbutyrate in a subset of MSUD patients. Phenylbutyrate treatment may be a valuable treatment for reducing the plasma levels of neurotoxic BCAA and their corresponding BCKA in a subset of MSUD patients and studies of its long-term efficacy are indicated.

Maple syrup urine disease

MedlinePlus

... Kliegman RM, Stanton BF, St. Geme JW, Schor NF. Defects in metabolism of amino acids. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, ...

Co-infection with coxsackievirus A5 and norovirus GII.4 could have been the trigger of the first episode of severe acute encephalopathy in a six-year-old child with the intermittent form of maple syrup urine disease (MSUD).

PubMed

Boros, Ákos; Pankovics, Péter; Kőmíves, Sándor; Liptai, Zoltán; Dobner, Sarolta; Ujhelyi, Enikő; Várallyay, György; Zsidegh, Petra; Bolba, Nóra; Reuter, Gábor

2017-06-01

In this case study, a co-infection with coxsackievirus A5 (family Picornaviridae) and norovirus GII.4 (family Caliciviridae) was detected by RT-PCR in a faecal sample from a six-year-old girl with symptoms of severe acute encephalopathy subsequently diagnosed as the intermittent form of maple syrup urine disease (MSUD). The two co-infecting viruses, which had been detected previously, appeared to have triggered the underlying metabolic disorder. Here, we describe the genotyping of the viruses, as well as the chronological course, laboratory test results, and clinical presentation of this case, which included recurrent vomiting without diarrhoea, metabolic acidosis, unconsciousness, seizure and circulatory collapse, but with a positive final outcome.

A Whole-Cell Surface Plasmon Resonance Sensor Based on a Leucine Auxotroph of Escherichia coli Displaying a Gold-Binding Protein: Usefulness for Diagnosis of Maple Syrup Urine Disease.

PubMed

Woo, Min-Ah; Park, Jung Hun; Cho, Daeyeon; Sim, Sang Jun; Kim, Moon Il; Park, Hyun Gyu

2016-03-01

We developed a whole-cell surface plasmon resonance (SPR) sensor based on a leucine auxotroph of Escherichia coli displaying a gold-binding protein (GBP) in response to cell growth and applied this sensor to the diagnosis of maple syrup urine disease, which is represented by the elevated leucine level in blood. The leucine auxotroph was genetically engineered to grow displaying GBP in a proportion to the concentration of target amino acid leucine. The GBP expressed on the surface of the auxotrophs directly bound to the golden surface of an SPR chip without the need for any additional treatment or reagents, which consequently produced SPR signals used to determine leucine levels in a test sample. Gold nanoparticles (GNPs) were further applied to the SPR system, which significantly enhanced the signal intensity up to 10-fold by specifically binding to GBP expressed on the cell surface. Finally, the diagnostic utility of our system was demonstrated by its employment in reliably determining different statuses of maple syrup urine disease based on a known cutoff level of leucine. This new approach based on an amino acid-auxotrophic E. coli strain expressing a GBP that binds to an SPR sensor holds great promise for detection of other metabolic diseases of newborn babies including homocystinuria and phenylketonuria, which are also associated with abnormal levels of amino acids.

Cost of Maple Sap Production for Various Size Tubing Operations

Treesearch

Niel K. Huyler

2000-01-01

Reports sap production costs for small (500 to 1,000 taps), medium (1,000 to 5,000), and large (5,000 to 15,000) maple syrup operations that use plastic tubing with vacuum pumping. The average annual operating cost per tap ranged from $4.64 for a 500-tap sugarbush operation to $1.84 for a sugarbush with 10,000 taps. The weighted average was $2.87 per tap or $11.48 per...

Remarks on the physiological effects of defoliation on sugar maple and some impacts on syrup production

Treesearch

Philip M. Wargo

1991-01-01

The information I am going to present today is a conglomeration of some of the research on the effects of defoliation that has been done on sugar maple and oak. It involves work done by Drs. David Houston, Johnson Parker, Robert Gregory and me. Dr. Houston, a plant pathologist, and Dr. Parker, a plant physiologist, work with the USDA Forest Service in Hamden, Ct.; Dr....

29 CFR 780.800 - Scope and significance of interpretative bulletin.

Code of Federal Regulations, 2011 CFR

2011-07-01

... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane..., sugar-beet molasses, sugarcane or maple sap, into sugar (other than refined sugar) or syrup. The limited...

29 CFR 780.800 - Scope and significance of interpretative bulletin.

Code of Federal Regulations, 2010 CFR

2010-07-01

... STANDARDS ACT Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane..., sugar-beet molasses, sugarcane or maple sap, into sugar (other than refined sugar) or syrup. The limited...

Genetics Home Reference: maple syrup urine disease

MedlinePlus

... disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants' urine. It is also characterized ...

[GENETIC AND METABOLIC URGENCIES IN THE NEONATAL INTENSIVE CARE UNIT: MAPLE SYRUP URINE DISEASE].

PubMed

Páez Rojas, Paola Liliana; Suarez Obando, Fernando

2015-07-01

Maple syrup urine disease (MSUD) is a hereditary disorder of branched chain amino/keto acid metabolism, caused by a decreased activity of the branched-chain alpha- ketoacid dehydrogenase complex (BCKAD), which leads to abnormal elevated plasma concentrations of branched-chain amino acids (BCAAs) clinically manifested as a heavy burden for Central Nervous system. The toxic accumulation of substrates promotes the development of a severe and rapidly progressive neonatal encephalopathy if treatment is not immediately given. This disorder has a specific medical management in acute phase in order to minimize mortality and morbidity. For all those reasons, it is important to include the MSUD as a possible diagnosis in a encephalopathic newborn. We present a colombian newborn with classical MSUD with fatal outcome as an example of metabolic emergency and a differential diagnosis in the encephalopathic newborn. Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.

The management of pregnancy in maple syrup urine disease: experience with two patients.

PubMed

Tchan, Michel; Westbrook, M; Wilcox, G; Cutler, R; Smith, N; Penman, R; Strauss, B J G; Wilcken, B

2013-01-01

We describe the management and outcomes of pregnancy in two women affected with Maple syrup urine disease (MSUD). Both patients had classical disease diagnosed in the newborn period and were managed with low-protein diets and supplements, although compliance was moderately poor throughout life. Both pregnancies were complicated by poor compliance and one patient had a metabolic decompensation, which included seizures and profound encephalopathy, at the end of the first trimester. Peri-partum management required a coordinated team approach including a high-calorie and low-protein diet. Both patients had elevated leucine levels in the post-partum period - one due to mastitis and the other due to poor dietary and supplement compliance combined with uterine involution. On later review, leucine had returned to pre-pregnancy levels. Both infants were unaffected and have made normal developmental progress in the subsequent 1 to 2 years.

Acute Illness Protocol for Maple Syrup Urine Disease.

PubMed

Rodan, Lance H; Aldubayan, Saud H; Berry, Gerard T; Levy, Harvey L

2018-01-01

Inborn errors of metabolism (IEMs) are genetic disorders that disrupt enzyme activity, cellular transport, or energy production. They are individually rare but collectively have an incidence of 1:1000. Most patients with IEMs are followed up by a physician with expertise in biochemical genetics (metabolism), but may present outside this setting. Because IEMs can present acutely with life-threatening crises that require specific interventions, it is critical for the emergency medicine physician, pediatrician, internist, and critical care physician as well as the biochemical geneticist to have information on the initial assessment and management of patients with these disorders. Appropriate early care can be lifesaving. This protocol is not designed to replace the expert consultation of a biochemical geneticist, but rather to improve early care and increase the level of comfort of the acute care physician with initial management of maple syrup urine disease until specialty consultation is obtained.

Two consecutive partial liver transplants in a patient with Classic Maple Syrup Urine Disease☆☆☆

PubMed Central

Chin, H.L.; Aw, M.M.; Quak, S.H.; Huang, J.; Hart, C.E.; Prabhakaran, K.; Goh, D.L.

2015-01-01

Maple syrup urine disease is caused by a deficiency in the branched chain ketoacid dehydrogenase (BCKAD) complex. This results in the accumulation of branched chain amino acids (BCAA) and branched chain ketoacids in the body. Even when aggressively treated with dietary restriction of BCAA, patients experience long term cognitive, neurological and psychosocial problems. Liver transplantation from deceased donors has been shown to be an effective modality in introducing adequate BCKAD activity, attaining a metabolic cure for patients. Here, we report the clinical course of the first known patient with classic MSUD who received two consecutive partial liver grafts from two different living non-carrier donors and his five year outcome posttransplant. We also show that despite the failure of the first liver graft, and initial acute cellular rejection of the second liver graft in our patient, his metabolic control remained good without metabolic decompensation. PMID:26937410

Integration of targeted sequencing and NIPT into clinical practice in a Chinese family with maple syrup urine disease.

PubMed

You, Yanqin; Sun, Yan; Li, Xuchao; Li, Yali; Wei, Xiaoming; Chen, Fang; Ge, Huijuan; Lan, Zhangzhang; Zhu, Qian; Tang, Ying; Wang, Shujuan; Gao, Ya; Jiang, Fuman; Song, Jiaping; Shi, Quan; Zhu, Xuan; Mu, Feng; Dong, Wei; Gao, Vince; Jiang, Hui; Yi, Xin; Wang, Wei; Gao, Zhiying

2014-08-01

This article demonstrates a prominent noninvasive prenatal approach to assist the clinical diagnosis of a single-gene disorder disease, maple syrup urine disease, using targeted sequencing knowledge from the affected family. The method reported here combines novel mutant discovery in known genes by targeted massively parallel sequencing with noninvasive prenatal testing. By applying this new strategy, we successfully revealed novel mutations in the gene BCKDHA (Ex2_4dup and c.392A>G) in this Chinese family and developed a prenatal haplotype-assisted approach to noninvasively detect the genotype of the fetus (transmitted from both parents). This is the first report of integration of targeted sequencing and noninvasive prenatal testing into clinical practice. Our study has demonstrated that this massively parallel sequencing-based strategy can potentially be used for single-gene disorder diagnosis in the future.

Two consecutive partial liver transplants in a patient with Classic Maple Syrup Urine Disease.

PubMed

Chin, H L; Aw, M M; Quak, S H; Huang, J; Hart, C E; Prabhakaran, K; Goh, D L

2015-09-01

Maple syrup urine disease is caused by a deficiency in the branched chain ketoacid dehydrogenase (BCKAD) complex. This results in the accumulation of branched chain amino acids (BCAA) and branched chain ketoacids in the body. Even when aggressively treated with dietary restriction of BCAA, patients experience long term cognitive, neurological and psychosocial problems. Liver transplantation from deceased donors has been shown to be an effective modality in introducing adequate BCKAD activity, attaining a metabolic cure for patients. Here, we report the clinical course of the first known patient with classic MSUD who received two consecutive partial liver grafts from two different living non-carrier donors and his five year outcome posttransplant. We also show that despite the failure of the first liver graft, and initial acute cellular rejection of the second liver graft in our patient, his metabolic control remained good without metabolic decompensation.

Brain–blood amino acid correlates following protein restriction in murine maple syrup urine disease

PubMed Central

2014-01-01

Background Conventional therapy for patients with maple syrup urine disease (MSUD) entails restriction of protein intake to maintain acceptable levels of the branched chain amino acid, leucine (LEU), monitored in blood. However, no data exists on the correlation between brain and blood LEU with protein restriction, and whether correction in blood is reflected in brain. Methods To address this question, we fed intermediate MSUD mice diets of 19% (standard) and 6% protein, with collection of sera (SE), striata (STR), cerebellum (CE) and cortex (CTX) for quantitative amino acid analyses. Results LEU and valine (VAL) levels in all brain regions improved on average 28% when shifting from 19% to 6% protein, whereas the same improvements in SE were on average 60%. Isoleucine (ILE) in brain regions did not improve, while the SE level improved 24% with low-protein consumption. Blood-branched chain amino acids (LEU, ILE, and VAL in sera (SE)) were 362-434 μM, consistent with human values considered within control. Nonetheless, numerous amino acids in brain regions remained abnormal despite protein restriction, including glutamine (GLN), aspartate (ASP), glutamate (GLU), gamma-aminobutyric acid (GABA), asparagine (ASN), citrulline (CIT) and serine (SER). To assess the specificity of these anomalies, we piloted preliminary studies in hyperphenylalaninemic mice, modeling another large neutral aminoacidopathy. Employing an identical dietary regimen, we found remarkably consistent abnormalities in GLN, ASP, and GLU. Conclusions Our results suggest that blood amino acid analysis may be a poor surrogate for assessing the outcomes of protein restriction in the large neutral amino acidopathies, and further indicate that chronic neurotransmitter disruptions (GLU, GABA, ASP) may contribute to long-term neurocognitive dysfunction in these disorders. PMID:24886632

Brain-blood amino acid correlates following protein restriction in murine maple syrup urine disease.

PubMed

Vogel, Kara R; Arning, Erland; Wasek, Brandi L; McPherson, Sterling; Bottiglieri, Teodoro; Gibson, K Michael

2014-05-08

Conventional therapy for patients with maple syrup urine disease (MSUD) entails restriction of protein intake to maintain acceptable levels of the branched chain amino acid, leucine (LEU), monitored in blood. However, no data exists on the correlation between brain and blood LEU with protein restriction, and whether correction in blood is reflected in brain. To address this question, we fed intermediate MSUD mice diets of 19% (standard) and 6% protein, with collection of sera (SE), striata (STR), cerebellum (CE) and cortex (CTX) for quantitative amino acid analyses. LEU and valine (VAL) levels in all brain regions improved on average 28% when shifting from 19% to 6% protein, whereas the same improvements in SE were on average 60%. Isoleucine (ILE) in brain regions did not improve, while the SE level improved 24% with low-protein consumption. Blood-branched chain amino acids (LEU, ILE, and VAL in sera (SE)) were 362-434 μM, consistent with human values considered within control. Nonetheless, numerous amino acids in brain regions remained abnormal despite protein restriction, including glutamine (GLN), aspartate (ASP), glutamate (GLU), gamma-aminobutyric acid (GABA), asparagine (ASN), citrulline (CIT) and serine (SER). To assess the specificity of these anomalies, we piloted preliminary studies in hyperphenylalaninemic mice, modeling another large neutral aminoacidopathy. Employing an identical dietary regimen, we found remarkably consistent abnormalities in GLN, ASP, and GLU. Our results suggest that blood amino acid analysis may be a poor surrogate for assessing the outcomes of protein restriction in the large neutral amino acidopathies, and further indicate that chronic neurotransmitter disruptions (GLU, GABA, ASP) may contribute to long-term neurocognitive dysfunction in these disorders.

Maple Syrup Urine Disease in Cypriot Families: Identification of Three Novel Mutations and Biochemical Characterization of the p.Thr211Met Mutation in the E1α Subunit

PubMed Central

Georgiou, Theodoros; Chuang, Jacinta L.; Wynn, R. Max; Stylianidou, Goula; Korson, Mark; Chuang, David T.

2009-01-01

We report five mutations, three of them novel, responsible for maple syrup urine disease in four unrelated Cypriot families. The five children studied are the first cases of classic maple syrup urine disease to be reported among Cypriots. The first novel mutation identified is a single-base deletion in exon 6 of the Elα gene (c.718delG), which leads to a frameshift after Ala240 and to a stop codon 89 residues further downstream. The other two novel mutations identified are in the Elβ subunit: a two-base deletion in exon 6, c.662_663delCC, which leads to a frameshift after Ala221 and creates a stop codon 17 residues further downstream, as well as a splice mutation, IVS3[+3]delA, which results in the skipping of exon 3. The two known mutations identified are in the Elα gene: the G > C transversion at the 3′-splice acceptor site, (IVS5-1G > C), which results in the deletion of the entire exon 6, and the missense mutation in exon 5 (c.632C > T), which corresponds to a p.Thr211Met substitution. The p.Thr211Met substitution is located in a potassium-ion pocket in the E1 component required for stability of the bound cofactor thiamine diphosphate. The mutant E1 protein harboring the p.Thr211Met substitution was shown unable to bind thiamine diphosphate, leading to undetectable E1 activity. PMID:19715473

9 CFR 205.206 - Farm products.

Code of Federal Regulations, 2012 CFR

2012-01-01

... specify by name) Dry beans, dry peas, potatoes, sweet potatoes, taro, other vegetables (system must..., carrots, cauliflower, celery, corn sweet, cucumbers, eggplant, escarole, garlic, lettuce, onions, peas... nuts (system must specify by name) Bees wax, honey, maple syrup, sugar beets, sugar cane, other sugar...

Changing carbon isotope ratio of atmospheric carbon dioxide: implications for food authentication.

PubMed

Peck, William H; Tubman, Stephanie C

2010-02-24

Carbon isotopes are often used to detect the addition of foreign sugars to foods. This technique takes advantage of the natural difference in carbon isotope ratio between C(3) and C(4) plants. Many foods are derived from C(3) plants, but the low-cost sweeteners corn and sugar cane are C(4) plants. Most adulteration studies do not take into account the secular shift of the carbon isotope ratio of atmospheric carbon dioxide caused by fossil fuel burning, a shift also seen in plant tissues. As a result statistical tests and threshold values that evaluate authenticity of foods based on carbon isotope ratios may need to be corrected for changing atmospheric isotope values. Literature and new data show that the atmospheric trend in carbon isotopes is seen in a 36-year data set of maple syrup analyses (n = 246), demonstrating that published thresholds for cane or corn sugar adulteration in maple syrup (and other foods) have become progressively more lenient over time.

A new missense mutation in the BCKDHB gene causes the classic form of maple syrup urine disease (MSUD).

PubMed

Miryounesi, Mohammad; Ghafouri-Fard, Soudeh; Goodarzi, Hamedreza; Fardaei, Majid

2015-05-01

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disease caused by mutations in the BCKDHA, BCKDHB, DBT and DLD genes, which encode the E1α, E1β, E2 and E3 subunits of the branched chain α ketoacid dehydrogenase (BCKD) complex, respectively. This complex is involved in the metabolism of branched-chain amino acids. In this study, we analyzed the DNA sequences of BCKDHA and BCKDHB genes in an infant who suffered from MSUD and died at the age of 6 months. We found a new missense mutation in exon 5 of BCKDHB gene (c.508C>T). The heterozygosity of the parents for the mentioned nucleotide change was confirmed by direct sequence analysis of the corresponding segment. Another missense mutation has been found in the same codon previously and shown by in silico analyses to be deleterious. This report provides further evidence that this amino acid change can cause classic MSUD.

Acrodermatitis dysmetabolica in an infant with maple syrup urine disease.

PubMed

Flores, K; Chikowski, R; Morrell, D S

2016-08-01

Acrodermatitis dysmetabolica (AD) is a rare, newly termed, and poorly understood disease that appears to be clinically similar to acrodermatitis enteropathica (AE). Both diseases are characterized by the triad of periorificial and acral dermatitis, diarrhoea, and alopecia. Unlike AE, which is caused by zinc deficiency, AD is caused by numerous metabolic disorders. One such disorder is maple syrup urine disease (MSUD), a genetic deficiency of branched chain α-ketoacid dehydrogenase, the enzyme that degrades the branched-chain amino acids (BCAAs) isoleucine, leucine and valine. Treatment involves restricting BCAAs to prevent accumulation. We report a case of an infant being treated for MSUD, who developed the triad of AE/AD after a period of poor BCAA formula intake. The child was found to have low isoleucine and normal zinc levels. Increasing the isoleucine dose improved the eruption, thus the diagnosis of AD secondary to isoleucine deficiency was made. This case emphasizes the importance of carefully balancing BCAA levels while treating MSUD, as deficiency can precipitate AD. © 2016 British Association of Dermatologists.

Ultra-fast liquid chromatography coupled with electrospray ionization time-of-flight mass spectrometry for the rapid phenolic profiling of red maple (Acer rubrum) leaves.

PubMed

Li, Chunting; Seeram, Navindra P

2018-03-07

The red maple (Acer rubrum) species is economically important to North America because of its sap, which is used to produce maple syrup. In addition, various other red maple plant parts, including leaves, were used as a traditional medicine by the Native Americans. Currently, red maple leaves are being used for nutraceutical and cosmetic applications but there are no published analytical methods for comprehensive phytochemical characterization of this material. Herein, a rapid and sensitive method using liquid chromatography with electrospray ionization time-of-flight tandem mass spectrometry was developed to characterize the phenolics in a methanol extract of red maple leaves and a proprietary phenolic-enriched red maple leaves extract (Maplifa™). Time-of-flight mass spectrometry and tandem mass spectrometry experiments led to the identification of 106 phenolic compounds in red maples leaves with the vast majority of these compounds also detected in Maplifa™. The compounds included 68 gallotannins, 25 flavonoids, gallic acid, quinic acid, catechin, epicatechin, and nine other gallic acid derivatives among which 11 are potentially new and 75 are being reported from red maple for the first time. The developed method to characterize red maple leaves phenolics is rapid and highly sensitive and could aid in future standardization and quality control of this botanical ingredient. © 2018 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

The Blackout of Native American Cultural Achievements.

ERIC Educational Resources Information Center

Vogel, Virgil J.

1987-01-01

Notes that American Indian achievements are overlooked and even specifically denied in the face of overwhelming evidence of their reality. Examines the denials of Indian originality in three specific matters that have been controversial in recent decades: medicine, the manufacture of maple syrup, and the use of fertilizer. (JHZ)

From Sap to Syrup

ERIC Educational Resources Information Center

Bjork, Janna

2005-01-01

Warm days, cold nights, melting snow-signs winter is waning and spring is nearing. Though winter may just be getting started in some areas, it's always fun to appreciate the good things about winter, including the special time at the end of winter in New England known as "sugaring time." The sap starts flowing in the sugar maples, and…

29 CFR 780.804 - “Ginning” of cotton.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 3 2014-07-01 2014-07-01 false âGinningâ of cotton. 780.804 Section 780.804 Labor... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for...

29 CFR 780.804 - “Ginning” of cotton.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 3 2012-07-01 2012-07-01 false âGinningâ of cotton. 780.804 Section 780.804 Labor... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for...

29 CFR 780.804 - “Ginning” of cotton.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 29 Labor 3 2011-07-01 2011-07-01 false âGinningâ of cotton. 780.804 Section 780.804 Labor... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for...

29 CFR 780.804 - “Ginning” of cotton.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 3 2013-07-01 2013-07-01 false âGinningâ of cotton. 780.804 Section 780.804 Labor... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for...

29 CFR 780.804 - “Ginning” of cotton.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 29 Labor 3 2010-07-01 2010-07-01 false âGinningâ of cotton. 780.804 Section 780.804 Labor... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for...

Efficiency of using solid wood fuels in maple syrup evaporators

Treesearch

Lawrence D. Garrett

1981-01-01

A study of commercial, wood-fired evaporators revealed that normal expected thermal efficiencies are between 35 and 50 percent. The moisture content and quality of wood fuels used and the design and method of firing the evaporator are critical in determining evaporator efficiency and the economic implications of using wood.

Inhibition, by 2-oxo acids that accumulate in maple-syrup-urine disease, of lactate, pyruvate, and 3-hydroxybutyrate transport across the blood-brain barrier.

PubMed

Cremer, J E; Teal, H M; Cunningham, V J

1982-09-01

Data are presented in support of the transport of (-)-D-3-hydroxybutyrate across the blood-brain barrier (BBB) being a carrier-mediated process. The kinetic parameters in 21-day-old pentobarbital-anaesthetized rats were Vmax 2.0 mumol.g-1.min-1, Km 29 mM, and KD 0.024 ml.g-1.min-1. The value for Vmax was the same as that for L-lactate and pyruvate transport in animals of the same age. The transport of all three substrates was sensitive to inhibition by low concentrations of either 2-oxo-3-methylbutanoate or 2-oxo-4-methylpentanoate, the 2-oxo acids that can accumulate in patients with maple-syrup-urine disease. The Ki values for the 2-oxo acids were severalfold lower than the respective Km values. 2-Oxo-3-phenylpropionate was a poor inhibitor. The relative affinities of the various monocarboxylic acids for the transport system of the BBB distinguished it from similar systems described in brain, heart, and liver mitochondria; human erythrocytes; and Ehrlich ascites-tumour cells.

Twenty novel mutations in BCKDHA, BCKDHB and DBT genes in a cohort of 52 Saudi Arabian patients with maple syrup urine disease.

PubMed

Imtiaz, Faiqa; Al-Mostafa, Abeer; Allam, Rabab; Ramzan, Khushnooda; Al-Tassan, Nada; Tahir, Asma I; Al-Numair, Nouf S; Al-Hamed, Mohamed H; Al-Hassnan, Zuhair; Al-Owain, Mohammad; Al-Zaidan, Hamad; Al-Amoudi, Mohammad; Qari, Alya; Balobaid, Ameera; Al-Sayed, Moeenaldeen

2017-06-01

Maple syrup urine disease (MSUD), an autosomal recessive inborn error of metabolism due to defects in the branched-chain α-ketoacid dehydrogenase (BCKD) complex, is commonly observed among other inherited metabolic disorders in the kingdom of Saudi Arabia. This report presents the results of mutation analysis of three of the four genes encoding the BCKD complex in 52 biochemically diagnosed MSUD patients originating from Saudi Arabia. The 25 mutations (20 novel) detected spanned across the entire coding regions of the BCKHDA , BCKDHB and DBT genes. There were no mutations found in the DLD gene in this cohort of patients. Prediction effects, conservation and modelling of novel mutations demonstrated that all were predicted to be disease-causing. All mutations presented in a homozygous form and we did not detect the presence of a "founder" mutation in any of three genes. In addition, prenatal molecular genetic testing was successfully carried out on chorionic villus samples or amniocenteses in 10 expectant mothers with affected children with MSUD, molecularly characterized by this study.

Diagnostic tools of early brain disturbances in an asymptomatic neonate with maple syrup urine disease.

PubMed

Terek, Demet; Koroglu, Ozge; Yalaz, Mehmet; Gokben, Sarenur; Calli, Cem; Coker, Mahmut; Kultursay, Nilgun

2013-08-01

Maple syrup urine disease (MSUD) is a rare inherited metabolic disorder resulting from the defective activity of branched-chain 2-ketoacid dehydrogenase complex. Routine screening of newborn with tandem mass spectroscopy on the third day of life may detect elevated branched-chain amino acids in blood before the appearance of encephalopathic symptoms in MSUD cases. If undiagnosed by such a routine screening test, patients often present with encephalopathy and seizures. Clinical neurologic examination is supplemented by electroencephalography and imaging. Here, we report abnormal amplitude-integrated electroencephalography, electroencephalography, magnetic resonance imaging, and magnetic resonance imaging spectroscopy findings in a neurologically asymptomatic male newborn who was diagnosed with MSUD at the third week of life. These neurologic disturbances disappeared at the fourth month of life with appropriate special diet. Therefore, even in already asymptomatic cases, early neurologic deterioration of brain metabolism and structure can be detected with these early laboratory findings, indicating the importance of early diagnosis and management. Patients may also benefit from these investigations during the follow-up period. Georg Thieme Verlag KG Stuttgart · New York.

MRI and clinical features of maple syrup urine disease: preliminary results in 10 cases.

PubMed

Cheng, Ailan; Han, Lianshu; Feng, Yun; Li, Huimin; Yao, Rong; Wang, Dengbin; Jin, Biao

2017-01-01

We aimed to evaluate the magnetic resonance imaging (MRI) and clinical features of maple syrup urine disease (MSUD). This retrospective study consisted of 10 MSUD patients confirmed by genetic testing. All patients underwent brain MRI. Phenotype, genotype, and areas of brain injury on MRI were retrospectively reviewed. Six patients (60%) had the classic form of MSUD with BCKDHB mutation, three patients (30%) had the intermittent form (two with BCKDHA mutations and one with DBT mutation), and one patient (10%) had the thiamine-responsive form with DBT mutation. On diffusion-weighted imaging, nine cases presented restricted diffusion in myelinated areas, and one intermittent case with DBT mutation was normal. The classic form of MSUD involved the basal ganglia in six cases; the cerebellum, mesencephalon, pons, and supratentorial area in five cases; and the thalamus in four cases, respectively. The intermittent form involved the cerebellum, pons, and supratentorial area in two cases. The thiamine-responsive form involved the basal ganglia and supratentorial area. Our preliminary results indicate that patients with MSUD presented more commonly in classic form with BCKDHB mutation and displayed extensive brain injury on MRI.

Maple syrup urine disease (MSUD): a case with long-term follow-up after liver transplantation.

PubMed

McLaughlin, Paula M; Hinshaw, Jessica; Stringer, Anthony Y

2013-01-01

Maple syrup urine disease (MSUD) is a rare hereditary metabolic condition where the body is unable to breakdown amino acids causing toxic buildup. Acute and long-term management of MSUD involves a restricted diet and regular monitoring of amino acid levels; however, more recently liver transplants have been shown to be successful in treating this condition. Even with successful management of MSUD there is evidence from pediatric cases that shows a distinct pattern of neurocognitive deficits associated with this condition, including impaired nonverbal skills and psychomotor functioning with relatively intact verbal abilities. In the present paper, we report an adult case of MSUD with associated neurocognitive deficits and functional limitations following liver transplantation. Neuroimaging revealed no structural abnormalities, while the results from the neuropsychological evaluation showed impairment in visual-spatial processing, attention, executive functioning, and psychomotor abilities, with relative strengths in verbal skills. The patient also showed reduced adaptive functioning and mild anxiety. This case demonstrates neurocognitive deficiencies within the context of normal magnetic resonance imaging. The possible underlying mechanism of this neuropsychological profile is discussed in relation to other neurodevelopmental models.

Effects of a Standardized Phenolic-Enriched Maple Syrup Extract on β-Amyloid Aggregation, Neuroinflammation in Microglial and Neuronal Cells, and β-Amyloid Induced Neurotoxicity in Caenorhabditis elegans.

PubMed

Ma, Hang; DaSilva, Nicholas A; Liu, Weixi; Nahar, Pragati P; Wei, Zhengxi; Liu, Yongqiang; Pham, Priscilla T; Crews, Rebecca; Vattem, Dhiraj A; Slitt, Angela L; Shaikh, Zahir A; Seeram, Navindra P

2016-11-01

Published data supports the neuroprotective effects of several phenolic-containing natural products, including certain fruit, berries, spices, nuts, green tea, and olive oil. However, limited data are available for phenolic-containing plant-derived natural sweeteners including maple syrup. Herein, we investigated the neuroprotective effects of a chemically standardized phenolic-enriched maple syrup extract (MSX) using a combination of biophysical, in vitro, and in vivo studies. Based on biophysical data (Thioflavin T assay, transmission electron microscopy, circular dichroism, dynamic light scattering, and zeta potential), MSX reduced amyloid β 1-42 peptide (Aβ 1-42 ) fibrillation in a concentration-dependent manner (50-500 μg/mL) with similar effects as the neuroprotective polyphenol, resveratrol, at its highest test concentration (63.5 % at 500 μg/mL vs. 77.3 % at 50 μg/mL, respectively). MSX (100 μg/mL) decreased H 2 O 2 -induced oxidative stress (16.1 % decrease in ROS levels compared to control), and down-regulated the production of lipopolysaccharide (LPS)-stimulated inflammatory markers (22.1, 19.9, 74.8, and 87.6 % decrease in NOS, IL-6, PGE 2 , and TNFα levels, respectively, compared to control) in murine BV-2 microglial cells. Moreover, in a non-contact co-culture cell model, differentiated human SH-SY5Y neuronal cells were exposed to conditioned media from BV-2 cells treated with MSX (100 μg/mL) and LPS or LPS alone. MSX-BV-2 media increased SH-SY5Y cell viability by 13.8 % compared to media collected from LPS-BV-2 treated cells. Also, MSX (10 μg/mL) showed protective effects against Aβ 1-42 induced neurotoxicity and paralysis in Caenorhabditis elegans in vivo. These data support the potential neuroprotective effects of MSX warranting further studies on this natural product.

Effects of a Standardized Phenolic-Enriched Maple Syrup Extract on β-Amyloid Aggregation, Neuroinflammation in Microglial and Neuronal Cells, and β-Amyloid Induced Neurotoxicity in Caenorhabditis elegans

PubMed Central

Ma, Hang; DaSilva, Nicholas A.; Liu, Weixi; Nahar, Pragati P.; Wei, Zhengxi; Liu, Yongqiang; Pham, Priscilla T.; Crews, Rebecca; Vattem, Dhiraj A.; Slitt, Angela L.; Shaikh, Zahir A.; Seeram, Navindra P.

2018-01-01

Published data supports the neuroprotective effects of several phenolic-containing natural products, including certain fruit, berries, spices, nuts, green tea, and olive oil. However, limited data are available for phenolic-containing plant-derived natural sweeteners including maple syrup. Herein, we investigated the neuroprotective effects of a chemically standardized phenolic-enriched maple syrup extract (MSX) using a combination of biophysical, in vitro, and in vivo studies. Based on biophysical data (Thioflavin T assay, transmission electron microscopy, circular dichroism, dynamic light scattering, and zeta potential), MSX reduced amyloid β1–42 peptide (Aβ1–42) fibrillation in a concentration-dependent manner (50–500 μg/mL) with similar effects as the neuroprotective polyphenol, resveratrol, at its highest test concentration (63.5% at 500 μg/mL vs. 77.3% at 50 μg/mL, respectively). MSX (100 μg/mL) decreased H2O2-induced oxidative stress (16.1% decrease in ROS levels compared to control), and down-regulated the production of lipopolysaccharide (LPS)-stimulated inflammatory markers (22.1, 19.9, 74.8, and 87.6% decrease in NOS, IL-6, PGE2, and TNFα levels, respectively, compared to control) in murine BV-2 microglial cells. Moreover, in a non-contact co-culture cell model, differentiated human SH-SY5Y neuronal cells were exposed to conditioned media from BV-2 cells treated with MSX (100 μg/mL) and LPS or LPS alone. MSX-BV-2 media increased SH-SY5Y cell viability by 13.8% compared to media collected from LPS-BV-2 treated cells. Also, MSX (10 μg/mL) showed protective effects against Aβ1–42 induced neurotoxicity and paralysis in Caenorhabditis elegans in vivo. These data support the potential neuroprotective effects of MSX warranting further studies on this natural product. PMID:27418278

29 CFR 780.805 - Ginning of “cotton.”

Code of Federal Regulations, 2014 CFR

2014-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for Market § 780.805 Ginning of “cotton.” Only the ginning of “cotton” is within the first part of the...

29 CFR 780.805 - Ginning of “cotton.”

Code of Federal Regulations, 2011 CFR

2011-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for Market § 780.805 Ginning of “cotton.” Only the ginning of “cotton” is within the first part of the...

29 CFR 780.805 - Ginning of “cotton.”

Code of Federal Regulations, 2013 CFR

2013-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for Market § 780.805 Ginning of “cotton.” Only the ginning of “cotton” is within the first part of the...

29 CFR 780.805 - Ginning of “cotton.”

Code of Federal Regulations, 2012 CFR

2012-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for Market § 780.805 Ginning of “cotton.” Only the ginning of “cotton” is within the first part of the...

29 CFR 780.805 - Ginning of “cotton.”

Code of Federal Regulations, 2010 CFR

2010-07-01

... Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton for Market § 780.805 Ginning of “cotton.” Only the ginning of “cotton” is within the first part of the...

The economics of a threatened tradition

Treesearch

Richard Matthews

1991-01-01

Writing a magazine article on thrips in November for an issue of Country Journal that would appear three months later was a considerable challenge, and I apologize now for any mistakes you may find in it. Yet in the process of researching it, I talked to dozens of farmers who count heavily on their annual maple syrup production to supplement their incomes. It is this...

29 CFR 780.807 - Cotton must be ginned “for market.”

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 3 2013-07-01 2013-07-01 false Cotton must be ginned âfor market.â 780.807 Section 780.807... Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton...

29 CFR 780.807 - Cotton must be ginned “for market.”

Code of Federal Regulations, 2011 CFR

2011-07-01

... 29 Labor 3 2011-07-01 2011-07-01 false Cotton must be ginned âfor market.â 780.807 Section 780.807... Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton...

29 CFR 780.807 - Cotton must be ginned “for market.”

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 3 2014-07-01 2014-07-01 false Cotton must be ginned âfor market.â 780.807 Section 780.807... Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton...

29 CFR 780.807 - Cotton must be ginned “for market.”

Code of Federal Regulations, 2010 CFR

2010-07-01

... 29 Labor 3 2010-07-01 2010-07-01 false Cotton must be ginned âfor market.â 780.807 Section 780.807... Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton...

29 CFR 780.807 - Cotton must be ginned “for market.”

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 3 2012-07-01 2012-07-01 false Cotton must be ginned âfor market.â 780.807 Section 780.807... Employment in Ginning of Cotton and Processing of Sugar Beets, Sugar-Beet Molasses, Sugarcane, or Maple Sap into Sugar or Syrup; Exemption From Overtime Pay Requirements Under Section 13(b)(15) Ginning of Cotton...

Force Health Protection: Nutrition and Exercise Resource Manual

DTIC Science & Technology

1999-09-01

table sugar (sucrose), sugars in fruit ( fructose ), honey ( fructose and glucose), sugar in milk (lactose), maple syrup , and molasses. Some are added in...saunas, stream rooms, and whirlpools. High blood pressure. Irregular heart beats. Anemia. Premature labor. History of premature labor...Exercise Resource Manual.” Wellness and health promotion are high priority goals for the Navy. Maintaining personal fitness and sensible nutrition

Application of liquid chromatography-tandem mass spectrometry in the diagnosis and follow-up of maple syrup urine disease in a Chinese population.

PubMed

Lin, Na; Ye, Jun; Qiu, Wenjuan; Han, Lianshu; Zhang, Huiwen; Gu, Xuefan

2013-01-01

Maple syrup urine disease (MSUD) is an inherited disorder caused by a deficiency of the mitochondrial branched-chain keto acid dehydrogenase complex. We investigated whether liquid chromatography-tandem mass spectrometry (LC-MS/MS) is a more reliable and accurate method than MS/MS in the diagnosis and management of patients with MSUD in a Chinese population. A total of 370 dried blood spots (DBS) from healthy neonates, 44 DBS specimens from phenylketonuria neonates, and 38 DBS samples from 10 MSUD patients were retrospectively tested using the LC-MS/MS method. The results were compared with those obtained by the MS/MS method. The reference intervals of branched-chain amino acids (BCAAs) and alloiosleucine (Allo-Ile) were estimated for both sexes. In classic MSUD patients, Allo-Ile was markedly elevated (average of 136 μmol/L, which was significantly higher than the normal value, <5 μmol/L). The averages of BCAAs were also markedly elevated continually during the treatment. The application of the LC-MS/MS method in the measurement of Allo-Ile and BCAAs in DBS is more useful for diagnosing and managing classic MSUD than the MS/MS method.

Biochemical correlates of neuropsychiatric illness in maple syrup urine disease.

PubMed

Muelly, Emilie R; Moore, Gregory J; Bunce, Scott C; Mack, Julie; Bigler, Don C; Morton, D Holmes; Strauss, Kevin A

2013-04-01

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain amino acid metabolism presenting with neonatal encephalopathy, episodic metabolic decompensation, and chronic amino acid imbalances. Dietary management enables survival and reduces risk of acute crises. Liver transplantation has emerged as an effective way to eliminate acute decompensation risk. Psychiatric illness is a reported MSUD complication, but has not been well characterized and remains poorly understood. We report the prevalence and characteristics of neuropsychiatric problems among 37 classical MSUD patients (ages 5-35 years, 26 on dietary therapy, 11 after liver transplantation) and explore their underlying mechanisms. Compared with 26 age-matched controls, MSUD patients were at higher risk for disorders of cognition, attention, and mood. Using quantitative proton magnetic resonance spectroscopy, we found lower brain glutamate, N-acetylaspartate (NAA), and creatine concentrations in MSUD patients, which correlated with specific neuropsychiatric outcomes. Asymptomatic neonatal course and stringent longitudinal biochemical control proved fundamental to optimizing long-term mental health. Neuropsychiatric morbidity and neurochemistry were similar among transplanted and nontransplanted MSUD patients. In conclusion, amino acid dysregulation results in aberrant neural networks with neurochemical deficiencies that persist after transplant and correlate with neuropsychiatric morbidities. These findings may provide insight into general mechanisms of psychiatric illness.

A Novel Whole Gene Deletion of BCKDHB by Alu-Mediated Non-allelic Recombination in a Chinese Patient With Maple Syrup Urine Disease

PubMed Central

Liu, Gang; Ma, Dingyuan; Hu, Ping; Wang, Wen; Luo, Chunyu; Wang, Yan; Sun, Yun; Zhang, Jingjing; Jiang, Tao; Xu, Zhengfeng

2018-01-01

Maple syrup urine disease (MSUD) is an autosomal recessive inherited metabolic disorder caused by mutations in the BCKDHA, BCKDHB, DBT, and DLD genes. Among the wide range of disease-causing mutations in BCKDHB, only one large deletion has been associated with MSUD. Compound heterozygous mutations in BCKDHB were identified in a Chinese patient with typical MSUD using next-generation sequencing, quantitative PCR, and array comparative genomic hybridization. One allele presented a missense mutation (c.391G > A), while the other allele had a large deletion; both were inherited from the patient’s unaffected parents. The deletion breakpoints were characterized using long-range PCR and sequencing. A novel 383,556 bp deletion (chr6: g.80811266_81194921del) was determined, which encompassed the entire BCKDHB gene. The junction site of the deletion was localized within a homologous sequence in two AluYa5 elements. Hence, Alu-mediated non-allelic homologous recombination is speculated as the mutational event underlying the large deletion. In summary, this study reports a recombination mechanism in the BCKDHB gene causing a whole gene deletion in a newborn with MSUD. PMID:29740478

Structural white matter changes in adolescents and young adults with maple syrup urine disease.

PubMed

Klee, D; Thimm, E; Wittsack, H J; Schubert, D; Primke, R; Pentang, G; Schaper, J; Mödder, U; Antoch, A; Wendel, U; Cohnen, M

2013-11-01

To get insight into the nature of magnetic resonance (MR) white matter abnormalities of patients with classic maple syrup urine disease (MSUD) under diet control. Ten patients with classic MSUD and one with a severe MSUD variant (mean age 21.5 ± 5.1 years) on diet and 11 age and sex-matched healthy subjects were enrolled. Apart from standard MR sequences, diffusion weighted images (DWI), diffusion tensor images (DTI), and magnetization transfer images (MT) were obtained and comparatively analyzed for apparent diffusion coefficient (ADC), tensor fractional anisotropy (FA) and MT maps in 11 regions of interest (ROI) within the white matter. In MSUD patients DWI, DTI and FA showed distinct signal changes in the cerebral hemispheres, the dorsal limb of internal capsule, the brain stem and the central cerebellum. Signal intensity was increased in DWI with a reduced ADC and decreased values for FA. MT did not reveal differences between patients and control subjects. Signal abnormalities in the white matter of adolescents and young adults under diet control may be interpreted as consequence of structural alterations like dysmyelination. The reduced ADC and FA in the white matter with preserved MT indicate a reduction in fiber tracks.

Maple syrup urine disease: The E1{beta} gene of human branched-chain {alpha}-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3{prime} UTR in one of the two E1{beta} mRNAs arises from intronic sequences

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chuang, J.L.; Chuang, D.T.; Cox, R.P.

1996-06-01

Maple syrup urine disease (MSUD) or branched-chain ketoaciduria is caused by a deficiency in the mitochondrial branched-chain {alpha}-ketoacid dehydrogenase (BCKAD) complex. The clinical manifestations are characterized by accumulation of branched chain amino and {alpha}-ketoacids, which leads to severe cerebral edema with seizures, ketoacidosis, and mental retardation. The BCKAD complex comprises three catalytic components, i.e., a decarboxylase (E1) consisting of two E1{alpha} (M{sub r} = 46,000) and two E1{Beta} (M{sub r} = 37,500) subunits, a transacylase (E2) that contains 24 lipoic acid-bearing subunits, and a dehydrogenase (E3), which is a homodimeric flavoprotein. MSUD is genetically heterogeneous, since mutations in the E1{alpha}more » subunit (type IA MSUD), the E1{Beta} subunit (type IB), the E2 subunit (type II) and the E3 subunit (type III) have been described. The functional consequences of certain mutations in the BCKAD complex have been studied. 23 refs., 3 figs.« less

Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease.

PubMed

Demirkol, Demet; Şık, Güntülü; Topal, Nilüfer; Çıtak, Agop; Zeybek, Çigdem; Tüten, Abdülhamit; Bilge, Ilmay

2016-01-01

The study aims to define the efficacy of continuous renal replacement therapy in acute metabolic decompensation treatment of maple syrup urine disease (MSUD). All the neonates, infants and children who have had life threatening conditions due to MSUD and were treated with continuous venovenous hemodiafiltration (CVVHDF) were analyzed retrospectively. Fourteen patients underwent 15 sessions of CVVHDF (age range 15 days to 87 months, mean 40.8 ± 31.4 months). One patient required additional CVVHDF 1 week after cessation of CVVHDF. Twenty seven percent (n = 4) of the patients were intubated and mechanically ventilated. Twelve patients responded to treatment and dramatic neurological improvement was observed within 24 h. Two of the 14 patients required 36 h of CVVHDF for neurological improvement. The mean duration of CVVHDF was 20.2 ± 8.6 (9-36) h. The mean leucine level was 1,648 ± 623.8 (714-2,768) μmol/l before and was 256.5 ± 150.6 (117-646) μmol/l at the end of treatment. No mortality was observed. Continuous hemodiafiltration is an effective and safe method in correcting metabolic disturbances in MSUD. © 2016 S. Karger AG, Basel.

Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease

PubMed Central

Uaariyapanichkul, Jaraspong; Saengpanit, Puthita; Damrongphol, Ponghatai; Suphapeetiporn, Kanya

2017-01-01

Introduction Maple syrup urine disease (MSUD) is an inborn error of branched chain amino acids (BCAAs) metabolism. We report an infant with MSUD who developed 2 episodes of cutaneous lesions as a result of isoleucine deficiency and zinc deficiency, respectively. Case Presentation A 12-day-old male infant was presented with poor milk intake and lethargy. The diagnosis of MSUD was made based on clinical and biochemical data. Management and Outcome Specific dietary restriction of BCAAs was given. Subsequently, natural protein was stopped as the patient developed hospital-acquired infections which resulted in an elevation of BCAAs. Acrodermatitis dysmetabolica developed and was confirmed to be from isoleucine deficiency. At the age of 6 months, the patient developed severe lethargy and was on natural protein exclusion for an extended period. Despite enteral supplementation of zinc sulfate, cutaneous manifestations due to zinc deficiency occurred. Discussion Skin lesions in MSUD patients could arise from multiple causes. Nutritional deficiency including isoleucine and zinc deficiencies can occur and could complicate the treatment course as a result of malabsorption, even while on enteral supplementation. Parenteral nutrition should be considered and initiated accordingly. Clinical status, as well as BCAA levels, should be closely monitored in MSUD patients. PMID:29209542

MRI and clinical features of maple syrup urine disease: preliminary results in 10 cases

PubMed Central

Cheng, Ailan; Han, Lianshu; Feng, Yun; Li, Huimin; Yao, Rong; Wang, Dengbin; Jin, Biao

2017-01-01

PURPOSE We aimed to evaluate the magnetic resonance imaging (MRI) and clinical features of maple syrup urine disease (MSUD). METHODS This retrospective study consisted of 10 MSUD patients confirmed by genetic testing. All patients underwent brain MRI. Phenotype, genotype, and areas of brain injury on MRI were retrospectively reviewed. RESULTS Six patients (60%) had the classic form of MSUD with BCKDHB mutation, three patients (30%) had the intermittent form (two with BCKDHA mutations and one with DBT mutation), and one patient (10%) had the thiamine-responsive form with DBT mutation. On diffusion-weighted imaging, nine cases presented restricted diffusion in myelinated areas, and one intermittent case with DBT mutation was normal. The classic form of MSUD involved the basal ganglia in six cases; the cerebellum, mesencephalon, pons, and supratentorial area in five cases; and the thalamus in four cases, respectively. The intermittent form involved the cerebellum, pons, and supratentorial area in two cases. The thiamine-responsive form involved the basal ganglia and supratentorial area. CONCLUSION Our preliminary results indicate that patients with MSUD presented more commonly in classic form with BCKDHB mutation and displayed extensive brain injury on MRI. PMID:28830848

A Novel Whole Gene Deletion of BCKDHB by Alu-Mediated Non-allelic Recombination in a Chinese Patient With Maple Syrup Urine Disease.

PubMed

Liu, Gang; Ma, Dingyuan; Hu, Ping; Wang, Wen; Luo, Chunyu; Wang, Yan; Sun, Yun; Zhang, Jingjing; Jiang, Tao; Xu, Zhengfeng

2018-01-01

Maple syrup urine disease (MSUD) is an autosomal recessive inherited metabolic disorder caused by mutations in the BCKDHA, BCKDHB, DBT , and DLD genes. Among the wide range of disease-causing mutations in BCKDHB , only one large deletion has been associated with MSUD. Compound heterozygous mutations in BCKDHB were identified in a Chinese patient with typical MSUD using next-generation sequencing, quantitative PCR, and array comparative genomic hybridization. One allele presented a missense mutation (c.391G > A), while the other allele had a large deletion; both were inherited from the patient's unaffected parents. The deletion breakpoints were characterized using long-range PCR and sequencing. A novel 383,556 bp deletion (chr6: g.80811266_81194921del) was determined, which encompassed the entire BCKDHB gene. The junction site of the deletion was localized within a homologous sequence in two AluYa5 elements. Hence, Alu-mediated non-allelic homologous recombination is speculated as the mutational event underlying the large deletion. In summary, this study reports a recombination mechanism in the BCKDHB gene causing a whole gene deletion in a newborn with MSUD.

[Maple syrup urine disease and gene mutations in twin neonates].

PubMed

Li, Tao; Wang, Yu; Li, Cui; Xu, Wei-Wei; Niu, Feng-Hai; Zhang, Di

2016-12-01

To investigate the clinical features of one pair of twin neonates with maple syrup urine disease (MSUD) in the Chinese Han population and pathogenic mutations in related genes, and to provide guidance for the early diagnosis and treatment of MSUD. The clinical and imaging data of the twin neonates were collected. The peripheral blood samples were collected from the twin neonates and their parents to detect the genes related to MSUD (BCKDHA, BCKDHB, DBT, and DLD). The loci with gene mutations were identified, and a bioinformatic analysis was performed. Two mutations were detected in the BCKDHB gene, missense mutation c.304G>A (p.Gly102Arg) and nonsense mutation c.331C>T (p.Arg111*), and both of them were heterozygotes. The mutation c.304G>A (p.Gly102Arg) had not been reported in the world. Their father carried the missense mutation c.304G>A (p.Gly102Arg), and their mother carried the nonsense mutation c.331C>T (p.Arg111*). The c.331C>T (p.Arg111*) heterozygous mutation in BCKDHB gene is the pathogenic mutation in these twin neonates and provides a genetic and molecular basis for the clinical features of children with MSUD.

Insects attracted to Maple Sap: Observations from Prince Edward Island, Canada

PubMed Central

Majka, Christopher G.

2010-01-01

Abstract The collection of maple sap for the production of maple syrup is a large commercial enterprise in Canada and the United States. In Canada, which produces 85% of the world’s supply, it has an annual value of over $168 million CAD. Over 38 million trees are tapped annually, 6.5% of which use traditional buckets for sap collection. These buckets attract significant numbers of insects. Despite this, there has been very little investigation of the scale of this phenomenon and the composition of insects that are attracted to this nutrient source. The present paper reports the results of a preliminary study conducted on Prince Edward Island, Canada. Twenty-eight species of Coleoptera, Lepidoptera, and Trichoptera were found in maple sap buckets, 19 of which are known to be attracted to saps and nectars. The physiological role of sap feeding is discussed with reference to moths of the tribe Xylenini, which are active throughout the winter, and are well documented as species that feed on sap flows. Additionally, 18 of the 28 species found in this study are newly recorded in Prince Edward Island. PMID:21594122

Insects attracted to Maple Sap: Observations from Prince Edward Island, Canada.

PubMed

Majka, Christopher G

2010-07-23

The collection of maple sap for the production of maple syrup is a large commercial enterprise in Canada and the United States. In Canada, which produces 85% of the world's supply, it has an annual value of over $168 million CAD. Over 38 million trees are tapped annually, 6.5% of which use traditional buckets for sap collection. These buckets attract significant numbers of insects. Despite this, there has been very little investigation of the scale of this phenomenon and the composition of insects that are attracted to this nutrient source. The present paper reports the results of a preliminary study conducted on Prince Edward Island, Canada. Twenty-eight species of Coleoptera, Lepidoptera, and Trichoptera were found in maple sap buckets, 19 of which are known to be attracted to saps and nectars. The physiological role of sap feeding is discussed with reference to moths of the tribe Xylenini, which are active throughout the winter, and are well documented as species that feed on sap flows. Additionally, 18 of the 28 species found in this study are newly recorded in Prince Edward Island.

Effects of maple (Acer) plant part extracts on proliferation, apoptosis and cell cycle arrest of human tumorigenic and non-tumorigenic colon cells.

PubMed

González-Sarrías, Antonio; Li, Liya; Seeram, Navindra P

2012-07-01

Phenolic-enriched extracts of maple sap and syrup, obtained from the sugar and red maple species (Acer saccharum Marsh, A. rubrum L., respectively), are reported to show anticancer effects. Despite traditional medicinal uses of various other parts of these plants by Native Americans, they have not been investigated for anticancer activity. Here leaves, stems/twigs, barks and sapwoods of both maple species were evaluated for antiproliferative effects against human colon tumorigenic (HCT-116, HT-29, Caco-2) and non-tumorigenic (CCD-18Co) cells. Extracts were standardized to total phenolic and ginnalin-A (isolated in our laboratory) levels. Overall, the extracts inhibited the growth of the colon cancer more than normal cells (over two-fold), their activities increased with their ginnalin-A levels, with red > sugar maple extracts. The red maple leaf extract, which contained the highest ginnalin-A content, was the most active extract (IC₅₀  = 35 and 16 µg/mL for extract and ginnalin-A, respectively). The extracts were not cytotoxic nor did they induce apoptosis of the colon cancer cells. However, cell cycle analyses revealed that the antiproliferative effects of the extracts were mediated through cell cycle arrest in the S-phase. The results from the current study suggest that these maple plant part extracts may have potential anticolon cancer effects. Copyright © 2011 John Wiley & Sons, Ltd.

Definition of the mutation responsible for maple syrup urine disease in Poll Shorthorns and genotyping Poll Shorthorns and Poll Herefords for maple syrup urine disease alleles.

PubMed

Dennis, J A; Healy, P J

1999-08-01

The organisation of the E1alpha subunit of bovine branched-chain alpha-keto acid dehydrogenase gene was established. c DNA was cloned from Poll Shorthorn x Poll Hereford calves affected with Maple Syrup Urine Disease to identify the mutation responsible for the disease in Poll Shorthorns. Clones containing the c DNA sequences inherited from the Poll Shorthorn sire of the affected calves were identified. Paternal clones were sequenced and a cytidine to thymidine transition was found at nucleotide 1380. The mutation is predicted to substitute leucine in place of a highly conserved proline at codon 372. A polymerase chain reaction procedure was developed for detection of the 1380C-->T mutation in genomic DNA. Three Poll Shorthorn parents of affected calves and three affected Poll Shorthorn x Poll Hereford calves were heterozygous and an affected Poll Shorthorn calf was homozygous for this mutation. An improved polymerase chain reaction procedure was also devised to genotype Poll Herefords for the 248C-->T mutation. The procedures will facilitate disease prevention programs and assist in differential diagnosis of conditions in new-born calves that present with a rapid onset of progressive neurological disease and are characterised histologically by 'status spongiosus'. Maple Syrup Urine Disease (MSUD) is an autosomal recessive defect reported in humans (Danner and Elsas 1989), and in Poll Hereford (PH) and Poll Shorthorn (PS) calves (Harper et al 1986, Healy et al 1992). The clinical, biochemical and pathological manifestations of the disease are identical in the two breeds of cattle, and are characterised by the rapid onset of progressive neurological disease, leading to death within a few days of birth. The disease is caused by a deficiency of activity of the mitochondrial enzyme branched-chain alpha-keto acid dehydrogenase (BCKADH). This deficiency leads to elevated concentrations, in blood and tissues, of branched chain alpha-keto acids and their precursors, the branched chain amino acids, valine, leucine and isoleucine. BCKADH consists of four subunits E1alpha, E1beta, E2 and E3 that are encoded by separate genes, and MSUD may result from deficiency of any of the subunits. In PH s, the disease in caused by premature termination of translation, of the E1alpha subunit, that is induced by a cytidine to thymidine transition exon 2 (248C-->T), that converts the glutamine codon -6 to a stop codon (Q-6ST; Zhang et al 1990). We have shown that MSUD -affected PSxPH calves are heterozygous at the PH locus, illustrating molecular heterogeneity exists for bovine MSUD (Healy and Dennis 1994a). The fact that these crossbred calves are affected, indicates the PS, like the PH mutation, resides in the E1alpha subunit. Copyright 1999 Harcourt Publishers Ltd.

Black Males in the Green Mountains: Colorblindness and Cultural Competence in Vermont Public Schools. Black Studies and Critical Thinking. Volume 38

ERIC Educational Resources Information Center

Dunbar, Denise Helen

2013-01-01

Mention the state of Vermont and images of maple syrup, scenic mountains, and progressive politics come to mind. But in addition to skiing, farming, and fall foliage, there is also a startling history of racial and religious intolerance and bigotry. Burlington is known as the birthplace of John Dewey, whose enlightened views about education…

Climate Change in the School Yard: Monitoring the Health of Acer Saccharum with A Maple Report Card

NASA Astrophysics Data System (ADS)

Carlson, M.; Diller, A.; Rock, B. N.

2012-12-01

K-12 Teachers and students engage in authentic science and a research partnership with scientists in Maple Watch, a University of New Hampshire outreach program. Maple Watch is a hands-on, inquiry-based program in which students learn about climate change and air quality as well as many other environmental stress factors which may affect the health of sugar maple. The iconic New England tree is slated to lose 52% of its range in this century. Maple Watch builds on the 20-year record of Forest Watch, a K-12 program in which students and teachers have contributed annual research specimens and data to a UNH study of tropospheric ozone and its impact on white pine (Pinus strobus). Maple Watch students monitor sugar maples (Acer saccharum) year-round for signals of strain and disease. Students report the first run in sap season, bud burst and leaf development, and leaf senescence and fall. Across New England the timing of these phenologic events is changing with climate warming. Students assess maple health with simple measures of leaf development in May, leaf senescence in early fall and bud quality in late fall. Simple student arithmetic rankings of leaf and bud health correlate with chlorophyll content and spectral reflectance measures that students can analyze and compare with researchers at UNH. Grading their trees for each test on a one-two-three scale, students develop a Maple Report Card for each type of measurement, which presents an annual portrait of tree health. Year-by-year, schools across the sugar maple's 31 million acre range could monitor changes in tree health. The change over time in maple health can be graphed in parallel with the Goddard Space Institute's Common Sense Climate Index. Four teachers, listed as co-authors here, began a pilot study with Maple Watch in 2010, contributing sap samples and sharing curricular activities with UNH. Pilot Maple Watch schools already manage stands of sugar maples and make maple syrup and are assisting in training new schools in Maple Watch protocols. The program also includes assessment of educational philosophy as we consider how to discuss climate change with K-12 students. Maple Watch teachers are learning how to discuss the death of a tree, extirpation of a species, and human connections with nature.

Maple polyphenols, ginnalins A-C, induce S- and G2/M-cell cycle arrest in colon and breast cancer cells mediated by decreasing cyclins A and D1 levels.

PubMed

González-Sarrías, Antonio; Ma, Hang; Edmonds, Maxwell E; Seeram, Navindra P

2013-01-15

Polyphenols are bioactive compounds found in plant foods. Ginnalins A-C are polyphenols present in the sap and other parts of the sugar and red maple species which are used to produce maple syrup. Here we evaluated the antiproliferative effects of ginnalins A-C on colon (HCT-116) and breast (MCF-7) tumourigenic and non-tumourigenic colon (CCD-18Co) cells and investigated whether these effects were mediated through cell cycle arrest and/or apoptosis. Ginnalins A-C were twofold more effective against the tumourigenic than non-tumourigenic cells. Among the polyphenols, ginnalin A (84%, HCT-116; 49%, MCF-7) was more effective than ginnalins B and C (50%, HCT-116; 30%, MCF-7) at 50 μM concentrations. Ginnalin A did not induce apoptosis of the cancer cells but arrested cell cycle (in the S- and G(2)/M-phases) and decreased cyclins A and D1 protein levels. These results suggest that maple polyphenols may have potential cancer chemopreventive effects mediated through cell cycle arrest. Copyright © 2012 Elsevier Ltd. All rights reserved.

Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach.

PubMed

Frazier, Dianne M; Allgeier, Courtney; Homer, Caroline; Marriage, Barbara J; Ogata, Beth; Rohr, Frances; Splett, Patricia L; Stembridge, Adrya; Singh, Rani H

2014-07-01

In an effort to increase harmonization of care and enable outcome studies, the Genetic Metabolic Dietitians International (GMDI) and the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) are partnering to develop nutrition management guidelines for inherited metabolic disorders (IMD) using a model combining both evidence- and consensus-based methodology. The first guideline to be completed is for maple syrup urine disease (MSUD). This report describes the methodology used in its development: formulation of five research questions; review, critical appraisal and abstraction of peer-reviewed studies and unpublished practice literature; and expert input through Delphi surveys and a nominal group process. This report includes the summary statements for each research question and the nutrition management recommendations they generated. Each recommendation is followed by a standardized rating based on the strength of the evidence and consensus used. The application of technology to build the infrastructure for this project allowed transparency during development of this guideline and will be a foundation for future guidelines. Online open access of the full, published guideline allows utilization by health care providers, researchers, and collaborators who advise, advocate and care for individuals with MSUD and their families. There will be future updates as warranted by developments in research and clinical practice. Copyright © 2014 The Authors. Published by Elsevier Inc. All rights reserved.

Two homozygous mutations in the exon 5 of BCKDHB gene that may cause the classic form of maple syrup urine disease.

PubMed

Su, Ling; Lu, Zhikun; Li, Fatao; Shao, Yongxian; Sheng, Huiying; Cai, Yanna; Liu, Li

2017-06-01

Maple syrup urine disease (MSUD) is a rare autosomal recessive genetic disorder caused by defects in the catabolism of the branched-chain amino acids (BCAAs). Classic form of MSUD (CMSUD) is caused by mutations in BCKDHA, BCKDHB, DBT genes mostly. In this study, we analyzed the clinical and genetic characteristics of two patients with CMSUD. Two homozygous mutations, c.517G > T (p.Asp173Tyr) and c.503G > A (p.Arg168His), both in the exon 5 of BCKDHB were detected respectively. The novel mutation p.Asp173Tyr of patient A, inherited from his parents, is predicted to affect conformation of protein by computer analysis. The reported mutation p.Arg168His observed in patient B seemed to occur in a maternal uniparental disomy inheritance manner. Review of related literature revealed that most missense mutations in exon 5 of BCKDHB in homozygous genotype often result in CMSUD because of its incorrect conformation, and exon 5 of BCKDHB might be a susceptible region. Thus the novel homozygous mutation p.Asp173Tyr and the founder homozygous mutation p.Arg168His may be responsible for the clinical presentation of the two CMSUD patients, facilitating the future genetic counselling and prenatal diagnosis.

Maple Syrup Urine Disease: Identification and Carrier-Frequency Determination of a Novel Founder Mutation in the Ashkenazi Jewish Population

PubMed Central

Edelmann, Lisa; Wasserstein, Melissa P.; Kornreich, Ruth; Sansaricq, Claude; Snyderman, Selma E.; Diaz, George A.

2001-01-01

Maple syrup urine disease (MSUD) is a rare, autosomal recessive disorder of branched-chain amino acid metabolism. We noted that a large proportion (10 of 34) of families with MSUD that were followed in our clinic were of Ashkenazi Jewish (AJ) descent, leading us to search for a common mutation within this group. On the basis of genotyping data suggestive of a conserved haplotype at tightly linked markers on chromosome 6q14, the BCKDHB gene encoding the E1β subunit was sequenced. Three novel mutations were identified in seven unrelated AJ patients with MSUD. The locations of the affected residues in the crystal structure of the E1β subunit suggested possible mechanisms for the deleterious effects of these mutations. Large-scale population screening of AJ individuals for R183P, the mutation present in six of seven patients, revealed that the carrier frequency of the mutant allele was ∼1/113; the patient not carrying R183P had a previously described homozygous mutation in the gene encoding the E2 subunit. These findings suggested that a limited number of mutations might underlie MSUD in the AJ population, potentially facilitating prenatal diagnosis and carrier detection of MSUD in this group. PMID:11509994

Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population.

PubMed

Edelmann, L; Wasserstein, M P; Kornreich, R; Sansaricq, C; Snyderman, S E; Diaz, G A

2001-10-01

Maple syrup urine disease (MSUD) is a rare, autosomal recessive disorder of branched-chain amino acid metabolism. We noted that a large proportion (10 of 34) of families with MSUD that were followed in our clinic were of Ashkenazi Jewish (AJ) descent, leading us to search for a common mutation within this group. On the basis of genotyping data suggestive of a conserved haplotype at tightly linked markers on chromosome 6q14, the BCKDHB gene encoding the E1beta subunit was sequenced. Three novel mutations were identified in seven unrelated AJ patients with MSUD. The locations of the affected residues in the crystal structure of the E1beta subunit suggested possible mechanisms for the deleterious effects of these mutations. Large-scale population screening of AJ individuals for R183P, the mutation present in six of seven patients, revealed that the carrier frequency of the mutant allele was approximately 1/113; the patient not carrying R183P had a previously described homozygous mutation in the gene encoding the E2 subunit. These findings suggested that a limited number of mutations might underlie MSUD in the AJ population, potentially facilitating prenatal diagnosis and carrier detection of MSUD in this group.

Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino-acid mixture.

PubMed

Servais, A; Arnoux, J B; Lamy, C; Hummel, A; Vittoz, N; Katerinis, I; Bazzaoui, V; Dubois, S; Broissand, C; Husson, M C; Berleur, M P; Rabier, D; Ottolenghi, C; Valayannopoulos, V; de Lonlay, P

2013-11-01

Acute decompensation of maple syrup urine disease (MSUD) is usually treated by enteral feeding with an amino-acid mixture without leucine (Leu), valine or isoleucine. However, its administration is ineffective in cases of gastric intolerance and some adult patients refuse enteral feeding via a nasogastric tube. We developed a new parenteral amino-acid mixture for patients with MSUD. Seventeen decompensation episodes in four adult patients with MSUD treated with a parenteral amino-acid mixture (group P) were compared to 18 previous episodes in the same patients treated by enteral feeding (group E). The mean Leu concentration at presentation was similar in the groups P and E (1196.9 μmol/L and 1212.2 μmol/L, respectively). The mean decrease in the Leu concentration during the first 3 days of hospitalisation was significantly higher in group P than group E (p = 0.0026); there were no side effects. The mean duration of hospitalisation was similar (4 vs. 4.5 days, p = NS). No patient in group P deteriorated whereas one patient in group E required dialysis. This new parenteral amino-acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.

Four novel mutations of the BCKDHA, BCKDHB and DBT genes in Iranian patients with maple syrup urine disease.

PubMed

Zeynalzadeh, Monica; Tafazoli, Alireza; Aarabi, Azadeh; Moghaddassian, Morteza; Ashrafzadeh, Farah; Houshmand, Massoud; Taghehchian, Negin; Abbaszadegan, Mohammad Reza

2018-01-26

Maple syrup urine disease (MSUD) is a rare metabolic autosomal recessive disorder caused by dysfunction of the branched-chain α-ketoacid dehydrogenase (BCKDH) complex. Mutations in the BCKDHA, BCKDHB and DBT genes are responsible for MSUD. The current study analyzed seven Iranian MSUD patients genetically and explored probable correlations between their genotype and phenotype. The panel of genes, including BCKDHA, BCKDHB and DBT, was evaluated, using routine the polymerase chain reaction (PCR)-sequencing method. In addition, protein modeling (homology and threading modeling) of the deduced novel mutations was performed. The resulting structures were then analyzed, using state-of-the-art bioinformatics tools to better understand the structural and functional effects caused by mutations. Seven mutations were detected in seven patients, including four novel pathogenic mutations in BCKDHA (c.1198delA, c.629C>T), BCKDHB (c.652C>T) and DBT (c.1150A>G) genes. Molecular modeling of the novel mutations revealed clear changes in the molecular energy levels and stereochemical traits of the modeled proteins, which may be indicative of strong correlations with the functional modifications of the genes. Structural deficiencies were compatible with the observed phenotypes. Any type of MSUD can show heterogeneous clinical manifestations in different ethnic groups. Comprehensive molecular investigations would be necessary for differential diagnosis.

Maple Syrup Urine Disease Complicated with Kyphoscoliosis and Myelopathy.

PubMed

Hou, Jia-Woei

2016-10-01

Maple syrup urine disease (MSUD) is an autosomal recessive aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain amino acids (BCAAs: leucine, isoleucine, and valine). Accumulation of their corresponding keto-acids leads to encephalopathy if not treated in time. A newborn male patient was suspected to have MSUD after tandem mass study when he presented symptoms and signs suggestive neonatal sepsis, anemia, and diarrhea. Food restriction of BCAAs was started; however, acrodermatitis enteropathica-like skin eruptions occurred at age 2 months. The skin rashes resolved after adding BCAAs and adjusting the infant formula. At age 7 months, he suffered from recurrent skin lesions, zinc deficiency, osteoporosis, and kyphosis of the thoracic spine with acute angulation over the T11-T12 level associated with spinal compression and myelopathy. After supplementation of zinc products and pamidronate, skin lesions and osteopenia improved gradually. Direct sequencing of the DBT gene showed a compound heterozygous mutation [4.7 kb deletion and c.650-651insT (L217F or L217fsX223)]. It is unusual that neurodegeneration still developed in this patient despite diet restriction. Additionally, brain and spinal magnetic resonance imaging, bone mineral density study, and monitoring of zinc status are suggested in MSUD patients. Copyright © 2014. Published by Elsevier B.V.

Identification of a novel homozygous mutation (S144I) in a Malay patient with maple syrup urine disease.

PubMed

Ali, Ernie Zuraida; Yunus, Zabedah Md; Desa, Norsiah Md; Hock, Ngu Lock

2013-01-01

Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder of branched-chain amino acid metabolism caused by the defective function of branched-chain α-ketoacid dehydrogenase complex (BCKDH). It is characterised by increased plasma leucine, isoleucine, and valine levels, and mutations can be detected in any one of the BCKDHA, BCKDHB, and DBT genes. In this study, we describe the molecular basis of a novel mutation found in one MSUD Malay patient from consanguineous parents. A homozygous mutation has been detected in this patient whose both parents carried a heterozygous mutation at DNA coding region c.431G>T in exon 4, which resulted in a substitution of serine to isoleucine at codon 144 (p.S144I). In silico analysis predicted S144I to be potentially damaging. The mutation was located on the alpha helical region of the BCKDHA protein, and it is predicted to affect the stability of protein due to the loss of various polar interactions between local secondary structures. Homology analysis revealed that this mutation occurred in a highly conserved region (100%). This result indicates that S144I mutation is likely pathogenic and may contribute to the classic form of MSUD in this patient.

Biochemical correlates of neuropsychiatric illness in maple syrup urine disease

PubMed Central

Muelly, Emilie R.; Moore, Gregory J.; Bunce, Scott C.; Mack, Julie; Bigler, Don C.; Morton, D. Holmes; Strauss, Kevin A.

2013-01-01

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain amino acid metabolism presenting with neonatal encephalopathy, episodic metabolic decompensation, and chronic amino acid imbalances. Dietary management enables survival and reduces risk of acute crises. Liver transplantation has emerged as an effective way to eliminate acute decompensation risk. Psychiatric illness is a reported MSUD complication, but has not been well characterized and remains poorly understood. We report the prevalence and characteristics of neuropsychiatric problems among 37 classical MSUD patients (ages 5–35 years, 26 on dietary therapy, 11 after liver transplantation) and explore their underlying mechanisms. Compared with 26 age-matched controls, MSUD patients were at higher risk for disorders of cognition, attention, and mood. Using quantitative proton magnetic resonance spectroscopy, we found lower brain glutamate, N-acetylaspartate (NAA), and creatine concentrations in MSUD patients, which correlated with specific neuropsychiatric outcomes. Asymptomatic neonatal course and stringent longitudinal biochemical control proved fundamental to optimizing long-term mental health. Neuropsychiatric morbidity and neurochemistry were similar among transplanted and nontransplanted MSUD patients. In conclusion, amino acid dysregulation results in aberrant neural networks with neurochemical deficiencies that persist after transplant and correlate with neuropsychiatric morbidities. These findings may provide insight into general mechanisms of psychiatric illness. PMID:23478409

Two novel compound heterozygous mutations in the BCKDHB gene that cause the intermittent form of maple syrup urine disease.

PubMed

Guo, Yi; Liming, Liu; Jiang, Li

2015-12-01

Intermittent maple syrup urine disease (MSUD) is a potentially life-threatening metabolic disorder caused by a deficiency of branched chain α-ketoacid dehydrogenase (BCKD) complex. In contrast to classic MSUD, children with the intermittent form usually have an atypical clinical manifestation. Here, we describe the presenting symptoms and clinical course of a Chinese boy with intermittent MSUD. Mutation analysis identified two previously unreported mutations in exon 7 of the BCKDHB gene: c.767A > G (p.Y256C) and c.768C > G (p.Y256X); the parents were each heterozygous for one of these mutations. In silico analysis predicted Y256C probably affects protein structure; Y256X leads to a premature stop codon. This case demonstrates intermittent MSUD should be suspected in cases with symptoms of recurrent encephalopathy, especially ataxia or marked drowsiness, which usually present after the neonatal period and in conjunction with infection. symmetrical basal ganglia damage but normal myelination in the posterior limb will assist differential diagnosis; alloisoleucine is a useful diagnostic marker and mutation analysis may be of prognostic value. These novel mutations Y256C and Y256X result in the clinical manifestation of a variant form of MSUD, expanding the mutation spectrum of this disease.

Domino liver transplantation in maple syrup urine disease: a case report and review of the literature.

PubMed

Badell, I R; Hanish, S I; Hughes, C B; Hewitt, W R; Chung, R T; Spivey, J R; Knechtle, S J

2013-03-01

Improved outcomes have expanded the indications for liver transplantation, thus aggravating the already limited supply of donor organs. Domino liver transplantation (DLT) has been one strategy to augment the supply of donor organs in cases of inborn errors of metabolism. One such disease is maple syrup urine disease (MSUD), an inherited disorder of branched-chain amino acid (BCAA) metabolism. We report on the transplantation of a deceased donor liver into a patient with MSUD, and the sequential transplantation of the explanted liver into a patient with hemophilia A, HIV, hepatitis C, and a low priority on the transplant waiting list. At 30 months, the MSUD recipient has had significant correction of BCAA metabolism on a protein-unrestricted diet and no progression of neuropsychiatric symptoms. The DLT recipient has been cured of hemophilia and has normal BCAA homeostasis. This case provides further evidence that elective orthotopic liver transplantation for MSUD attenuates the disease with restoration of BCAA metabolism, and that DLT in this setting can achieve excellent results in ESLD patients. It is possible that domino grafts from patients with MSUD could be used in more conventional recipients, but additional studies and longer-term outcomes are needed to determine the validity of DLT in MSUD. Copyright © 2013 Elsevier Inc. All rights reserved.

A ten-year regional assessment of sugar maple mortality

Treesearch

Douglas C. Allen; Andrew W. Molloy; Robert R. Cooke; Bruce A. Pendrel

1999-01-01

The North American Maple Project (NAMP) monitored annual sugar maple mortality from 1988 through 1997 in Maine, Massachusetts, New Brunswick/Nova Scotia, New Hampshire, New York. Ontario, Quebec, Vermont, and Wisconsin. Annual mortality in Minnesota, Ohio and Pennsylvania was evaluated for 1992 through 1997. When data from the dominant/codominant and intermediate/...

Co-existence of phenylketonuria either with maple syrup urine disease or Sandhoff disease in two patients from Iran: emphasizing the role of consanguinity.

PubMed

Abiri, Maryam; Talebi, Saeed; Uitto, Jouni; Youssefian, Leila; Vahidnezhad, Hassan; Shirzad, Tina; Salehpour, Shadab; Zeinali, Sirous

2016-10-01

Most inborn errors of metabolism (IEMs) are inherited in an autosomal recessive manner. IEMs are one of the major concerns in Iran due to its extensive consanguineous marriages. Herein, we report two patients with two co-existent IEMs: a girl affected by classic phenylketonuria (PKU) and maple syrup urine disease (MSUD) and a male patient affected with Sandhoff disease and PKU, where Sandhoff disease was suspected due to the presence of a cherry-red spot in the eyes at 6 months which is unrelated to PKU. Sequencing of candidate genes in the first patient revealed one novel and three recurrent compound heterozygous mutations of p.Ser231Pro and p.Ala300Ser in the PAH gene and p.Glu330Lys and p.Arg170Cys mutations in the BCKDHB gene. Genetic testing results in the second patient showed previously reported homozygous mutations of p.Arg261Gln in the PAH and p.Arg533Cys mutation in the HEXB gene. Genetic testing confirmed the clinical diagnosis of both diseases in both patients. To the best of our knowledge; this is the first report of the co-existence of two distinct genetic disorders in two individuals from Iran. Co-existent different IEMs in patients complicated the clinical diagnosis and management of the diseases.

Selected reaction monitoring as an effective method for reliable quantification of disease-associated proteins in maple syrup urine disease.

PubMed

Fernández-Guerra, Paula; Birkler, Rune I D; Merinero, Begoña; Ugarte, Magdalena; Gregersen, Niels; Rodríguez-Pombo, Pilar; Bross, Peter; Palmfeldt, Johan

2014-09-01

Selected reaction monitoring (SRM) mass spectrometry can quantitatively measure proteins by specific targeting of peptide sequences, and allows the determination of multiple proteins in one single analysis. Here, we show the feasibility of simultaneous measurements of multiple proteins in mitochondria-enriched samples from cultured fibroblasts from healthy individuals and patients with mutations in branched-chain α-ketoacid dehydrogenase (BCKDH) complex. BCKDH is a mitochondrial multienzyme complex and its defective activity causes maple syrup urine disease (MSUD), a rare but severe inherited metabolic disorder. Four different genes encode the catalytic subunits of BCKDH: E1α (BCKDHA), E1β (BCKDHB), E2 (DBT), and E3 (DLD). All four proteins were successfully quantified in healthy individuals. However, the E1α and E1β proteins were not detected in patients carrying mutations in one of those genes, whereas mRNA levels were almost unaltered, indicating instability of E1α and E1β monomers. Using SRM we elucidated the protein effects of mutations generating premature termination codons or misfolded proteins. SRM is a complement to transcript level measurements and a valuable tool to shed light on molecular mechanisms and on effects of pharmacological therapies at protein level. SRM is particularly effective for inherited disorders caused by multiple proteins such as defects in multienzyme complexes.

Two novel mutations in the BCKDHB gene that cause maple syrup urine disease.

PubMed

Han, Bingjuan; Han, Bingchao; Guo, Bin; Liu, Yingxia; Cao, Zhiyang

2018-01-06

Maple syrup urine disease (MSUD) is a rare metabolic disorder of autosomal recessive inheritance caused by decreased activity of branched-chain α-ketoacid dehydrogenase complex (BCKD). Mutations in the three genes (BCKDHA, BCKDHB and DBT) are associated with MSUD. Here, we describe the presenting symptoms, clinical course and gene mutation analysis of a Chinese boy with MSUD. Plasma amino acid analysis was performed by tandem mass spectrometry and the levels of organic acids in urine were measured with gas chromatography-mass spectrometry. The BCKDHB gene was sequenced by Sanger method. Furthermore, the significance of the novel mutations was predicted by Polyphen and Mutationtaster. After diagnosis, the patient was fed with protein-restricted diet to reduce intake of BCAA and was treated with l -carnitine. Metabolic parameters, clinical presentation and mental development were followed up. The patient was diagnosed as MSUD. Two novel BCKDHB mutations (c.523 T > C and c.478-25_552del100) were identified. In silico analysis predicted that the two mutations were "disease causing". The boy tolerated the treatment well and had symptomatic improvement. He presented with mild hypotonia and had nearly normal DQ scores at the age of 10 months. The two novel mutations resulted in the clinical manifestations of MSUD. Our results may reflect the heterogeneity of the pathogenic variants found in patients with MSUD. Copyright © 2018. Published by Elsevier B.V.

Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.

PubMed

Mescka, Caroline Paula; Wayhs, Carlos Alberto Yasin; Vanzin, Camila Simioni; Biancini, Giovana Brondani; Guerreiro, Gilian; Manfredini, Vanusa; Souza, Carolina; Wajner, Moacir; Dutra-Filho, Carlos Severo; Vargas, Carmen Regla

2013-02-01

Maple syrup urine disease (MSUD) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids (BCAA) leucine, isoleucine, valine and the corresponding branched-chain α-keto acids. This disorder is clinically characterized by ketoacidosis, seizures, coma, psychomotor delay and mental retardation whose pathophysiology is not completely understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. l-Carnitine (l-Car) plays a central role in the cellular energy metabolism because it transports long-chain fatty acids for oxidation and ATP generation. In recent years many studies have demonstrated the antioxidant role of this compound. In this work, we investigated the effect of BCAA-restricted diet supplemented or not with l-Car on lipid peroxidation and in protein oxidation in MSUD patients. We found a significant increase of malondialdehyde and of carbonyl content in plasma of MSUD patients under BCAA-restricted diet compared to controls. Furthermore, patients under BCAA-restricted diet plus l-Car supplementation presented a marked reduction of malondialdehyde content in relation to controls, reducing the lipid peroxidation. In addition, free l-Car concentrations were negatively correlated with malondialdehyde levels. Our data show that l-Car may have an antioxidant effect, protecting against the lipid peroxidation and this could represent an additional therapeutic approach to the patients affected by MSUD. Copyright © 2012 ISDN. Published by Elsevier Ltd. All rights reserved.

Maple syrup urine disease in Brazil: a panorama of the last two decades.

PubMed

Herber, Silvani; Schwartz, Ida Vanessa D; Nalin, Tatiéle; Oliveira Netto, Cristina Brinkmann; Camelo Junior, José Simon; Santos, Mara Lúcia; Ribeiro, Erlane Marques; Schüler-Faccini, Lavinia; Souza, Carolina Fischinger Moura de

2015-01-01

To characterize a sample of Brazilian patients with maple syrup urine disease (MSUD) diagnosed between 1992 and 2011. In this retrospective study, patients were identified through a national reference laboratory for the diagnosis of MSUD and through contact with other medical genetics services across Brazil. Data were collected by means of a chart review. Eighty-three patients from 75 families were enrolled in the study (median age, 3 years; interquartile range [IQR], 0.57-7). Median age at onset of symptoms was 10 days (IQR 5-30), whereas median age at diagnosis was 60 days (IQR 29-240, p=0.001). Only three (3.6%) patients were diagnosed before the onset of clinical manifestations. A comparison between patients with (n=12) and without (n=71) an early diagnosis shows that early diagnosis is associated with the presence of positive family history and decreased prevalence of clinical manifestations at the time of diagnosis, but not with a better outcome. Overall, 98.8% of patients have some psychomotor or neurodevelopmental delay. In Brazil, patients with MSUD are usually diagnosed late and exhibit neurological involvement and poor survival even with early diagnosis. We suggest that specific public policies for diagnosis and treatment of MSUD should be developed and implemented in the country. Copyright © 2014 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Serum Markers of Neurodegeneration in Maple Syrup Urine Disease.

PubMed

Scaini, Giselli; Tonon, Tássia; de Souza, Carolina F Moura; Schuk, Patricia F; Ferreira, Gustavo C; Neto, Joao Seda; Amorin, Tatiana; Schwartz, Ida Vanessa D; Streck, Emilio L

2017-09-01

Maple syrup urine disease (MSUD) is an inherited disorder caused by deficient activity of the branched-chain α-keto acid dehydrogenase complex involved in the degradation pathway of branched-chain amino acids (BCAAs) and their respective α-keto-acids. Patients affected by MSUD present severe neurological symptoms and brain abnormalities, whose pathophysiology is poorly known. However, preclinical studies have suggested alterations in markers involved with neurodegeneration. Because there are no studies in the literature that report the neurodegenerative markers in MSUD patients, the present study evaluated neurodegenerative markers (brain-derived neurotrophic factor (BDNF), cathepsin D, neural cell adhesion molecule (NCAM), plasminogen activator inhibitor-1 total (PAI-1 (total)), platelet-derived growth factor AA (PDGF-AA), PDGF-AB/BB) in plasma from 10 MSUD patients during dietary treatment. Our results showed a significant decrease in BDNF and PDGF-AA levels in MSUD patients. On the other hand, NCAM and cathepsin D levels were significantly greater in MSUD patients compared to the control group, while no significant changes were observed in the levels of PAI-1 (total) and PDGF-AB/BB between the control and MSUD groups. Our data show that MSUD patients present alterations in proteins involved in the neurodegenerative process. Thus, the present findings corroborate previous studies that demonstrated that neurotrophic factors and lysosomal proteases may contribute, along with other mechanisms, to the intellectual deficit and neurodegeneration observed in MSUD.

Clinical characteristics and mutation analysis of five Chinese patients with maple syrup urine disease.

PubMed

Li, Xiaomei; Yang, Yali; Gao, Qing; Gao, Min; Lv, Yvqiang; Dong, Rui; Liu, Yi; Zhang, Kaihui; Gai, Zhongtao

2018-06-01

Maple syrup urine disease (MSUD) is an autosomal recessive disorder affecting branched-chain amino acids (BCAAs) metabolism and caused by a defect in the thiamine-dependent enzyme branched chain α-ketoacid dehydrogenase (BCKD) with subsequent accumulation of BCAAs and corresponding branched-chain keto acids (BCKAs) metabolites. Presently, at least 4 genes of BCKDHA, BCKDHB, DLD and DBT have been reported to cause MSUD. Furthermore, more than 265 mutations have been identified as the cause across different populations worldwide. Some studies have reported the data of gene mutations in Chinese people with MSUD. In this study, we present clinical characteristics and mutational analyses in five Chinese Han child with MSUD, which had been screened out by tandem mass spectrometry detection of amino acids in blood samples. High-throughput sequencing, Sanger sequence and real-time qualitative PCR were performed to detect and verify the genetic mutations. Six different novel genetic variants were validated in BCKDHB gene and BCKDHA gene, including c.523 T > C, c.659delA, c.550delT, c.863G > A and two gross deletions. Interestingly, 3 cases had identical mutation of BCKDHB gene (c.659delA). We predicted the pathogenicity and analyzed the clinical characteristics. The identification of these mutations in this study further expands the mutation spectrum of MSUD and contributes to prenatal molecular diagnosis of MSUD.

Identification of six novel mutations in Iranian patients with maple syrup urine disease and their in silico analysis.

PubMed

Abiri, Maryam; Karamzadeh, Razieh; Karimipoor, Morteza; Ghadami, Shirin; Alaei, Mohammad Reza; Bagheri, Samira Dabagh; Bagherian, Hamideh; Setoodeh, Aria; Noori-Daloii, Mohammad Reza; Sirous Zeinali

2016-04-01

Maple syrup urine disease (MSUD) is a rare inborn error of branched-chain amino acid metabolism. The disease prevalence is higher in populations with elevated rate of consanguineous marriages such as Iran. Different types of disease causing mutations have been previously reported in BCKDHA, BCKDHB, DBT and DLD genes known to be responsible for MSUD phenotype. In this study, two sets of multiplex polymorphic STR (Short Tandem Repeat) markers linked to the above genes were used to aid in homozygosity mapping in order to find probable pathogenic change(s) in the studied families. The families who showed homozygote haplotype for the BCKDHA gene were subsequently sequenced. Our findings showed that exons 2, 4 and 6 contain most of the mutations which are novel. The changes include two single nucleotide deletion (i.e. c. 143delT and c.702delT), one gross deletion covering the whole exon four c.(375+1_376-1)_(8849+1_885-1), two splice site changes (c.1167+1G>T, c. 288+1G>A), and one point mutation (c.731G>A). Computational approaches were used to analyze these two novel mutations in terms of their impact on protein structure. Computational structural modeling indicated that these mutations might affect structural stability and multimeric assembly of branched-chain α-keto acid dehydrogenase complex (BCKDC). Copyright © 2016. Published by Elsevier B.V.

Selected reaction monitoring as an effective method for reliable quantification of disease-associated proteins in maple syrup urine disease

PubMed Central

Fernández-Guerra, Paula; Birkler, Rune I D; Merinero, Begoña; Ugarte, Magdalena; Gregersen, Niels; Rodríguez-Pombo, Pilar; Bross, Peter; Palmfeldt, Johan

2014-01-01

Selected reaction monitoring (SRM) mass spectrometry can quantitatively measure proteins by specific targeting of peptide sequences, and allows the determination of multiple proteins in one single analysis. Here, we show the feasibility of simultaneous measurements of multiple proteins in mitochondria-enriched samples from cultured fibroblasts from healthy individuals and patients with mutations in branched-chain α-ketoacid dehydrogenase (BCKDH) complex. BCKDH is a mitochondrial multienzyme complex and its defective activity causes maple syrup urine disease (MSUD), a rare but severe inherited metabolic disorder. Four different genes encode the catalytic subunits of BCKDH: E1α (BCKDHA), E1β (BCKDHB), E2 (DBT), and E3 (DLD). All four proteins were successfully quantified in healthy individuals. However, the E1α and E1β proteins were not detected in patients carrying mutations in one of those genes, whereas mRNA levels were almost unaltered, indicating instability of E1α and E1β monomers. Using SRM we elucidated the protein effects of mutations generating premature termination codons or misfolded proteins. SRM is a complement to transcript level measurements and a valuable tool to shed light on molecular mechanisms and on effects of pharmacological therapies at protein level. SRM is particularly effective for inherited disorders caused by multiple proteins such as defects in multienzyme complexes. PMID:25333063

A Stocking Guide for Allegheny Hardwoods and Its Use in Controlling Intermediate Cuttings

Treesearch

Benjamin A. Roach

1977-01-01

A stocking guide for stands of Allegheny hardwoods (sugar maple or sugar maple-beech with varying admixtures of black cherry, red maple, white ash, sweet birch, and other species) on the Allegheny Plateau in northwestern Pennsylvania. Included are procedures for evaluating stocking and stand conditions, thinning even-aged stands, determining minimum residual stocking,...

Crown releasing of red maple poles to shorten high-quality sawlog rotations

Treesearch

Gayne G. Erdmann; Ralph M., Jr. Peterson; Robert R. Orberg

1985-01-01

The effects of six crown-release treatments on growth and bole quality development of 54-year-old dominant, codominant, and intermediate red maples (Acer rubrum L.) were evaluated on a good red maple site (site index = 19.3 mat 50 years) in upper Michigan. Results showed that crown release stimulated the growth of dominants, codominants, and strong...

Oxidative stress as a mechanism of added sugar-induced cardiovascular disease.

PubMed

Prasad, Kailash; Dhar, Indu

2014-12-01

Added sugars comprising of table sugar, brown sugar, corn syrup, maple syrup, honey, molasses, and other sweeteners in the prepared processed foods and beverages have been implicated in the pathophysiology of cardiovascular diseases. This article deals with the reactive oxygen species (ROS) as a mechanism of sugar-induced cardiovascular diseases. There is an association between the consumption of high levels of serum glucose with cardiovascular diseases. Various sources of sugar-induced generation of ROS, including mitochondria, nicotinamide adenine dinucleotide phosphate-oxidase, advanced glycation end products, insulin, and uric acid have been discussed. The mechanism by which ROS induce the development of atherosclerosis, hypertension, peripheral vascular disease, coronary artery disease, cardiomyopathy, heart failure, and cardiac arrhythmias have been discussed in detail. In conclusion, the data suggest that added sugars induce atherosclerosis, hypertension, peripheral vascular disease, coronary artery disease, cardiomyopathy, heart failure, and cardiac arrhythmias and that these effects of added sugars are mediated through ROS.

How fresh is maple syrup? Sugar maple trees mobilize carbon stored several years previously during early springtime sap-ascent.

PubMed

Muhr, Jan; Messier, Christian; Delagrange, Sylvain; Trumbore, Susan; Xu, Xiaomei; Hartmann, Henrik

2016-03-01

While trees store substantial amounts of nonstructural carbon (NSC) for later use, storage regulation and mobilization of stored NSC in long-lived organisms like trees are still not well understood. At two different sites with sugar maple (Acer saccharum), we investigated ascending sap (sugar concentration, δ(13) C, Δ(14) C) as the mobilized component of stored stem NSC during early springtime. Using the bomb-spike radiocarbon approach we were able to estimate the average time elapsed since the mobilized carbon (C) was originally fixed from the atmosphere and to infer the turnover time of stem storage. Sites differed in concentration dynamics and overall δ(13) C, indicating different growing conditions. The absence of temporal trends for δ(13) C and Δ(14) C indicated sugar mobilization from a well-mixed pool with average Δ(14) C consistent with a mean turnover time (TT) of three to five years for this pool, with only minor differences between the sites. Sugar maple trees hence appear well buffered against single or even several years of negative plant C balance from environmental stress such as drought or repeated defoliation by insects. Manipulative investigations (e.g. starvation via girdling) combined with Δ(14) C measurements of this mobilized storage pool will provide further new insights into tree storage regulation and functioning. © 2015 The Authors. New Phytologist © 2015 New Phytologist Trust.

Evaluation of plasma biomarkers of inflammation in patients with maple syrup urine disease.

PubMed

Scaini, Giselli; Tonon, Tássia; Moura de Souza, Carolina F; Schuck, Patricia F; Ferreira, Gustavo C; Quevedo, João; Neto, João Seda; Amorim, Tatiana; Camelo, Jose S; Margutti, Ana Vitoria Barban; Hencke Tresbach, Rafael; Sperb-Ludwig, Fernanda; Boy, Raquel; de Medeiros, Paula F V; Schwartz, Ida Vanessa D; Streck, Emilio Luiz

2018-05-08

Maple syrup urine disease (MSUD) is an autosomal recessive inherited disorder that affects branched-chain amino acid (BCAA) catabolism and is associated with acute and chronic brain dysfunction. Recent studies have shown that inflammation may be involved in the neuropathology of MSUD. However, these studies have mainly focused on single or small subsets of proteins or molecules. Here we performed a case-control study, including 12 treated-MSUD patients, in order to investigate the plasmatic biomarkers of inflammation, to help to establish a possible relationship between these biomarkers and the disease. Our results showed that MSUD patients in treatment with restricted protein diets have high levels of pro-inflammatory cytokines [IFN-γ, TNF-α, IL-1β and IL-6] and cell adhesion molecules [sICAM-1 and sVCAM-1] compared to the control group. However, no significant alterations were found in the levels of IL-2, IL-4, IL-5, IL-7, IL-8, and IL-10 between healthy controls and MSUD patients. Moreover, we found a positive correlation between number of metabolic crisis and IL-1β levels and sICAM-1 in MSUD patients. In conclusion, our findings in plasma of patients with MSUD suggest that inflammation may play an important role in the pathogenesis of MSUD, although this process is not directly associated with BCAA blood levels. Overall, data reported here are consistent with the working hypothesis that inflammation may be involved in the pathophysiological mechanism underlying the brain damage observed in MSUD patients.

Clinical and biochemical profiles of maple syrup urine disease in malaysian children.

PubMed

Yunus, Z Md; Kamaludin, Dp Abg; Mamat, M; Choy, Y S; Ngu, Lh

2012-01-01

Maple Syrup Urine Disease (MSUD) is an autosomal recessive disorder caused by defects in the branched-chain α-ketoacid dehydrogenase complex resulting in accumulation of branched-chain amino acids (BCAAs) and corresponding branched-chain ketoacids (BCKAs) in tissues and plasma, which are neurotoxic. Early diagnosis and subsequent nutritional modification management can reduce the morbidity and mortality. Prior to 1990s, the diagnosis of MSUD and other inborn errors of metabolism (IEM) in Malaysia were merely based on clinical suspicion and qualitative one-dimensional thin layer chromatography technique. We have successfully established specific laboratory diagnostic techniques to diagnose MSUD and other IEM. We described here our experience in performing high-risk screening for IEM in Malaysia from 1999 to 2006. We analysed the clinical and biochemical profiles of 25 patients with MSUD. A total of 12,728 plasma and urine samples from patients suspected of having IEM were received from physicians all over Malaysia. Plasma amino acids quantitation using fully automated amino acid analyzer and identification of urinary organic acids using Gas Chromatography Mass Spectrometry (GCMS). Patients' clinical information were obtained from the request forms and case records Results: Twenty-five patients were diagnosed MSUD. Nineteen patients (76%) were affected by classical MSUD, whereas six patients had non-classical MSUD. Delayed diagnosis was common among our case series, and 80% of patients had survived with treatment with mild-to-moderate learning difficulties. Our findings suggested that MSUD is not uncommon in Malaysia especially among the Malay and early laboratory diagnosis is crucial.

Identification of three novel mutations by studying the molecular genetics of Maple Syrup Urine Disease (MSUD) in the Lebanese population.

PubMed

Tabbouche, Omar; Saker, Amer; Mountain, Harry

2014-01-01

Maple Syrup Urine Disease (MSUD) is a genetically heterogeneous metabolic disorder that is transmitted in an autosomal recessive manner. According to clinical data, MSUD prevalence in Lebanon is expected to be higher than the International prevalence because of consanguineous marriage. Novel mutations are still getting detected by using DNA sequencing for mutation analysis in MSUD patients. In the current study, we have extracted DNA from Lebanese MSUD patients in order to amplify the exonic and flanking intronic regions of the genes implicated in MSUD ( BCKDHA , BCKDHB , and DBT ) and sequenced the resultant amplified products to assess the molecular genetics of MSUD in the Lebanese population studied. All of the mutations identified occurred in the homozygous state, which reflects the high rate of consanguineous marriage in Lebanon. In the current study, we have identified one previously cited mutation and three novel mutations not previously described in the scientific literature. The identified mutations were distributed as follows: three patients (60%) had two nucleotide substitutions in the DBT gene (c.224G>A and c.1430T>G), one patient (20%) had a gross deletion in the BCKDHA gene (c.488_1167+3del), and one patient (20%) had a small deletion in the BCKDHB gene (c.92_102del). The majority of the mutations identified in the Lebanese MSUD patients occurred in the DBT gene. Consanguineous marriage is a major risk factor for the prevalence of MSUD in Lebanon.

Successful pregnancy in maple syrup urine disease: a case report and review of the literature.

PubMed

Grünert, Sarah Catharina; Rosenbaum-Fabian, Stefanie; Schumann, Anke; Schwab, Karl Otfried; Mingirulli, Nadja; Spiekerkoetter, Ute

2018-05-12

Maple syrup urine disease (MSUD) is an autosomal recessive disorder of branched-chain amino acid metabolism. Patients with MSUD are at risk of life-threatening metabolic decompensations with ketoacidosis and encephalopathy. These episodes are often triggered by physiological stress. Only few cases of pregnancies in MSUD mothers have been reported so far. We present the favorable outcome of a pregnancy in a woman with classical MSUD. She presented in the metabolic outpatient clinic in week 7 of gestation. Branched-chain amino acid concentrations were measured at least weekly to adjust dietary leucine intake. Despite excellent compliance, leucine concentrations frequently exceeded the target value of < 300 μmol/L during the first trimester. From the second trimester until delivery, protein and leucine intake increased continuously to about threefold compared to pre-pregnancy values. To maximize patient safety during delivery and the postpartum period, a detailed plan including peripartal infusion therapy, dietary recommendations and monitoring parameters was developed. Primary Caesarean section was performed in week 38 of gestation, and the patient gave birth to a healthy girl. Lactation was successfully implemented. Leucine levels were maintained within the target range throughout the complete postpartum period. In addition to our case, we give an overview about all cases of pregnancies in MSUD mothers published so far. Management of pregnancy, delivery, postpartum period and lactation may be challenging in patients with MSUD. Careful monitoring and interdisciplinary collaboration is essential to minimize the risk of metabolic crisis, especially after delivery.

Mutation of zebrafish dihydrolipoamide branched-chain transacylase E2 results in motor dysfunction and models maple syrup urine disease

PubMed Central

Friedrich, Timo; Lambert, Aaron M.; Masino, Mark A.; Downes, Gerald B.

2012-01-01

SUMMARY Analysis of zebrafish mutants that demonstrate abnormal locomotive behavior can elucidate the molecular requirements for neural network function and provide new models of human disease. Here, we show that zebrafish quetschkommode (que) mutant larvae exhibit a progressive locomotor defect that culminates in unusual nose-to-tail compressions and an inability to swim. Correspondingly, extracellular peripheral nerve recordings show that que mutants demonstrate abnormal locomotor output to the axial muscles used for swimming. Using positional cloning and candidate gene analysis, we reveal that a point mutation disrupts the gene encoding dihydrolipoamide branched-chain transacylase E2 (Dbt), a component of a mitochondrial enzyme complex, to generate the que phenotype. In humans, mutation of the DBT gene causes maple syrup urine disease (MSUD), a disorder of branched-chain amino acid metabolism that can result in mental retardation, severe dystonia, profound neurological damage and death. que mutants harbor abnormal amino acid levels, similar to MSUD patients and consistent with an error in branched-chain amino acid metabolism. que mutants also contain markedly reduced levels of the neurotransmitter glutamate within the brain and spinal cord, which probably contributes to their abnormal spinal cord locomotor output and aberrant motility behavior, a trait that probably represents severe dystonia in larval zebrafish. Taken together, these data illustrate how defects in branched-chain amino acid metabolism can disrupt nervous system development and/or function, and establish zebrafish que mutants as a model to better understand MSUD. PMID:22046030

DNA Carrier Testing and Newborn Screening for Maple Syrup Urine Disease in Old Order Mennonite Communities

PubMed Central

Carleton, Stephanie M.; Peck, Dawn S.; Grasela, Julie; Dietiker, Kristin L.

2010-01-01

Maple syrup urine disease (MSUD) is an inherited metabolic disorder caused by mutations in the branched chain α-keto acid dehydrogenase complex. Worldwide incidence of MSUD is 1:225,000 live births. However, within Old Order Mennonite communities, the incidence is 1:150 live births and results from a common tyrosine to asparagine substitution (Y438N) in the E1α subunit of branched chain α-keto acid dehydrogenase. We developed a new DNA diagnostic assay utilizing TaqMan® technology and compared its efficacy, sensitivity, and duration with an existing polymerase chain reaction–restriction fragment length polymorphism (PCR-RFLP) assay. Carrier testing was performed by both TaqMan technology and PCR-RFLP on DNA isolated from buccal swabs of 160 individuals as well as from buccal swabs and blood spots of nine at-risk newborns; assay time, sensitivity, and reliability were also evaluated. The TaqMan assay, like the PCR-RFLP assay, accurately determined Y438N E1α allele status. However, the TaqMan assay appeared (1) more sensitive than the PCR-RFLP assay, requiring 10-fold less DNA (10 ng) to reliably determine genotype status and (2) faster, reducing the assay time required for diagnosis from ∼12 to 5 h. TaqMan technology allowed more rapid DNA diagnoses of MSUD in the neonate, thereby reducing the likelihood of neurological impairment while enhancing health and prognosis for affected infants. PMID:20136525

Liver Transplantation for Classical Maple Syrup Urine Disease: Long-Term Follow-Up in 37 Patients and Comparative United Network for Organ Sharing Experience

PubMed Central

Mazariegos, George V.; Morton, D. Holmes; Sindhi, Rakesh; Soltys, Kyle; Nayyar, Navdeep; Bond, Geoffrey; Shellmer, Diana; Shneider, Benjamin; Vockley, Jerry; Strauss, Kevin A.

2012-01-01

Objective To assess clinical and neurocognitive function in children who have undergone liver transplantation for classical maple syrup urine disease (MSUD). Study design A total of 35 patients with classical MSUD (age 9.9 ± 7.9 years) underwent liver transplantation between 2004 and 2009. Six patients donated their liver to recipients without MSUD (“domino” transplant). We analyzed clinical outcomes for our cohort and 17 additional cases from the national United Network for Organ Sharing registry; 33 patients completed IQ and adaptive testing before transplantation, and 14 completed testing 1 year later. Results Patient and graft survival were 100% at 4.5 ± 2.2 years of follow-up. Liver function was normal in all patients. Branched-chain amino acid levels were corrected within hours after surgery and remained stable, with leucine tolerance increasing more than 10-fold. All domino transplant recipients were alive and well with normal branched-chain amino acid homeostasis at the time of this report. Patient and graft survival for all 54 patients with MSUD undergoing liver transplantation in the United States during this period were 98%and 96%, respectively. One-third of our patients were mentally impaired (IQ ≤ 70) before transplantation, with no statistically significant change 1 year later. Conclusion Liver transplantation is an effective long-term treatment for classical MSUD and may arrest brain damage, but will not reverse it. PMID:21839471

Successful domino liver transplantation in maple syrup urine disease using a related living donor

PubMed Central

Feier, F.H.; Miura, I.K.; Fonseca, E.A.; Porta, G.; Pugliese, R.; Porta, A.; Schwartz, I.V.D.; Margutti, A.V.B.; Camelo, J.S.; Yamaguchi, S.N.; Taveira, A.T.; Candido, H.; Benavides, M.; Danesi, V.; Guimaraes, T.; Kondo, M.; Chapchap, P.; Seda, J.

2014-01-01

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient's mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD. PMID:24770567

A novel regulatory defect in the branched-chain α-keto acid dehydrogenase complex due to a mutation in the PPM1K gene causes a mild variant phenotype of maple syrup urine disease.

PubMed

Oyarzabal, Alfonso; Martínez-Pardo, Mercedes; Merinero, Begoña; Navarrete, Rosa; Desviat, Lourdes R; Ugarte, Magdalena; Rodríguez-Pombo, Pilar

2013-02-01

This article describes a hitherto unreported involvement of the phosphatase PP2Cm, a recently described member of the branched-chain α-keto acid dehydrogenase (BCKDH) complex, in maple syrup urine disease (MSUD). The disease-causing mutation was identified in a patient with a mild variant phenotype, involving a gene not previously associated with MSUD. SNP array-based genotyping showed a copy-neutral homozygous pattern for chromosome 4 compatible with uniparental isodisomy. Mutation analysis of the candidate gene, PPM1K, revealed a homozygous c.417_418delTA change predicted to result in a truncated, unstable protein. No PP2Cm mutant protein was detected in immunocytochemical or Western blot expression analyses. The transient expression of wild-type PPM1K in PP2Cm-deficient fibroblasts recovered 35% of normal BCKDH activity. As PP2Cm has been described essential for cell survival, apoptosis and metabolism, the impact of its deficiency on specific metabolic stress variables was evaluated in PP2Cm-deficient fibroblasts. Increases were seen in ROS levels along with the activation of specific stress-signaling MAP kinases. Similar to that described for the pyruvate dehydrogenase complex, a defect in the regulation of BCKDH caused the aberrant metabolism of its substrate, contributing to the patient's MSUD phenotype--and perhaps others. © 2012 WILEY PERIODICALS, INC.

Evaluation of acetylcholinesterase in an animal model of maple syrup urine disease.

PubMed

Scaini, Giselli; de Rochi, Natália; Jeremias, Isabela C; Deroza, Pedro F; Zugno, Alexandra I; Pereira, Talita C B; Oliveira, Giovanna M T; Kist, Luiza W; Bogo, Maurício R; Schuck, Patrícia F; Ferreira, Gustavo C; Streck, Emilio L

2012-04-01

Maple syrup urine disease is an inherited metabolic disease predominantly characterized by neurological dysfunction. However, the mechanisms underlying the neuropathology of this disease are still not defined. Therefore, the aim of this study was to investigate the effect of acute and chronic administration of a branched-chain amino acids (BCAA) pool (leucine, isoleucine, and valine) on acetylcholinesterase (AChE) activity and gene expression in the brain and serum of rats and to assess if antioxidant treatment prevented the alterations induced by BCAA administration. Our results show that the acute administration of a BCAA pool in 10- and 30-day-old rats increases AChE activity in the cerebral cortex, striatum, hippocampus, and serum. Moreover, chronic administration of the BCAA pool also increases AChE activity in the structures studied, and antioxidant treatment prevents this increase. In addition, we show a significant decrease in the mRNA expression of AChE in the hippocampus following acute administration in 10- and 30-day-old rats. On the other hand, AChE expression increased significantly after chronic administration of the BCAA pool. Interestingly, the antioxidant treatment was able to prevent the increased AChE activity without altering AChE expression. In conclusion, the results from the present study demonstrate a marked increase in AChE activity in all brain structures following the administration of a BCAA pool. Moreover, the increased AChE activity is prevented by the coadministration of N-acetylcysteine and deferoxamine as antioxidants.

A validated fast difference spectrophotometric method for 5-hydroxymethyl-2-furfural (HMF) determination in corn syrups.

PubMed

de Andrade, Jucimara Kulek; de Andrade, Camila Kulek; Komatsu, Emy; Perreault, Hélène; Torres, Yohandra Reyes; da Rosa, Marcos Roberto; Felsner, Maria Lurdes

2017-08-01

Corn syrups, important ingredients used in food and beverage industries, often contain high levels of 5-hydroxymethyl-2-furfural (HMF), a toxic contaminant. In this work, an in house validation of a difference spectrophotometric method for HMF analysis in corn syrups was developed using sophisticated statistical tools by the first time. The methodology showed excellent analytical performance with good selectivity, linearity (R 2 =99.9%, r>0.99), accuracy and low limits (LOD=0.10mgL -1 and LOQ=0.34mgL -1 ). An excellent precision was confirmed by repeatability (RSD (%)=0.30) and intermediate precision (RSD (%)=0.36) estimates and by Horrat value (0.07). A detailed study of method precision using a nested design demonstrated that variation sources such as instruments, operators and time did not interfere in the variability of results within laboratory and consequently in its intermediate precision. The developed method is environmentally friendly, fast, cheap and easy to implement resulting in an attractive alternative for corn syrups quality control in industries and official laboratories. Copyright © 2017 Elsevier Ltd. All rights reserved.

Growth and Survival of Northern Hardwood Sprouts After Burning

Treesearch

Donald A. Perala

1974-01-01

Root collar sprouting of nine hardwoods was measured annually after a prescribed burn. Basswood, red oak, and paper birch were the most vigorous sprouters; sugar maple and yellow birch the least; and American elm, bur oak, ironwood and red maple were intermediate. Parent tree diameter influenced spreading.

Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.

PubMed

Wynn, R M; Chuang, J L; Sansaricq, C; Mandel, H; Chuang, D T

2001-09-28

Maple syrup urine disease (MSUD) is a metabolic disorder associated with often-fatal ketoacidosis, neurological derangement, and mental retardation. In this study, we identify and characterize two novel type IB MSUD mutations in Israeli patients, which affect the E1beta subunit in the decarboxylase (E1) component of the branched-chain alpha-ketoacid dehydrogenase complex. The recombinant mutant E1 carrying the prevalent S289L-beta (TCG --> TTG) mutation in the Druze kindred exists as a stable inactive alphabeta heterodimer. Based on the human E1 structure, the S289L-beta mutation disrupts the interactions between Ser-289-beta and Glu-290-beta', and between Arg-309-beta and Glu-290-beta', which are essential for native alpha(2)beta(2) heterotetrameric assembly. The R133P-beta (CGG --> CCG) mutation, on the other hand, is inefficiently expressed in Escherichia coli as heterotetramers in a temperature-dependent manner. The R133P-beta mutant E1 exhibits significant residual activity but is markedly less stable than the wild-type, as measured by thermal inactivation and free energy change of denaturation. The R133P-beta substitution abrogates the coordination of Arg-133-beta to Ala-95-beta, Glu-96-beta, and Ile-97-beta, which is important for strand-strand interactions and K(+) ion binding in the beta subunit. These findings provide new insights into folding and assembly of human E1 and will facilitate DNA-based diagnosis for MSUD in the Israeli population.

Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro.

PubMed

Mescka, Caroline Paula; Wayhs, Carlos Alberto Yasin; Guerreiro, Gilian; Manfredini, Vanusa; Dutra-Filho, Carlos Severo; Vargas, Carmen Regla

2014-09-15

Maple syrup urine disease (MSUD) is an inherited aminoacidopathy caused by a deficiency in branched-chain α-keto acid dehydrogenase complex activity that leads to the accumulation of the branched-chain amino acids (BCAAs) leucine (Leu), isoleucine, and valine and their respective α-keto-acids, α-ketoisocaproic acid (KIC), α keto-β-methylvaleric acid, and α-ketoisovaleric acid. The major clinical features presented by MSUD patients include ketoacidosis, failure to thrive, poor feeding, apnea, ataxia, seizures, coma, psychomotor delay, and mental retardation; however, the pathophysiology of this disease is poorly understood. MSUD treatment consists of a low protein diet supplemented with a mixture containing micronutrients and essential amino acids but excluding BCAAs. Studies have shown that oxidative stress may be involved in the neuropathology of MSUD, with the existence of lipid and protein oxidative damage in affected patients. In recent years, studies have demonstrated the antioxidant role of L-carnitine (L-Car), which plays a central function in cellular energy metabolism and for which MSUD patients have a deficiency. In this work, we investigated the in vitro effect of Leu and KIC in the presence or absence of L-Car on DNA damage in peripheral whole blood leukocytes using the alkaline comet assay with silver staining and visual scoring. Leu and KIC resulted in a DNA damage index that was significantly higher than that of the control group, and L-Car was able to significantly prevent this damage, mainly that due to KIC. Copyright © 2014 Elsevier B.V. All rights reserved.

Analysis of gene mutations among South Indian patients with maple syrup urine disease: identification of four novel mutations.

PubMed

Narayanan, M P; Menon, Krishnakumar N; Vasudevan, D M

2013-10-01

Maple syrup urine disease (MSUD) is predominantly caused by mutations in the BCKDHA, BCKDHB and DBT genes, which encode for the E1alpha, E1beta and E2 subunits of the branched-chain alpha-keto acid dehydrogenase complex, respectively. Because disease causing mutations play a major role in the development of the disease, prenatal diagnosis at gestational level may have significance in making decisions by parents. Thus, this study was aimed to screen South Indian MSUD patients for mutations and assess the genotype-phenotype correlation. Thirteen patients diagnosed with MSUD by conventional biochemical screening such as urine analysis by DNPH test, thin layer chromatography for amino acids and blood amino acid quantification by HPLC were selected for mutation analysis. The entire coding regions of the BCKDHA, BCKDHB and DBT genes were analyzed for mutations by PCR-based direct DNA sequencing. BCKDHA and BCKDHB mutations were seen in 43% of the total ten patients, while disease-causing DBT gene mutation was observed only in 14%. Three patients displayed no mutations. Novel mutations were c.130C>T in BCKDHA gene, c. 599C>T and c.121_122delAC in BCKDHB gene and c.190G>A in DBT gene. Notably, patients harbouring these mutations were non-responsive to thiamine supplementation and other treatment regimens and might have a worse prognosis as compared to the patients not having such mutations. Thus, identification of these mutations may have a crucial role in the treatment as well as understanding the molecular mechanisms in MSUD.

Analysis of gene mutations in Chinese patients with maple syrup urine disease.

PubMed

Yang, Nan; Han, Lianshu; Gu, Xuefan; Ye, Jun; Qiu, Wenjuan; Zhang, Huiwen; Gong, Zhuwen; Zhang, Yafen

2012-08-01

Maple syrup urine disease (MSUD) is predominantly caused by mutations in the BCKDHA, BCKDHB and DBT genes, which encode for the E1α, E1β and E2 subunits of the branched-chain α-keto acid dehydrogenase complex, respectively. The aim of this study was to screen DNA samples from 16 Chinese MSUD patients and assess a potential correlation between genotype and phenotype. BCKDHA, BCKDHB and DBT genes were analyzed by polymerase chain reaction (PCR) and direct sequencing. Segments bearing novel mutations were identified by PCR-restriction fragment length polymorphism (PCR-RFLP) analysis. Within the variant alleles, 28 mutations (28/32, 87.5%), were detected in 15 patients, while one patient displayed no mutations. Mutations were comprised of 20 different: 6 BCKDHA gene mutations in 4 cases, 10 BCKDHB gene mutations in 8 cases and 4 DBT gene mutations in 3 cases. From these, 14 were novel, which included 3 mutations in the BCKDHA gene, 7 in the BCKDHB gene and 4 in the DBT gene. Only two patients with mutations in the BCKDHB and DBT genes were thiamine-responsive and presented a better clinical outcome. We identified 20 different mutations within the BCKDHA, BCKDHB and DBT genes among 16 Chinese MSUD patients, including 14 novel mutations. The majority were non-responsive to thiamine, associating with a worse clinical outcome. Our data provide the basis for further genotype-phenotype correlation studies in these patients, which will be beneficial for early diagnosis and in directing the approach to clinical intervention. Copyright © 2012 Elsevier Inc. All rights reserved.

Anti-glycation and anti-oxidative effects of a phenolic-enriched maple syrup extract and its protective effects on normal human colon cells.

PubMed

Liu, Weixi; Wei, Zhengxi; Ma, Hang; Cai, Ang; Liu, Yongqiang; Sun, Jiadong; DaSilva, Nicholas A; Johnson, Shelby L; Kirschenbaum, Louis J; Cho, Bongsup P; Dain, Joel A; Rowley, David C; Shaikh, Zahir A; Seeram, Navindra P

2017-02-22

Oxidative stress and free radical generation accelerate the formation of advanced glycation endproducts (AGEs) which are linked to several chronic diseases. Published data suggest that phenolic-rich plant foods, show promise as natural anti-AGEs agents due to their anti-oxidation capacities. A phenolic-enriched maple syrup extract (MSX) has previously been reported to show anti-inflammatory and neuroprotective effects but its anti-AGE effects remain unknown. Therefore, herein, we investigated the anti-glycation and anti-oxidation effects of MSX using biochemical and biophysical methods. MSX (500 μg mL -1 ) reduced the formation of AGEs by 40% in the bovine serum albumin (BSA)-fructose assay and by 30% in the BSA-methylglyoxal (MGO) assay. MSX also inhibited the formation of crosslinks typically seen in the late stage of glycation. Circular dichroism and differential scanning calorimeter analyses demonstrated that MSX maintained the structure of BSA during glycation. In the anti-oxidant assays, MSX (61.7 μg mL -1 ) scavenged 50% of free radicals (DPPH assay) and reduced free radical generation by 20% during the glycation process (electron paramagnetic resonance time scan). In addition, the intracellular levels of hydrogen peroxide induced reactive oxygen species were reduced by 27-58% with MSX (50-200 μg mL -1 ) in normal/non-tumorigenic human colon CCD-18Co cells. Moreover, in AGEs and MGO challenged CCD-18Co cells, higher cellular viabilities and rapid extracellular signal-regulated kinase (ERK) phosphorylation were observed in MSX treated cells, indicating its protective effects against AGEs-induced cytotoxicity. Overall, this study supports the biological effects of MSX, and warrants further investigation of its potential as a dietary agent against diseases mediated by oxidative stress and inflammation.

Plasma amino acid and urine organic acid profiles of Filipino patients with maple syrup urine disease (MSUD) and correlation with their neurologic features.

PubMed

Chiong, Mary Anne D; Tan, Marilyn A; Cordero, Cynthia P; Fodra, Esphie Grace D; Manliguis, Judy S; Lopez, Cristine P; Dalmacio, Leslie Michelle M

2016-12-01

Maple syrup urine disease (MSUD) is the most common inborn error of metabolism in the country. The cause of the neuropathology is still not well established although accumulation of branched chain amino acids (BCAA) and alteration in large neutral amino acids (LNAA) as well as energy deprivation are suggested. It is therefore the aim of this study to determine the plasma amino acid and urine organic acid profiles of patients with MSUD and correlate the findings with their neurologic features. Twenty six Filipino patients with MSUD were studied in terms of their plasma amino acid and urine organic acid profiles. Their results were compared with 26 age and sex matched controls. The neurologic features were correlated with the results of the plasma amino acids and urine organic acids. Majority of the patients with MSUD had developmental delay/intellectual disability (88%), speech delay (69%), and seizures (65%). Their amino acid profiles revealed low glutamine and alanine with high levels of leucine, isoleucine, phenylalanine, threonine and alloisoleucine compared to controls (p < 0.05). The urine organic acids showed significantly elevated excretion of the branched chain ketoacids and succinate (p < 0.05). However there were no biochemical markers that correlated significantly with the neurologic features. The findings suggest that there could still be altered LNAA metabolism among patients with MSUD when the BCAAs are elevated. Although the biochemical findings were not significantly correlated with the neurologic features, the study showed that prevention and avoidance of neurologic disturbances may still rely primarily on early diagnosis and prompt institution of treatment, along with strict compliance with the dietary regimen and maintenance of good metabolic control over time.

Functional characterization of the novel intronic nucleotide change c.288+9C>T within the BCKDHA gene: understanding a variant presentation of maple syrup urine disease.

PubMed

Fernández-Guerra, Paula; Navarrete, Rosa; Weisiger, Kara; Desviat, Lourdes R; Packman, Seymour; Ugarte, Magdalena; Rodríguez-Pombo, Pilar

2010-12-01

Mutations in any of the three different genes--BCKDHA, BCKDHB, and DBT--encoding for the E1α, E1β, and E2 catalytic components of the branched-chain α-ketoacid dehydrogenase complex can cause maple syrup urine disease (MSUD). Disease severity ranges from the classic to the mildest variant types and precise genotypes, mostly based on missense mutations, have been associated to the less severe presentations of the disease. Herein, we examine the consequences at the messenger RNA (mRNA) level of the novel intronic alteration c.288+9C>T found in heterozygous fashion in a BCKDHA variant MSUD patient who also carries the nucleotide change c.745G>A (p.Gly249Ser), previously described as a severe change. Direct analysis of the processed transcripts from the patient showed--in addition to a low but measurable level of normal mRNA product--an aberrantly spliced mRNA containing a 7-bp fragment of intron 2, which could be rescued when the patient's cells were treated with emetine. This aberrant transcript with a premature stop codon would be unstable, supporting the possible activation of nonsense-mediated mRNA decay pathway. Consistent with this finding, minigene splicing assays demonstrated that the point mutation c.288+9C>T is sufficient to create a cryptic splice site and cause the observed 7-bp insertion. Furthermore, our results strongly suggest that the c.288+9C>T allele in the patient generates both normal and aberrant transcripts that could sustain the variant presentation of the disease, highlighting the importance of correct genotyping to establish genotype-phenotype correlations and as basis for the development of therapeutic interventions.

Living related versus deceased donor liver transplantation for maple syrup urine disease.

PubMed

Feier, Flavia; Schwartz, Ida Vanessa D; Benkert, Abigail R; Seda Neto, Joao; Miura, Irene; Chapchap, Paulo; da Fonseca, Eduardo Antunes; Vieira, Sandra; Zanotelli, Maria Lúcia; Pinto e Vairo, Filippo; Camelo, Jose Simon; Margutti, Ana Vitoria Barban; Mazariegos, George V; Puffenberger, Erik G; Strauss, Kevin A

2016-03-01

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain ketoacid (BCKA) oxidation associated with episodic and chronic brain disease. Transplantation of liver from an unrelated deceased donor restores 9-13% whole-body BCKA oxidation capacity and stabilizes MSUD. Recent reports document encouraging short-term outcomes for MSUD patients who received a liver segment from mutation heterozygous living related donors (LRDT). To investigate effects of living related versus deceased unrelated grafts, we studied four Brazilian MSUD patients treated with LRDT who were followed for a mean 19 ± 12 postoperative months, and compared metabolic and clinical outcomes to 37 classical MSUD patients treated with deceased donor transplant. Patient and graft survival for LRDT were 100%. Three of 4 MSUD livers were successfully domino transplanted into non-MSUD subjects. Following LRDT, all subjects resumed a protein-unrestricted diet as mean plasma leucine decreased from 224 ± 306 μM to 143 ± 44 μM and allo-isoleucine decreased 91%. We observed no episodes of hyperleucinemia during 80 aggregate postoperative patient-months. Mean plasma leucine:isoleucine:valine concentration ratios were ~2:1:4 after deceased donor transplant compared to ~1:1:1.5 following LRDT, resulting in differences of predicted cerebral amino acid uptake. Mutant heterozygous liver segments effectively maintain steady-state BCAA and BCKA homeostasis on an unrestricted diet and during most catabolic states, but might have different metabolic effects than grafts from unrelated deceased donors. Neither living related nor deceased donor transplant affords complete protection from metabolic intoxication, but both strategies represent viable alternatives to nutritional management. Copyright © 2016 Elsevier Inc. All rights reserved.

Long-term metabolic follow-up and clinical outcome of 35 patients with maple syrup urine disease.

PubMed

Abi-Wardé, Marie-Thérèse; Roda, Célina; Arnoux, Jean-Baptiste; Servais, Aude; Habarou, Florence; Brassier, Anais; Pontoizeau, Clément; Barbier, Valérie; Bayart, Manuella; Leboeuf, Virginie; Chadefaux-Vekemans, Bernadette; Dubois, Sandrine; Assoun, Murielle; Belloche, Claire; Alili, Jean-Meidi; Husson, Marie-Caroline; Lesage, Fabrice; Dupic, Laurent; Theuil, Benoit; Ottolenghi, Chris; de Lonlay, Pascale

2017-11-01

Maple syrup urine disease (MSUD) is a rare disease that requires a protein-restricted diet for successful management. Little is known, however, about the psychosocial outcome of MSUD patients. This study investigates the relationship between metabolic and clinical parameters and psychosocial outcomes in a cohort of patients with neonatal-onset MSUD. Data on academic achievement, psychological care, family involvement, and biochemical parameters were collected from the medical records of neonatal MSUD patients treated at Necker Hospital (Paris) between 1964 and 2013. Thirty-five MSUD patients with a mean age of 16.3 (2.1-49.0) years participated. Metabolic decompensations (plasma leucine >380 μmol/L) were more frequent during the first year of life and after 15 years, mainly due to infection and dietary noncompliance, respectively. Leucine levels increased significantly in adulthood: 61.5% of adults were independent and achieved adequate social and professional integration; 56% needed occasional or sustained psychological or psychiatric care (8/19, with externalizing, mood, emotional, and anxiety disorders being the most common). Patients needing psychiatric care were significantly older [mean and standard deviation (SD) 22.6 (7.7) years] than patients needing only psychological follow-up [mean (SD) 14.3 (8.9) years]. Patients with psychological follow-up experienced the highest lifetime number of decompensations; 45% of families had difficulty coping with the chronic disease. Parental involvement was negatively associated with the number of lifetime decompensations. Adults had increased levels of plasma leucine, consistent with greater chronic toxicity. Psychological care was associated with age and number of decompensations. In addition, parental involvement appeared to be crucial in the management of MSUD patients.

Liver transplantation for classical maple syrup urine disease: long-term follow-up.

PubMed

Díaz, Victoria M; Camarena, Carmen; de la Vega, Ángela; Martínez-Pardo, Mercedes; Díaz, Carmen; López, Manuel; Hernández, Francisco; Andrés, Ane; Jara, Paloma

2014-11-01

The aim of the study was to evaluate indications, results, and clinical and neurological evolution in children who have undergone liver transplantation for classical maple syrup urine disease (MSUD). Descriptive study of liver transplantation for MSUD between 1991 and 2012. Eight patients were transplanted. Indications for transplant were poor metabolic control expressed as significant psychomotor disabilities (4 had psychomotor delays, 5 had spasticity, and 5 had epilepsy) and poor quality of life (mean number of acute metabolic decompensations and mean number of total hospitalizations before transplantation 5 and 12, respectively). Four required nasogastric tube, with a maximum 4 g/day protein-restricted diet in all of them. Seven sustained significant alterations in brain magnetic resonance imaging. Mean leucine and alloisoleucine levels were 608 (standard deviation [SD] 516) and 218 μmol/L (SD 216), respectively. All of the patients received transplants with deceased-donor livers, with ages between 1.5 and 2.5 years (mean 1.78 years). Mean posttransplantation follow-up period was 12.2 years (range 5-21 years). Final patient and graft survival was 87.5% and 75%, respectively. Following transplantation, none required hospitalization in the last 3 years nor did any have new acute metabolic decompensations following a normal diet. Five followed normal schooling, 2 had motor disabilities, and 2 had convulsive crises. Brain magnetic resonance imaging was taken in 4 patients, showing neuroimage improvement in 3 of them. Mean leucine levels were <350 μmol/L from the immediate posttransplantation period (mean 225 μmol/L, SD 78), with a maximum alloisoleucine level of 20 μmol/L. Liver transplantation is an effective treatment for classical MSUD that arrests brain damage, although it does not reverse the process.

Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role.

PubMed

Guerreiro, Gilian; Mescka, Caroline Paula; Sitta, Angela; Donida, Bruna; Marchetti, Desirèe; Hammerschmidt, Tatiane; Faverzani, Jessica; Coelho, Daniella de Moura; Wajner, Moacir; Dutra-Filho, Carlos Severo; Vargas, Carmen Regla

2015-05-01

Maple syrup urine disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity leads to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car), which has demonstrated an important role as antioxidant by reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes, have been used in the treatment of some metabolic rare disorders. This study evaluated the oxidative stress parameters, di-tyrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients under protein-restricted diet supplemented or not with L-car capsules at a dose of 50 mg kg(-1) day(-1). It was also determined urinary α-keto isocaproic acid levels as well as blood free L-car concentrations in blood. It was found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity, were observed before the treatment with L-car. The L-car supplementation induced beneficial effects on these parameters reducing the di-tyrosine and isoprostanes levels and increasing the antioxidant capacity. It was also showed a significant increase in urinary of α-ketoisocaproic acid after 2 months of L-car treatment, compared to control group. In conclusion, our results suggest that L-car may have beneficial effects in the treatment of MSUD by preventing oxidative damage to the cells and that urine can be used to monitorize oxidative damage in patients affected by this disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease.

PubMed

Vilela, Thais C; Scaini, Giselli; Furlanetto, Camila B; Pasquali, Matheus A B; Santos, João Paulo A; Gelain, Daniel P; Moreira, José Cláudio F; Schuck, Patrícia F; Ferreira, Gustavo C; Streck, Emilio L

2017-02-01

Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of the branched-chain α-keto acid dehydrogenase complex activity. This blockage leads to accumulation of the branched-chain amino acids leucine, isoleucine and valine, as well as their corresponding α-keto acids and α-hydroxy acids. The affected patients present severe neurological symptoms, such as coma and seizures, as well as edema and cerebral atrophy. Considering that the mechanisms of the neurological symptoms presented by MSUD patients are still poorly understood, in this study, protein levels of apoptotic factors are measured, such as Bcl-2, Bcl-xL, Bax, caspase-3 and -8 in hippocampus and cerebral cortex of rats submitted to acute administration of branched-chain amino acids during their development. The results in this study demonstrated that BCAA acute exposure during the early postnatal period did not significantly change Bcl-2, Bcl-xL, Bax and caspase-8 protein levels. However, the Bax/Bcl-2 ratio and procaspase-3 protein levels were decreased in hippocampus. On the other hand, acute administration of BCAA in 30-day-old rats increase in Bax/Bcl-2 ratio followed by an increased caspase-3 activity in cerebral cortex, whereas BCAA induces apoptosis in hippocampus through activation and cleavage of caspase-3 and -8 without changing the Bax/Bcl-2 ratio. In conclusion, the results suggest that apoptosis could be of pivotal importance in the developmental neurotoxic effects of BCAA. In addition, the current studies also suggest that multiple mechanisms may be involved in BCAA-induced apoptosis in the cerebral cortex and hippocampus.

[Maple syrup urine disease caused by two novel BCKDHB gene mutations in a Chinese neonate].

PubMed

Shen, Yunlin; Gong, Xiaohui; Yan, Jingbin; Qin, Li; Qiu, Gang

2015-01-01

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder that is caused by mutations in the subunits of the branched chain α-ketoacid dehydrogenase (BCKD) complex. This report presents a Han ethnic Chinese newborn infant with the severe classic form of MSUD caused by two novel missense mutations in the BCKDHB gene. The clinical and biochemical data of a Chinese neonate with classic form of MSUD were analyzed, and the DNA sequences of BCKDHA, BCKDHB, DBT and DLD genes were investigated for mutations. Then the DNA samples of the proband and the patient's parents were tested with Sanger sequencing. The manifestations of this patient were poor feeding, low reaction, and compensatory metabolic acidosis. Tandem mass spectrometry (MS/MS) showed that leucine and valine were significantly higher than normal. Urine gas chromatography-mass spectrometry (GC/MS) showed significant abnormality. Brain CT scan showed white matter changes. We identified two previously unreported mutations in the BCKDHB gene, p.Leu194Phe (c.580 C>T) and p.Ser199Arg (c.597 T>G) in exon 5. Segregation analysis showed that the novel mutation p.Ser199Arg was maternally inherited and the novel mutation p.Leu194Phe was paternally inherited. Neither mutation was found in the 186 alleles of 93 normal Han ethnic Chinese individuals. In human BCKDHB protein crystal structure, the 194th and 199th amino acids changes are likely to affect the spatial structure of the protein. The 194th and 199th amino acid of human BCKDHB protein was conserved among species. PolyPhen protein function prediction indicated that the 194th and 199th amino acid changes were likely to affect protein function. Two novel missense mutations were identified in the BCKDHB gene in the Chinese patient with MSUD.

In silico prediction of the pathogenic effect of a novel variant of BCKDHA leading to classical maple syrup urine disease identified using clinical exome sequencing.

PubMed

Fernández-Lainez, Cynthia; Aláez-Verson, Carmen; Ibarra-González, Isabel; Enríquez-Flores, Sergio; Carrillo-Sanchez, Karol; Flores-Lagunes, Leonardo; Guillén-López, Sara; Belmont-Martínez, Leticia; Vela-Amieva, Marcela

2018-04-16

Maple syrup urine disease (MSUD) is a metabolic disorder caused by mutations in three of the branched-chain α-keto acid dehydrogenase complex (BCKDC) genes. Classical MSUD symptom can be observed immediately after birth and include ketoacidosis, irritability, lethargy, and coma, which can lead to death or irreversible neurodevelopmental delay in survivors. The molecular diagnosis of MSUD can be time-consuming and difficult to establish using conventional Sanger sequencing because it could be due to pathogenic variants of any of the BCKDC genes. Next-generation sequencing-based methodologies have revolutionized the molecular diagnosis of inborn errors in metabolism and offer a superior approach for genotyping these patients. Here, we report an MSUD case whose molecular diagnosis was performed by clinical exome sequencing (CES), and the possible structural pathogenic effect of a novel E1α subunit pathogenic variant was analyzed using in silico analysis of α and β subunit crystallographic structure. Molecular analysis revealed a new homozygous non-sense c.1267C>T or p.Gln423Ter variant of BCKDHA. The novel BCKDHA variant is considered pathogenic because it caused a premature stop codon that probably led to the loss of the last 22 amino acid residues of the E1α subunit C-terminal end. In silico analysis of this region showed that it is in contact with several residues of the E1β subunit mainly through polar contacts, hydrogen bonds, and hydrophobic interactions. CES strategy could benefit the patients and families by offering precise and prompt diagnosis and better genetic counseling. Copyright © 2018 Elsevier B.V. All rights reserved.

Identification of mutations, genotype-phenotype correlation and prenatal diagnosis of maple syrup urine disease in Indian patients.

PubMed

Gupta, Deepti; Bijarnia-Mahay, Sunita; Saxena, Renu; Kohli, Sudha; Dua-Puri, Ratna; Verma, Jyotsna; Thomas, E; Shigematsu, Yosuke; Yamaguchi, Seiji; Deb, Roumi; Verma, Ishwar Chander

2015-09-01

Maple syrup urine disease (MSUD) is caused by mutations in genes BCKDHA, BCKDHB, DBT encoding E1α, E1β, and E2 subunits of enzyme complex, branched-chain alpha-ketoacid dehydrogenase (BCKDH). BCKDH participates in catabolism of branched-chain amino acids (BCAAs) - leucine, isoleucine and valine in the energy production pathway. Deficiency or defect in the enzyme complex causes accumulation of BCAAs and keto-acids leading to toxicity. Twenty-four patients with MSUD were enrolled in the study for molecular characterization and genotype-phenotype correlation. Molecular studies were carried out by sequencing of the 3 genes by Sanger method. Bioinformatics tools were employed to classify novel variations into pathogenic or benign. The predicted effects of novel changes on protein structure were elucidated by 3D modeling. Mutations were detected in 22 of 24 patients (11, 7 and 4 in BCKDHB, BCKDHA and DBT genes, respectively). Twenty mutations including 11 novel mutations were identified. Protein modeling in novel mutations showed alteration of structure and function of these subunits. Mutations, c.1065 delT (BCKDHB gene) and c.939G > C (DBT gene) were noted to be recurrent, identified in 6 of 22 alleles and 5 of 8 alleles, respectively. Two-third patients were of neonatal classical phenotype (16 of 24). BCKDHB gene mutations were present in 10 of these 16 patients. Prenatal diagnoses were performed in 4 families. Consanguinity was noted in 37.5% families. Although no obvious genotype-phenotype correlation could be found in our study, most cases with mutation in BCKDHB gene presented in neonatal period. Large number of novel mutations underlines the heterogeneity and distinctness of gene pool from India. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Evaluation of dynamic thiol/disulphide homeostasis as a novel indicator of oxidative stress in maple syrup urine disease patients under treatment.

PubMed

Zubarioglu, Tanyel; Kiykim, Ertugrul; Cansever, Mehmet Serif; Neselioglu, Salim; Aktuglu-Zeybek, Cigdem; Erel, Ozcan

2017-02-01

Maple syrup urine disease (MSUD) is a metabolic disorder that is caused by deficiency of branched-chain α-keto acid dehydrogenase complex. Although accumulation of toxic metabolites is associated with neurotoxicity, mechanisms underlying brain damage remain unclear. Aim of this study is to evaluate thiol/disulphide homeostasis as a novel indicator of oxidative stress in MSUD patients under treatment. Twenty patients with MSUD and 20 healthy individuals were included in study. All patients were under regular follow-up and had a good metabolic control. Serum native thiol (-SH), total thiol (-SH + -S-S-), disulphide (-S-S) levels were measured in all subjects. Disulphide/native thiol, disulphide/total thiol and native thiol/total thiol ratios were calculated from these values. Simultaneous blood sampling for plasma quantitative amino acid analysis was performed in both groups. Any significant difference was not observed in -SH, -SH + -S-S-, -S-S levels between two groups. In addition no increase of disulphide/native thiol and disulphide/total thiol ratios was detected in patient group. This study is the first study that evaluates dynamic thiol/disulphide homeostasis as an indicator of oxidative stress in MSUD patients. Among previous studies that were made to determine oxidative stress in treated MSUD patients, this study had the largest sample size also. In recent studies, it was claimed that oxidative stress could be responsible from neurotoxicity even in treated patients. Here, dynamic thiol/disulfide homeostasis status showed that providing good metabolic control in MSUD patients prevent oxidative stress. Under regular follow-up and good compliance with diet, additional antioxidant therapies would possibly not be necessary.

Dihydroconiferyl alcohol - A cell division factor from Acer species.

PubMed

Lee, T S; Purse, J G; Pryce, R J; Horgan, R; Wareing, P F

1981-10-01

A compound that stimulated growth of soybean callus was isolated from spring sap of sycamore (Acer pseudoplatanus L.). Insufficient compound was isolated to permit it to be characterised. A compound with identical properties was isolated from commercial maple syrup, the concentrated spring sap of Acer saccharum L. The compound was identified as 3-(3-methoxy-4-hydroxyphenyl)-propan-1-ol (dihydroconiferyl alcohol, DCA). DCA was also active in the tobacco callus and radish leaf senescence assays, but was inactive in four other tests for cytokinin activity. DCA acted synergistically with kinetin to promote soybean callus growth. It is concluded that DCA has properties distinct from those of purine cytokinins.

The influence of soil-site factors on sugar maple (Acer saccharum Marsh.) growth response to climatic change in central Ontario

NASA Astrophysics Data System (ADS)

Schutten, K.; Gedalof, Z.

2010-12-01

Over the past several decades, concerns about climatic change and its potential impacts on Canada’s various geographical regions and associated ecological processes have grown steadily, especially among land and resource managers. As these risks transition into tangible outcomes in the field, it will be important for resource managers to understand historical climatic variability and natural ecological trends in order to effectively respond to a changing climate. Sugar maple (Acer saccharum Marsh.) is considered a stable endpoint for mature forests in the northern hardwood community of central Ontario, and it tends to be the dominant species, in a beech-ironwood-yellow birch matrix. In North America, this species is used for both hardwood lumber and for maple sugar (syrup) products; where it dominates, large recreational opportunities also exist. There are many biotic and abiotic factors that play a large role in the growth and productivity of sugar maple stands, such as soil pH, moisture regime, and site slope and aspect. This research undertaking aims to add to the body of literature addressing the following question: how do site factors influence the sensitivity of sugar maple growth to climatic change? The overall objective of the research is to evaluate how biotic and abiotic factors influence the sensitivity of sugar maple annual radial growth to climatic variability. This research will focus on sugar maple growth and productivity in Algonquin Provincial Park, and the impact that climatic variability has had in the past on these stands based on site-specific characteristics. In order to complete this goal, 20 sites were identified in Algonquin Provincial Park based on variability of known soil and site properties. These sites were visited in order to collect biotic and abiotic site data, and to measure annual radial growth increment of trees. Using regional climate records and standard dendrochronological methods, the collected increment growth data will be used to build site-specific chronologies in order to determine the differences in tree growth response to climatic variability due to differences in soil and site quality. Preliminary results suggest that variability in site-specific abiotic and biotic conditions may strongly influence individual stand growth responses to climatic variability.

Volatiles from a rare Acer spp. honey sample from Croatia.

PubMed

Jerković, Igor; Marijanović, Zvonimir; Malenica-Staver, Mladenka; Lusić, Drazen

2010-06-24

A rare sample of maple (Acer spp.) honey from Croatia was analysed. Ultrasonic solvent extraction (USE) using: 1) pentane, 2) diethyl ether, 3) a mixture of pentane and diethyl ether (1:2 v/v) and 4) dichloromethane as solvents was applied. All the extracts were analysed by GC and GC/MS. The most representative extracts were 3) and 4). Syringaldehyde was the most striking compound, being dominant in the extracts 2), 3) and 4) with percentages 34.5%, 33.1% and 35.9%, respectively. In comparison to USE results of other single Croatian tree honey samples (Robinia pseudoacacia L. nectar honey, Salix spp. nectar and honeydew honeys, Quercus frainetto Ten. honeydew as well as Abies alba Mill. and Picea abies L. honeydew) and literature data the presence of syringaldehyde, previously identified in maple sap and syrup, can be pointed out as a distinct characteristic of the Acer spp. honey sample. Headspace solid-phase microextraction (HS-SPME) combined with GC and GC/MS identified benzaldehyde (16.5%), trans-linalool oxide (20.5%) and 2-phenylethanol (14.9%) as the major compounds that are common in different honey headspace compositions.

Effects of crown release on growth and quality of even-aged red maple stands

Treesearch

Terry F. Strong; Audra E. Hubbell; Adam H. Weise; Gayne G. Erdmann

2006-01-01

The effects of six crown-release treatments on growth and bole quality of 54 dominant, codominant, and intermediate red maples (Acer rubrum L.) were examined in an even-aged stand in upper Michigan. Treatments included an unreleased control, a single-tree and a two-tree crown release, and a full crown-to-crown release of 5, 10, and 15ft. Twenty-two...

Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells.

PubMed

Lu, Gang; Sun, Haipeng; She, Pengxiang; Youn, Ji-Youn; Warburton, Sarah; Ping, Peipei; Vondriska, Thomas M; Cai, Hua; Lynch, Christopher J; Wang, Yibin

2009-06-01

The branched-chain amino acids (BCAA) are essential amino acids required for protein homeostasis, energy balance, and nutrient signaling. In individuals with deficiencies in BCAA, these amino acids can be preserved through inhibition of the branched-chain-alpha-ketoacid dehydrogenase (BCKD) complex, the rate-limiting step in their metabolism. BCKD is inhibited by phosphorylation of its E1alpha subunit at Ser293, which is catalyzed by BCKD kinase. During BCAA excess, phosphorylated Ser293 (pSer293) becomes dephosphorylated through the concerted inhibition of BCKD kinase and the activity of an unknown intramitochondrial phosphatase. Using unbiased, proteomic approaches, we have found that a mitochondrial-targeted phosphatase, PP2Cm, specifically binds the BCKD complex and induces dephosphorylation of Ser293 in the presence of BCKD substrates. Loss of PP2Cm completely abolished substrate-induced E1alpha dephosphorylation both in vitro and in vivo. PP2Cm-deficient mice exhibited BCAA catabolic defects and a metabolic phenotype similar to the intermittent or intermediate types of human maple syrup urine disease (MSUD), a hereditary disorder caused by defects in BCKD activity. These results indicate that PP2Cm is the endogenous BCKD phosphatase required for nutrient-mediated regulation of BCKD activity and suggest that defects in PP2Cm may be responsible for a subset of human MSUD.

Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD.

PubMed

S Sonnet, Davis; N O'Leary, Monique; A Gutierrez, Mark; M Nguyen, Steven; Mateen, Samiha; Hsu, Yuehmei; P Mitchell, Kylie; J Lopez, Antonio; Vockley, Jerry; K Kennedy, Brian; Ramanathan, Arvind

2016-07-04

Maple Syrup Urine Disease (MSUD) is an inherited disorder caused by the dysfunction in the branched chain keto-acid dehydrogenase (BCKDH) enzyme. This leads to buildup of branched-chain keto-acids (BCKA) and branched-chain amino acids (BCAA) in body fluids (e.g. keto-isocaproic acid from the BCAA leucine), leading to numerous clinical features including a less understood skeletal muscle dysfunction in patients. KIC is an inhibitor of mitochondrial function at disease relevant concentrations. A murine model of intermediate MSUD (iMSUD) shows significant skeletal muscle dysfunction as by judged decreased muscle fiber diameter. MSUD is an orphan disease with a need for novel drug interventions. Here using a 96-well plate (liquid chromatography- mass spectrometry (LC-MS) based drug-screening platform we show that Metformin, a widely used anti-diabetic drug, reduces levels of KIC in patient-derived fibroblasts by 20-50%. This Metformin-mediated effect was conserved in vivo; Metformin-treatment significantly reduced levels of KIC in the muscle (by 69%) and serum (by 56%) isolated from iMSUD mice, and restored levels of mitochondrial metabolites (e.g. AMP and other TCA). The drug also decreased the expression of mitochondrial branched chain amino transferase (BCAT) which produces KIC in skeletal muscle. This suggests that Metformin can restore skeletal muscle homeostasis in MSUD by decreasing mitochondrial KIC production.

Cerebral Oedema, Blood-Brain Barrier Breakdown and the Decrease in Na(+),K(+)-ATPase Activity in the Cerebral Cortex and Hippocampus are Prevented by Dexamethasone in an Animal Model of Maple Syrup Urine Disease.

PubMed

Rosa, Luciana; Galant, Leticia S; Dall'Igna, Dhébora M; Kolling, Janaina; Siebert, Cassiana; Schuck, Patrícia F; Ferreira, Gustavo C; Wyse, Angela T S; Dal-Pizzol, Felipe; Scaini, Giselli; Streck, Emilio L

2016-08-01

Maple syrup urine disease (MSUD) is a rare metabolic disorder associated with acute and chronic brain dysfunction. This condition has been shown to lead to macroscopic cerebral alterations that are visible on imaging studies. Cerebral oedema is widely considered to be detrimental for MSUD patients; however, the mechanisms involved are still poorly understood. Therefore, we investigated whether acute administration of branched-chain amino acids (BCAA) causes cerebral oedema, modifies the Na(+),K(+)-ATPase activity, affects the permeability of the blood-brain barrier (BBB) and alters the levels of cytokines in the hippocampus and cerebral cortex of 10-day-old rats. Additionally, we investigated the influence of concomitant administration of dexamethasone on the alterations caused by BCAA. Our results showed that the animals submitted to the model of MSUD exhibited an increase in the brain water content, both in the cerebral cortex and in the hippocampus. By investigating the mechanism of cerebral oedema, we discovered an association between H-BCAA and the Na(+),K(+)-ATPase activity and the permeability of the BBB to small molecules. Moreover, the H-BCAA administration increases Il-1β, IL-6 and TNF-α levels in the hippocampus and cerebral cortex, whereas IL-10 levels were decreased in the hippocampus. Interestingly, we showed that the administration of dexamethasone successfully reduced cerebral oedema, preventing the inhibition of Na(+),K(+)-ATPase activity, BBB breakdown and the increase in the cytokines levels. In conclusion, these findings suggest that dexamethasone can improve the acute cerebral oedema and brain injury associated with high levels of BCAA, either through a direct effect on brain capillary Na(+),K(+)-ATPase or through a generalized effect on the permeability of the BBB to all compounds.

Evolution of maple syrup urine disease in patients diagnosed by newborn screening versus late diagnosis.

PubMed

Couce, M L; Ramos, F; Bueno, M A; Díaz, J; Meavilla, S; Bóveda, M D; Fernández-Marmiesse, A; García-Cazorla, A

2015-11-01

Maple syrup urine disease (MSUD) is a rare metabolic disorder for which the newborn screening (NBS) is possible but it has not been yet implemented for most Spanish regions. In the present study, we assess the clinical features and outcome of 14 MSUD Spanish patients with similar treatment protocol diagnosed either by NBS or by clinical symptoms. Eight patients were detected by NBS, four classic and four moderate MSUD. The average age at detection was 4.6 days, the mean plasmatic concentration of leucine at diagnosis was 1807 μM; the average number of days with leucine >1000 μM was 0.7 (0-4) and the mean number of total hospitalizations was 1.6 (0-5). Mean follow-up time was 70 months. They had good evolution: all remain asymptomatic, but 2 patients have attention deficit and hyperactivity disorder. Six patients with late diagnosis of classic MSUD were followed during 41 months. All presented with acute encephalopathy during the first month of life, mean leucine levels of 2355 μM, mean number of days with leucine >1000 μM of 6.6 (1-13) and mean number of total hospitalizations of 5.3 (4-7). Only two patients have a psychomotor development index in the lower limit (80 and 83). For all patients a good genotype-phenotype correlation was found and four novel mutations were identified: p.A311H, p.T84S, p.T397L, pL398P. Our study support that NBS improves prognosis of MSUD patients. But early diagnosis and an aggressive treatment together with a close monitoring of leucine levels improve neurological evolution in MSUD patients, even for those not detected by NBS. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Different gene preferences of maple syrup urine disease in the aboriginal tribes of Taiwan.

PubMed

Hou, Jia-Woei; Hwang, Tsann-Long

2014-06-01

Maple syrup urine disease (MSUD) is a rare inborn error of metabolism caused by a deficiency of the branched-chain α-ketoacid dehydrogenase (BCKD) complex. Mutations in any one of the three different genes encoding for the BCKD components, namely, BCKDHA, BCKDHB, and DBT, may be responsible for this disease. In Taiwan, few MSUD cases were diagnosed clinically, and most of these patients are from Aboriginal tribes. To identify and detect the carrier frequency of MSUD in Taiwanese Aboriginal tribes, we performed biochemical and molecular studies from peripheral blood in MSUD patients and dried blood on filter paper in the enrolled screened populations. Homozygous A208T and I281T of BCKDHA were found in two patients from Hans (non-Aboriginal Taiwanese), respectively; compound heterozygous mutations of the DBT gene [4.7 kb deletion/c.650-651insT (L217F or L217fsX223) and c.650-651insT/c.88-89delAT] were found in two patients from Amis, respectively, after direct DNA sequencing and polymerase chain reaction-restriction fragment length polymorphism studies. There were no cases of deleted 4.7-kb heterozygote out of 302 normal people (Hans, n = 125; Atayal, n = 156; and Saisiyat, n = 21); by contrast, the DBT mutations c.650-651insT and deleted 4.7-kb heterozygote were noted in 2/121 and 1/121, respectively, from the general population of the Amis, a southeastern Taiwanese tribe. Although the Taiwanese Austronesian Aboriginal tribes are considered to share a common origin, different gene preferences of MSUD were noted. The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved in the southern and eastern, but not in northern Aboriginal tribes of Taiwan. Copyright © 2013. Published by Elsevier B.V.

Case report: Aqueous and Vitreous amino-acid concentrations in a patient with maple syrup urine disease operated on rhegmatogenous retinal detachment.

PubMed

Kanakis, Menelaos G; Michelakakis, Helen; Petrou, Petros; Koutsandrea, Chrysanthi; Georgalas, Ilias

2016-10-03

Maple syrup urine disease (MSUD) is a rare metabolic disorder, affecting the metabolism of branched chain amino-acids (Valine, Leukine, Isoleukine). We present a rare case of rhegmatogenous retinal detachment (RRD) in a MSUD patient. We performed amino acid analysis of aqueous humour, vitreous and serum samples obtained during surgery from a 24 year old female MSUD patient successfully operated on RRD. Serum values for a-amino-butyric acid, valine, isoleucine, leucine, tyrosine, phenylalanine, ornithine and histidine were low, while values for citrulline, methionine and lysine were borderline low, all attributed to the patient's special diet. Serum glutamate was above normal, probably due to the breakdown of glutamine to glutamate. In the aqueous and vitreous the amino acids implicated in MSUD (Valine, Leukine Isoleukine), were within normal range. Glutamate was absent in the vitreous and presented low levels in the aqueous. Glutamate has been reported to play an important role in retinal damage. Elevated glutamate levels have been reported in vitreous specimens from patients subjected to vitrectomy or buckling surgery for RRD. In MSUD, glutamate has been implicated in the pathogenesis of brain damage. Low levels of glutamate have been observed in the cerebellum of experimental MSUD animals, as well as postmortem brain tissue from a child that died of leucine intoxication. The reduction was attributed to the elevation of a-ketoisocaproic which reverses the net direction of nitrogen flow. It could be argued that this could impact on amino acid concentration in aqueous and vitreous fluids. Although no definite conclusions can be drawn by this extremely rare case, the low vitreous and aqueous levels of Glutamate is an interesting finding. Further studies are needed to provide a better insight in the role of amino acids as neurotransmitters in the human eye in health and disease.

In silico analysis of novel mutations in maple syrup urine disease patients from Iran.

PubMed

Abiri, Maryam; Karamzadeh, Razieh; Mojbafan, Marziyeh; Alaei, Mohammad Reza; Jodaki, Atefeh; Safi, Masomeh; Kianfar, Soodeh; Bandehi Sarhaddi, Ameneh; Noori-Daloii, Mohammad Reza; Karimipoor, Morteza; Zeinali, Sirous

2017-02-01

Maple Syrup Urine Disease (MSUD) is a rare autosomal recessive disorder of branched-chain amino acid (BCAA) metabolism. The disease is mainly caused by mutations either in the BCKDHA, BCKDHB, DBT or DLD genes encoding components of the E1α, E1β, E2 and E3 subunits of branched-chain α-keto acid dehydrogenase complex (BCKDC), respectively. BCKDC is a mitochondrial enzyme which is responsible for the normal breakdown of BCAA. The rate of consanguineous marriage in Iran is 38.6 %, so the prevalence of autosomal recessive disorders is higher in comparison to other countries. Consanguinity increases the chance of the presence of pathogenic mutations in a homoallelic state. This phenomenon has made homozygosity mapping a powerful tool for finding the probable causative gene in heterogeneous disorders like IEM (Inborn Errors of Metabolism). In this study, two sets of multiplex polymorphic STR (Short Tandem Repeat) markers linked to the above-mentioned genes were selected to identify the probable pathogenic gene in the studied families. The families who showed a homozygous haplotype for the STR markers of the BCKDHB gene were subsequently sequenced. Four novel mutations including c.633 + 1G > A, c.988G > A, c.833_834insCAC, and a homozygous deletion of whole exon 3 c. (274 + 1_275-1) _(343 + 1_344-1), as well as one recently reported (c. 508G > T) mutation have been identified. Interestingly, three families shared a common haplotype structure along with the c. 508G > T mutation. Also, four other families revealed another similar haplotype with c.988G > A mutation. Founder effect can be a suggestive mechanism for the disease. Additionally, structural models of MSUD mutations have been performed to predict the pathogenesis of the newly identified variants.

Methylglyoxal is associated with bacteriostatic activity of high fructose agave syrups.

PubMed

Corrales Escobosa, Alma Rosa; Gomez Ojeda, Armando; Wrobel, Kazimierz; Magana, Armando Alcazar; Wrobel, Katarzyna

2014-12-15

Three α-ketoaldehydes, potentially present in high fructose agave syrups (HFASs) as intermediates of the Maillard reaction, were determined. A previously reported HPLC-FLD procedure based on pre-column derivatisation with 4-methoxy-o-phenylenediamine was adopted, yielding the method quantification limits 0.11 mg/kg, 0.10mg/kg, 0.09 mg/kg for glyoxal, methylglyoxal (MGo) and diacetyl, respectively. The obtained results revealed high concentrations of methylglyoxal in HFASs (average 102 ± 91 mg/kg, range 15.6-315 mg/kg) as compared to commercial Mexican bee honeys or corn syrups. Hydrogen peroxide was generated in all HFASs upon dilution, yet to less extent than in bee honeys. HFASs presented bacteriostatic activity against Bacillus subtilis and Escherichia coli; catalase addition had minimum effect on the assay results in syrups with elevated MGo. Principal component analysis revealed direct association between growth inhibition and MGo. It is concluded that elevated concentration of MGo in HFASs is at least in part responsible for their non-peroxide bacteriostatic activity. Copyright © 2014 Elsevier Ltd. All rights reserved.

The major types of added sugars and non-nutritive sweeteners in a sample of Australian packaged foods.

PubMed

Probst, Yasmine C; Dengate, Alexis; Jacobs, Jenny; Louie, Jimmy Cy; Dunford, Elizabeth K

2017-12-01

Limiting the intake of added sugars in the diet remains a key focus of global dietary recommendations. To date there has been no systematic monitoring of the major types of added sugars used in the Australian food supply. The present study aimed to identify the most common added sugars and non-nutritive sweeteners in the Australian packaged food supply. Secondary analysis of data from the Australian FoodSwitch database was undertaken. Forty-six added sugars and eight non-nutritive sweetener types were extracted from the ingredient lists of 5744 foods across seventeen food categories. Australia. Not applicable. Added sugar ingredients were found in 61 % of the sample of foods examined and non-nutritive sweetener ingredients were found in 69 %. Only 31 % of foods contained no added sugar or non-nutritive sweetener. Sugar (as an ingredient), glucose syrup, maple syrup, maltodextrin and glucose/dextrose were the most common sugar ingredient types identified. Most Australian packaged food products had at least one added sugar ingredient, the most common being 'sugar'. The study provides insight into the most common types of added sugars and non-nutritive sweeteners used in the Australian food supply and is a useful baseline to monitor changes in how added sugars are used in Australian packaged foods over time.

[Clinical characteristics and analysis of mass spectrometric data in 33 patients with maple syrup urine disease].

PubMed

Yang, Nan; Han, Lian-shu; Ye, Jun; Qiu, Wen-juan; Zhang, Hui-wen; Gao, Xiao-lan; Wang, Yu; Li, Xiao-yan; Xu, Hao; Gu, Xue-fan

2012-10-30

To explore the clinical characteristics and the diagnostic method of maple syrup urine disease (MSUD). From January 2003 to December 2011, a total of 14 000 patients with suspected inherited metabolism diseases were tested. The blood levels of leucine and valine of these patients were detected by tandem mass spectrometry. The urinary level of branched-chain α-ketoacids was tested by gas chromatography-mass spectrometry. And the diagnosis was based on the elevated levels of leucine and valine in blood and branched-chain α-ketoacids in urine. Thirty-three MSUD patients were confirmed. Their median age of initial visit was 0.17 years old (range: 7 days to 30 years old). The peak onset age of them was 2-30 days old, including 28 cases of neonatal onset (84.8%). The presenting symptoms of 28 cases were feeding difficulties (n=14), poor response, lethargy and seizures. Their median blood levels of leucine and valine (1901 (458-5804) and 600 (315-1617) µmol/L) were significantly higher than their normal levels ((50-300) and (60-250) µmol/L, both P<0.01). Their urinary levels of 2-OH-isovaleric acid, 2-keto-isovaleric acid, 2-keto-3-methylvaleric acid, 2-keto-isocaproic and acetylglycine (262.5 (5.4-624.3), 35.8 (1.9-156.0), 133.8 (7.4-611.5), 518.7 (17.2-2121.2) and 280.5 (11.0-1087.9) respectively) significantly higher than their normal levels (0, <0.1, 0, 0, <0.1 respectively, all P<0.01). In 5 intermittent MSUD patients, their blood levels of leucine and valine (402 (348-958) and 556 (322-808) µmol/L) were significantly higher than their normal levels (both P<0.01). The urinary level of 2-OH-isovaleric acid was significantly higher than its normal levels (P<0.01) while the urinary levels of other α-ketoacids were normal. The confirmation of MSUD remains difficult because of a lack of specific clinical features. The detections of tandem mass spectrometry and gas chromatography-mass spectrometry may aid its early diagnosis.

Eleven novel mutations of the BCKDHA, BCKDHB and DBT genes associated with maple syrup urine disease in the Chinese population: Report on eight cases.

PubMed

Li, Xiyuan; Ding, Yuan; Liu, Yupeng; Ma, Yanyan; Song, Jinqing; Wang, Qiao; Li, Mengqiu; Qin, Yaping; Yang, Yanling

2015-11-01

Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder that affects the degradation of branched chain amino acids (BCAAs). Only a few cases of MSUD have been documented in Mainland China, and prenatal diagnosis has not been performed so far. In this report, 8 patients (4 girls and 4 boys) with MSUD from 8 unrelated Chinese families were diagnosed at the age of 9 days to 1 year and 8 months. The diagnosis was confirmed by serum BCAAs and genetic analyses. Among the 8 patients, only one was detected by newborn screening. The remaining 7 patients were admitted because of neurological disorders and underwent selective screening. Significantly elevated BCAAs were observed in 7 patients. One patient was diagnosed by post-mortem study. 12 mutations were found in the BCKDHA, BCKDHB and DBT genes. 11 of these mutations were novel: c.178G > T, c.491T > C, c.740A > G, c.1214_1219dupCCAACC and IVS6+1delG in BCKDHA; c.482T > G, c.508C > T, c.767A > G, c.768C > G and IVS4,-2A > C in BCKDHB; and c.1A > G in DBT. Only one mutation, c.659C > T in the BCKDHA gene, had been previously reported. 7 patients were treated by dietary intervention and symptomatic therapy. 6 of them showed clinical improvement. The mother of one patient who died from MSUD underwent amniocentesis during her second pregnancy. The BCAAs level in her amniotic fluid was normal. Only one heterozygous mutation, IVS4,-2A > C in the BCKDHB gene, was detected in the cultured amniocytes. The results revealed that the fetus was not affected by MSUD. Normal development and the blood BCAAs profile confirmed the prenatal diagnosis after birth. Thus, we identified eleven novel mutations associated with MSUD in the Chinese population. Prenatal diagnosis of MSUD was successfully performed on one fetus by genetic analysis of the cultured amniocytes. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Maple syrup urine disease (MSUD)--clinical profile of 47 Filipino patients.

PubMed

Lee, J Y; Chiong, M A; Estrada, S C; Cutiongco-De la Paz, E M; Silao, C L T; Padilla, C D

2008-12-01

Maple syrup urine disease (MSUD) is a very rare disorder of branched-chain amino acid metabolism. However, it is the most common inborn error of metabolism in the Philippines. We present a retrospective review of 21 patients diagnosed with MSUD between 1999 and 2004. The patients presented clinically between 2 and 14 days of life (mean 5 days) and the diagnosis of MSUD was established between 6 days and 11 months of age (mean 39 days). The classical burnt sugar odour was noted in the majority of patients (81%). The diagnosis of MSUD was initially based on clinical suspicion and confirmed biochemically by measurement of leucine/isoleucine levels by thin-layer chromatography. The acute management included removal of accumulated branched-chain amino acids by peritoneal dialysis in 62% of the patients. Mortality rate of this group of patients was 24% and follow-up rate was 87%. We compared this series with a previously reported series of 26 patients to determine whether diagnosis and the management of MSUD improved over the two periods. Four cases have been diagnosed early since 1992, the majority of whom had the classic form of MSUD with the onset of symptoms in the first two weeks of life. A small subset of patients with early nonspecific symptoms was diagnosed much later owing to a low-level clinical suspicion among clinicians. Overall, however, there appears to be a small but general trend towards earlier diagnosis, reduced mortality and long-term follow up in the later series. Although we are able to diagnose and manage MSUD in the Philippines, we recognize that the clinical outcome remains poor and is due mainly to late referral of cases and inadequate long-term management. In the Philippines, we recommend that all newborns who are considered to be septic, have feeding difficulties, fail to regain their birth weight or present with any other symptoms suggestive of MSUD be evaluated in the first instance by analysis of urine for ketones and if they are positive have blood collected and sent to our laboratory for leucine/isoleucine measurement.

Cognitive and Adaptive Functioning after Liver Transplantation for Maple Syrup Urine Disease: A Case Series

PubMed Central

Shellmer, D. A.; Dabbs, A. DeVito; Dew, M. A.; Noll, R. B.; Feldman, H.; Strauss, K.; Morton, D. H.; Vockley, G.; Mazariegos, G. V.

2011-01-01

MSUD is a complex metabolic disorder that has been associated with central nervous system damage, developmental delays, and neurocognitive deficits. Although liver transplantation provides a metabolic cure for MSUD, changes in cognitive and adaptive functioning following transplantation have not been investigated. In this report we present data from 14 patients who completed cognitive and adaptive functioning testing pre- and one year and/or three years post-liver transplantation. Findings show either no significant change or improvement in IQ scores pre- to post-liver transplantation. Greater variability was observed in adaptive functioning scores, but the majority of patients evidenced either no significant change or improvement in adaptive scores. In general, findings may indicate that liver transplantation curtails additional central nervous system damage and neurocognitive decline providing an opportunity for stabilization or improvement in functioning. PMID:20946191

A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate.

PubMed

Köse, Melis; Canda, Ebru; Kagnici, Mehtap; Uçar, Sema Kalkan; Çoker, Mahmut

2017-01-01

In treatment of metabolic imbalances caused by maple syrup urine disease (MSUD), peritoneal dialysis, and hemofiltration, pharmacological treatments for elimination of toxic metabolites can be used in addition to basic dietary modifications. Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate (NaPB) in urea-cycle disorder cases has been associated with a reduction in branched-chain amino acid (BCAA) concentrations when the patients are on adequate dietary protein intake. Moreover, NaPB in treatment of MSUD patients is also associated with reduction of BCAA levels in a limited number of cases. However, there are not enough studies in the literature about application and efficacy of this treatment. Our case report sets an example of an alternative treatment's efficacy when extracorporeal procedures are not available due to technical difficulties during attack period of the disease.

A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate

PubMed Central

Canda, Ebru; Kagnici, Mehtap; Uçar, Sema Kalkan; Çoker, Mahmut

2017-01-01

In treatment of metabolic imbalances caused by maple syrup urine disease (MSUD), peritoneal dialysis, and hemofiltration, pharmacological treatments for elimination of toxic metabolites can be used in addition to basic dietary modifications. Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate (NaPB) in urea-cycle disorder cases has been associated with a reduction in branched-chain amino acid (BCAA) concentrations when the patients are on adequate dietary protein intake. Moreover, NaPB in treatment of MSUD patients is also associated with reduction of BCAA levels in a limited number of cases. However, there are not enough studies in the literature about application and efficacy of this treatment. Our case report sets an example of an alternative treatment's efficacy when extracorporeal procedures are not available due to technical difficulties during attack period of the disease. PMID:28589054

A case of classical maple syrup urine disease that was successfully managed by living donor liver transplantation.

PubMed

Takano, Chika; Ishige, Mika; Ogawa, Erika; Usui, Hiromi; Kagawa, Reiko; Tajima, Go; Fujiki, Ryoji; Fukao, Toshiyuki; Mizuta, Koichi; Fuchigami, Tatsuo; Takahashi, Shori

2017-08-01

Classical MSUD is often fatal without appropriate medical interventions because of metabolic crisis. There are numerous reports suggesting the therapeutic potential of deceased donor liver transplantation for MSUD. However, the usefulness of LDLT for MSUD is unknown. We report a case of classical MSUD, which was successfully managed by LDLT from the patient's father at 1 year of age. Abnormal brain findings, which were cured with effective treatment, gradually disappeared after LDLT. The patient then developed normally. Findings from this case suggest the importance of LDLT for maintaining low leucine levels and subsequent normal neurological development. Although LDLT involves a modest surgical insult, LDLT with a related donor achieves acceptable leucine levels for life. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment.

PubMed

Bravo-Alonso, I; Oyarzabal, A; Sánchez-Aragó, M; Rejas, M T; Merinero, B; García-Cazorla, A; Artuch, R; Ugarte, M; Rodríguez-Pombo, P

2016-06-01

This data article contains complementary figures to the research article "Mitochondrial response to the BCKDK-deficiency: some clues to understand the positive dietary response in this form of autism" [1]. Herein we present data relative to the effect of knocking down BCKDK gene on the real time oxygen consumption rate of fibroblasts obtained from a Maple Syrup Urine Disease (MSUD) patient. Interference of BCKDK expression on such cells showing a reduced branched-chain α-ketoacid dehydrogenase (BCKDHc) activity; let us generate a scenario to study the direct effect of BCKDK absence in an environment of high branched-chain amino acids (BCAAs) concentrations. Data relative to the effectiveness of the knockdown together with the potentiality of the BCKDK-knockdown to increase the deficient branched-chain α-ketoacid dehydrogenase activity detected in MSUD patients are also shown.

Successful living donor liver transplantation for classical maple syrup urine disease.

PubMed

Yasui, Toshihiro; Suzuki, Tatsuya; Hara, Fujio; Watanabe, Shunsuke; Uga, Naoko; Naoe, Atsuki; Yoshikawa, Tetsushi; Ito, Tetsuya; Nakajima, Yoko; Miura, Hiroki; Sugioka, Atsushi; Kato, Yutaro; Tokoro, Takamasa; Tanahashi, Yoshinao; Kasahara, Mureo; Fukuda, Akinari; Kurahashi, Hiroki

2016-08-01

MSUD is an autosomal recessive condition characterized by a deficiency in the enzyme, BCKDH, which catalyzes the breakdown of BCAAs. If left untreated, MSUD can result in mental retardation, central nervous system disorders, and even death. Most patients with MSUD are treated with a restricted protein diet and milk from which BCAAs have been removed. LT has been shown effective in patients with MSUD. This report describes the case of a 15-month-old boy who received a liver graft from his mother. Transplantation was successful, and the patient was then able to ingest a normal diet. Despite episodes of acute rejection, chylous ascites, and high fever (40 °C), he has shown no evidence of MSUD recurrence. These findings indicate that patients with MSUD can be successfully treated by LDLT, even when the donor is a heterozygous carrier of a mutated BCKDH gene. © 2016 The Authors. Pediatric Transplantation Published by Wiley Periodicals, Inc.

A Review of Selected Genes with Known Effects on Performance and Health of Cattle

PubMed Central

Casas, Eduardo; Kehrli, Marcus E.

2016-01-01

There are genetic conditions that influence production in dairy and beef cattle. The objective of this review was to describe relevant genetic conditions that have been associated with productivity and health in cattle. Genes or genomic regions that have been identified as a candidate for the condition will be included, and the genetic basis of the condition will be defined. Genes and genetic conditions included in this review are bovine leukocyte adhesion deficiency, deficiency of the uridine monophosphate synthase, bovine chronic interstitial nephritis, horn development, myostatin, complex vertebral malformation, leptin, osteopetrosis, apoptosis peptide activating factor 1, chondrodysplastic dwarfism, caseins, calpastatin, umbilical hernia, lactoglobulin, citrullinemia, cholesterol deficiency, prions, thyroglobulin, diacylglycerol acyltransferase, syndactyly, maple syrup urine disease, slick hair, Factor XI deficiency, and μ-Calpain. This review is not meant to be comprehensive, and relevant information is provided to ascertain genetic markers associated with the conditions. PMID:28018909

Modeled effects of soil acidification on long-term ecological and economic outcomes for managed forests in the Adirondack region (USA).

PubMed

Caputo, Jesse; Beier, Colin M; Sullivan, Timothy J; Lawrence, Gregory B

2016-09-15

Sugar maple (Acer saccharum) is among the most ecologically and economically important tree species in North America, and its growth and regeneration is often the focus of silvicultural practices in northern hardwood forests. A key stressor for sugar maple (SM) is acid rain, which depletes base cations from poorly-buffered forest soils and has been associated with much lower SM vigor, growth, and recruitment. However, the potential interactions between forest management and soil acidification - and their implications for the sustainability of SM and its economic and cultural benefits - have not been investigated. In this study, we simulated the development of 50 extant SM stands in the western Adirondack region of NY (USA) for 100years under different soil chemical conditions and silvicultural prescriptions. We found that interactions between management prescription and soil base saturation will strongly shape the ability to maintain SM in managed forests. Below 12% base saturation, SM did not regenerate sufficiently after harvest and was replaced mainly by red maple (Acer rubrum) and American beech (Fagus grandifolia). Loss of SM on acid-impaired sites was predicted regardless of whether the shelterwood or diameter-limit prescriptions were used. On soils with sufficient base saturation, models predicted that SM will regenerate after harvest and be sustained for future rotations. We then estimated how these different post-harvest outcomes, mediated by acid impairment of forest soils, would affect the potential monetary value of ecosystem services provided by SM forests. Model simulations indicated that a management strategy focused on syrup production - although not feasible across the vast areas where acid impairment has occurred - may generate the greatest economic return. Although pollution from acid rain is declining, its long-term legacy in forest soils will shape future options for sustainable forestry and ecosystem stewardship in the northern hardwood forests of North America. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

Modeled effects of soil acidification on long-term ecological and economic outcomes for managed forests in the Adirondack region (USA)

USGS Publications Warehouse

Caputo, Jesse PhD.; Beier, Colin M.; Sullivan, Timothy J.; Lawrence, Gregory B.

2016-01-01

Sugar maple (Acer saccharum) is among the most ecologically and economically important tree species in North America, and its growth and regeneration is often the focus of silvicultural practices in northern hardwood forests. A key stressor for sugar maple (SM) is acid rain, which depletes base cations from poorly-buffered forest soils and has been associated with much lower SM vigor, growth, and recruitment. However, the potential interactions between forest management and soil acidification – and their implications for the sustainability of SM and its economic and cultural benefits – have not been investigated. In this study, we simulated the development of 50 extant SM stands in the western Adirondack region of NY (USA) for 100 years under different soil chemical conditions and silvicultural prescriptions. We found that interactions between management prescription and soil base saturation will strongly shape the ability to maintain SM in managed forests. Below 12% base saturation, SM did not regenerate sufficiently after harvest and was replaced mainly by red maple (Acer rubrum) and American beech (Fagus grandifolia). Loss of SM on acid-impaired sites was predicted regardless of whether the shelterwood or diameter-limit prescriptions were used. On soils with sufficient base saturation, models predicted that SM will regenerate after harvest and be sustained for future rotations. We then estimated how these different post-harvest outcomes, mediated by acid impairment of forest soils, would affect the potential monetary value of ecosystem services provided by SM forests. Model simulations indicated that a management strategy focused on syrup production – although not feasible across the vast areas where acid impairment has occurred – may generate the greatest economic return. Although pollution from acid rain is declining, its long-term legacy in forest soils will shape future options for sustainable forestry and ecosystem stewardship in the northern hardwood forests of North America.

Identification of protoxins and a microbial basis for red maple (Acer rubrum) toxicosis in equines.

PubMed

Agrawal, Karan; Ebel, Joseph G; Altier, Craig; Bischoff, Karyn

2013-01-01

The leaves of Acer rubrum (red maple), especially when wilted in the fall, cause severe oxidative damage to equine erythrocytes, leading to potentially fatal methemoglobinemia and hemolytic anemia. Gallic acid and tannins from A. rubrum leaves have been implicated as the toxic compounds responsible for red maple toxicosis, but the mechanism of action and toxic principle(s) have not been elucidated to date. In order to investigate further how red maple toxicosis occurs, aqueous solutions of gallic acid, tannic acid, and ground dried A. rubrum leaves were incubated with contents of equine ileum, jejunum, cecum, colon, and liver, and then analyzed for the metabolite pyrogallol, as pyrogallol is a more potent oxidizing agent. Gallic acid was observed to be metabolized to pyrogallol maximally in equine ileum contents in the first 24 hr. Incubation of tannic acid and A. rubrum leaves, individually with ileum contents, produced gallic acid and, subsequently, pyrogallol. Ileum suspensions, when passed through a filter to exclude microbes but not enzymes, formed no pyrogallol, suggesting a microbial basis to the pathway. Bacteria isolated from ileum capable of pyrogallol formation were identified as Klebsiella pneumoniae and Enterobacter cloacae. Therefore, gallotannins and free gallic acid are present in A. rubrum leaves and can be metabolized by K. pneumoniae and E. cloacae found in the equine ileum to form pyrogallol either directly or through a gallic acid intermediate (gallotannins). Identification of these compounds and their physiological effects is necessary for the development of effective treatments for red maple toxicosis in equines.

Response of naturally regenerated and underplanted white oak (Quercus alba L.) seedlings 6 years following midstory removal

Treesearch

Jared M. Craig; John M. Lhotka; Jeffrey W. Stringer

2013-01-01

Historically, the abundance of red maple and shade-tolerant understory species was limited by fire and other disturbances. In the absence of periodic disturbance, regeneration of oaks with intermediate shade tolerance has been hindered due to inadequate light conditions created by shade-tolerant midstory trees. Research suggests that midstory removal in oak-dominated...

Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain α-ketoacid dehydrogenase kinase

PubMed Central

Qi, Xiangbing; Gui, Wen-Jun; Morlock, Lorraine K.; Wallace, Amy L.; Ahmed, Kamran; Laxman, Sunil; Campeau, Philippe M.; Lee, Brendan H.; Hutson, Susan M.; Tu, Benjamin P.; Williams, Noelle S.; Tambar, Uttam K.; Wynn, R. Max; Chuang, David T.

2013-01-01

The branched-chain amino acids (BCAAs) leucine, isoleucine, and valine are elevated in maple syrup urine disease, heart failure, obesity, and type 2 diabetes. BCAA homeostasis is controlled by the mitochondrial branched-chain α-ketoacid dehydrogenase complex (BCKDC), which is negatively regulated by the specific BCKD kinase (BDK). Here, we used structure-based design to develop a BDK inhibitor, (S)-α-chloro-phenylpropionic acid [(S)-CPP]. Crystal structures of the BDK-(S)-CPP complex show that (S)-CPP binds to a unique allosteric site in the N-terminal domain, triggering helix movements in BDK. These conformational changes are communicated to the lipoyl-binding pocket, which nullifies BDK activity by blocking its binding to the BCKDC core. Administration of (S)-CPP to mice leads to the full activation and dephosphorylation of BCKDC with significant reduction in plasma BCAA concentrations. The results buttress the concept of targeting mitochondrial BDK as a pharmacological approach to mitigate BCAA accumulation in metabolic diseases and heart failure. PMID:23716694

Species-specific transpiration responses to intermediate disturbance in a northern hardwood forest

NASA Astrophysics Data System (ADS)

Matheny, Ashley M.; Bohrer, Gil; Vogel, Christoph S.; Morin, Timothy H.; He, Lingli; Frasson, Renato Prata de Moraes; Mirfenderesgi, Golnazalsadat; Schäfer, Karina V. R.; Gough, Christopher M.; Ivanov, Valeriy Y.; Curtis, Peter S.

2014-12-01

Intermediate disturbances shape forest structure and composition, which may in turn alter carbon, nitrogen, and water cycling. We used a large-scale experiment in a forest in northern lower Michigan where we prescribed an intermediate disturbance by stem girdling all canopy-dominant early successional trees to simulate an accelerated age-related senescence associated with natural succession. Using 3 years of eddy covariance and sap flux measurements in the disturbed area and an adjacent control plot, we analyzed disturbance-induced changes to plot level and species-specific transpiration and stomatal conductance. We found transpiration to be 15% lower in disturbed plots than in unmanipulated control plots. However, species-specific responses to changes in microclimate varied. While red oak and white pine showed increases in stomatal conductance during postdisturbance (62.5 and 132.2%, respectively), red maple reduced stomatal conductance by 36.8%. We used the hysteresis between sap flux and vapor pressure deficit to quantify diurnal hydraulic stress incurred by each species in both plots. Red oak, a ring porous anisohydric species, demonstrated the largest mean relative hysteresis, while red maple, bigtooth aspen, and paper birch, all diffuse porous species, had the lowest relative hysteresis. We employed the Penman-Monteith model for LE to demonstrate that these species-specific responses to disturbance are not well captured using current modeling strategies and that accounting for changes to leaf area index and plot microclimate are insufficient to fully describe the effects of disturbance on transpiration.

Silvical characteristics of red maple (Acer rubrum)

Treesearch

Russell J. Hutnik; Harry W. Yawney

1961-01-01

Red maple (Acer rubrum L.) is also known as Carolina red maple, scarlet maple, soft maple, swamp maple, water maple, and white maple. Taxonomists recognize several varieties of red maple. The most common is Drummond red maple (Acer rubrum var. drummondii (Hook, & Arn.) Sarg.).

The Pasting and Gel Textural Properties of Corn Starch in Glucose, Fructose and Maltose Syrup

PubMed Central

Sun, Qingjie; Xing, Yan; Qiu, Chao; Xiong, Liu

2014-01-01

The pasting and gel textural properties of corn starch in syrup at different concentrations were investigated by Rapid Visco Analyzer (RVA) and Texture profile analysis (TPA) tests. The results showed that the pasting temperatures of corn starch greatly increased, especially at higher sugar concentration. Increasing concentration of syrup caused an increase in peak, trough and final viscosity of corn starch. Peak viscosity and the disintegration rate of starch increased in the following order: fructose syrup> maltose syrup> glucose syrup. Increasing syrup concentration to 13%, 25% and 50% resulted in a lower retrogradation rate than the control. When the maltose syrup concentration increased to 50%, the retrogradation rate decreased to 14.30% from 33.38%. The highest hardness was observed when the syrup concentration was 25%. There was a particular low hardness when the concentration of syrup was 50%. The springiness of starch gels in syrup was similar at different concentrations. PMID:24755772

The pasting and gel textural properties of corn starch in glucose, fructose and maltose syrup.

PubMed

Sun, Qingjie; Xing, Yan; Qiu, Chao; Xiong, Liu

2014-01-01

The pasting and gel textural properties of corn starch in syrup at different concentrations were investigated by Rapid Visco Analyzer (RVA) and Texture profile analysis (TPA) tests. The results showed that the pasting temperatures of corn starch greatly increased, especially at higher sugar concentration. Increasing concentration of syrup caused an increase in peak, trough and final viscosity of corn starch. Peak viscosity and the disintegration rate of starch increased in the following order: fructose syrup> maltose syrup> glucose syrup. Increasing syrup concentration to 13%, 25% and 50% resulted in a lower retrogradation rate than the control. When the maltose syrup concentration increased to 50%, the retrogradation rate decreased to 14.30% from 33.38%. The highest hardness was observed when the syrup concentration was 25%. There was a particular low hardness when the concentration of syrup was 50%. The springiness of starch gels in syrup was similar at different concentrations.

Classical maple syrup urine disease and brain development: principles of management and formula design.

PubMed

Strauss, Kevin A; Wardley, Bridget; Robinson, Donna; Hendrickson, Christine; Rider, Nicholas L; Puffenberger, Erik G; Shellmer, Diana; Shelmer, Diana; Moser, Ann B; Morton, D Holmes

2010-04-01

Branched-chain ketoacid dehydrogenase deficiency results in complex and volatile metabolic derangements that threaten brain development. Treatment for classical maple syrup urine disease (MSUD) should address this underlying physiology while also protecting children from nutrient deficiencies. Based on a 20-year experience managing 79 patients, we designed a study formula to (1) optimize transport of seven amino acids (Tyr, Trp, His, Met, Thr, Gln, Phe) that compete with branched-chain amino acids (BCAAs) for entry into the brain via a common transporter (LAT1), (2) compensate for episodic depletions of glutamine, glutamate, and alanine caused by reverse transamination, and (3) correct deficiencies of omega-3 essential fatty acids, zinc, and selenium widespread among MSUD patients. The formula was enriched with LAT1 amino acid substrates, glutamine, alanine, zinc, selenium, and alpha-linolenic acid (18:3n-3). Fifteen Old Order Mennonite children were started on study formula between birth and 34 months of age and seen at least monthly in the office. Amino acid levels were checked once weekly and more often during illnesses. All children grew and developed normally over a period of 14-33 months. Energy demand, leucine tolerance, and protein accretion were tightly linked during periods of normal growth. Rapid shifts to net protein degradation occurred during illnesses. At baseline, most LAT1 substrates varied inversely with plasma leucine, and their calculated rates of brain uptake were 20-68% below normal. Treatment with study formula increased plasma concentrations of LAT1 substrates and normalized their calculated uptakes into the nervous system. Red cell membrane omega-3 polyunsaturated fatty acids and serum zinc and selenium levels increased on study formula. However, selenium and docosahexaenoic acid (22:6n-3) levels remained below normal. During the study period, hospitalizations decreased from 0.35 to 0.14 per patient per year. There were 28 hospitalizations managed with MSUD hyperalimentation solution; 86% were precipitated by common infections, especially vomiting and gastroenteritis. The large majority of catabolic illnesses were managed successfully at home using 'sick-day' formula and frequent amino acid monitoring. We conclude that the study formula is safe and effective for the treatment of classical MSUD. In principle, dietary enrichment protects the brain against deficiency of amino acids used for protein accretion, neurotransmitter synthesis, and methyl group transfer. Although the pathophysiology of MSUD can be addressed through rational formula design, this does not replace the need for vigilant clinical monitoring, frequent measurement of the complete amino acid profile, and ongoing dietary adjustments that match nutritional intake to the metabolic demands of growth and illness. Copyright 2009 Elsevier Inc. All rights reserved.

Classical maple syrup urine disease and brain development: Principles of management and formula design

PubMed Central

Strauss, Kevin A.; Wardley, Bridget; Robinson, Donna; Hendrickson, Christine; Rider, Nicholas L.; Puffenberger, Erik G.; Shelmer, Diana; Moser, Ann B.; Morton, D. Holmes

2012-01-01

Branched-chain ketoacid dehydrogenase deficiency results in complex and volatile metabolic derangements that threaten brain development. Treatment for classical maple syrup urine disease (MSUD) should address this underlying physiology while also protecting children from nutrient deficiencies. Based on a 20-year experience managing 79 patients, we designed a study formula to (1) optimize transport of seven amino acids (Tyr, Trp, His, Met, Thr, Gln, Phe) that compete with branched-chain amino acids (BCAAs) for entry into the brain via a common transporter (LAT1), (2) compensate for episodic depletions of glutamine, glutamate, and alanine caused by reverse transamination, and (3) correct deficiencies of omega-3 essential fatty acids, zinc, and selenium widespread among MSUD patients. The formula was enriched with LAT1 amino acid substrates, glutamine, alanine, zinc, selenium, and alphalinolenic acid (18:3n – 3). Fifteen Old Order Mennonite children were started on study formula between birth and 34 months of age and seen at least monthly in the office. Amino acid levels were checked once weekly and more often during illnesses. All children grew and developed normally over a period of 14– 33 months. Energy demand, leucine tolerance, and protein accretion were tightly linked during periods of normal growth. Rapid shifts to net protein degradation occurred during illnesses. At baseline, most LAT1 substrates varied inversely with plasma leucine, and their calculated rates of brain uptake were 20–68% below normal. Treatment with study formula increased plasma concentrations of LAT1 substrates and normalized their calculated uptakes into the nervous system. Red cell membrane omega-3 polyunsaturated fatty acids and serum zinc and selenium levels increased on study formula. However, selenium and docosahexaenoic acid (22:6n – 3) levels remained below normal. During the study period, hospitalizations decreased from 0.35 to 0.14 per patient per year. There were 28 hospitalizations managed with MSUD hyperalimentation solution; 86% were precipitated by common infections, especially vomiting and gastroenteritis. The large majority of catabolic illnesses were managed successfully at home using ‘sickday’ formula and frequent amino acid monitoring. We conclude that the study formula is safe and effective for the treatment of classical MSUD. In principle, dietary enrichment protects the brain against deficiency of amino acids used for protein accretion, neurotransmitter synthesis, and methyl group transfer. Although the pathophysiology of MSUD can be addressed through rational formula design, this does not replace the need for vigilant clinical monitoring, frequent measurement of the complete amino acid profile, and ongoing dietary adjustments that match nutritional intake to the metabolic demands of growth and illness. PMID:20061171

Tree species and soil nutrient profiles in old-growth forests of the Oregon Coast Range

USGS Publications Warehouse

Cross, Alison; Perakis, Steven S.

2011-01-01

Old-growth forests of the Pacific Northwest provide a unique opportunity to examine tree species – soil relationships in ecosystems that have developed without significant human disturbance. We characterized foliage, forest floor, and mineral soil nutrients associated with four canopy tree species (Douglas-fir (Pseudotsuga menziesii (Mirbel) Franco), western hemlock (Tsuga heterophylla (Raf.) Sarg.), western redcedar (Thuja plicata Donn ex D. Don), and bigleaf maple (Acer macrophyllum Pursh)) in eight old-growth forests of the Oregon Coast Range. The greatest forest floor accumulations of C, N, P, Ca, Mg, and K occurred under Douglas-fir, primarily due to greater forest floor mass. In mineral soil, western hemlock exhibited significantly lower Ca concentration and sum of cations (Ca + Mg + K) than bigleaf maple, with intermediate values for Douglas-fir and western redcedar. Bigleaf maple explained most species-based differences in foliar nutrients, displaying high concentrations of N, P, Ca, Mg, and K. Foliar P and N:P variations largely reflected soil P variation across sites. The four tree species that we examined exhibited a number of individualistic effects on soil nutrient levels that contribute to biogeochemical heterogeneity in these ecosystems. Where fire suppression and long-term succession favor dominance by highly shade-tolerant western hemlock, our results suggest a potential for declines in both soil Ca availability and soil biogeochemical heterogeneity in old-growth forests.

Removal or retention of unmerchantable saplings in Allegheny hardwoods: effect on regeneration after clearcutting

DOE Office of Scientific and Technical Information (OSTI.GOV)

Marquis, D.A.

1981-01-01

Merchantable trees in an old growth stand (mainly beech, (Fagus grandifolia) and supar maple) in NW Pennsylvania were clear felled in September and October 1937, and 4 plots set up containing (a) large (d.b.h. over 1.5 inch), and small residual saplings, (b) primarily small residuals (1-inch d.b.h.) and (c) plots as (a) and (b) but with saplings over 0.5 inch d.b.h. chopped down (moved) in April and May 1938. Plots were tallied in 1938 after mowing, and in 1942, 1947, 1957 and 1972. After 35 years, the 2 mowed plots (c) were dominated by fast-growing, shade-intolerant black cherry (Prunus serotina)more » which accounted for 65% of the b.a. The plot with treatment (a) had fewer, larger trees with some sawtimber size maple and beech. Cherry accounted for 32% b.a. The plot with treatment (b) had trees intermediate in size and composition (cherry 53% b.a.). (Refs. 10).« less

Detecting adulterated commercial sweet sorghum syrups with ion chromatography oligosaccharide fingerprint profiles

USDA-ARS?s Scientific Manuscript database

Some commercial sweet sorghum syrups can be fraudulently or accidently adulterated with inexpensive sugar syrups, particularly high fructose corn syrup (HFCS) or corn syrup, and sold at a relatively low market price or even mis-branded. This undermines the economic stability of the current small-sc...

Detecting adulterated commercial sweet sorghum syrups with ion chromatography oligosaccharide fingerprint profiles

USDA-ARS?s Scientific Manuscript database

Commercial sweet sorghum syrups can be adulterated with inexpensive sugar syrups, particularly high fructose corn syrup (HFCS) or corn syrup, and sold at a relatively low market price or even mis-branded. This undermines the economic stability of the current small-scale producers of food-grade swee...

Application of Fourier transform midinfrared spectroscopy to the discrimination between Irish artisanal honey and such honey adulterated with various sugar syrups.

PubMed

Kelly, J Daniel; Petisco, Cristina; Downey, Gerard

2006-08-23

A collection of authentic artisanal Irish honeys (n = 580) and certain of these honeys adulterated by fully inverted beet syrup (n = 280), high-fructose corn syrup (n = 160), partial invert cane syrup (n = 120), dextrose syrup (n = 160), and beet sucrose (n = 120) was assembled. All samples were adjusted to 70 degrees Bx and scanned in the midinfrared region (800-4000 cm(-1)) by attenuated total reflectance sample accessory. By use of soft independent modeling of class analogy (SIMCA) and partial least-squares (PLS) classification, authentic honey and honey adulterated by beet sucrose, dextrose syrups, and partial invert corn syrup could be identified with correct classification rates of 96.2%, 97.5%, 95.8%, and 91.7%, respectively. This combination of spectroscopic technique and chemometric methods was not able to unambiguously detect adulteration by high-fructose corn syrup or fully inverted beet syrup.

Identification, classification, and discrimination of agave syrups from natural sweeteners by infrared spectroscopy and HPAEC-PAD.

PubMed

Mellado-Mojica, Erika; López, Mercedes G

2015-01-15

Agave syrups are gaining popularity as new natural sweeteners. Identification, classification and discrimination by infrared spectroscopy coupled to chemometrics (NIR-MIR-SIMCA-PCA) and HPAEC-PAD of agave syrups from natural sweeteners were achieved. MIR-SIMCA-PCA allowed us to classify the natural sweeteners according to their natural source. Natural syrups exhibited differences in the MIR spectra region 1500-900 cm(-1). The agave syrups displayed strong absorption in the MIR spectra region 1061-1,063 cm(-1), in agreement with their high fructose content. Additionally, MIR-SIMCA-PCA allowed us to differentiate among syrups from different Agave species (Agavetequilana and Agavesalmiana). Thin-layer chromatography and HPAEC-PAD revealed glucose, fructose, and sucrose as the principal carbohydrates in all of the syrups. Oligosaccharide profiles showed that A. tequilana syrups are mainly composed of fructose (>60%) and fructooligosaccharides, while A. salmiana syrups contain more sucrose (28-32%). We conclude that MIR-SIMCA-PCA and HPAEC-PAD can be used to unequivocally identify and classified agave syrups. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

Detection of adulteration in mulberry pekmez samples added various sugar syrups with ¹³C/¹²C isotope ratio analysis method.

PubMed

Tosun, Murat

2014-12-15

Mulberry pekmez can be adulterated in different ways either during the production process or after production is completed. To identify these adulterations, stable carbon isotope ratio analysis (SCIRA) was performed on the model examples prepared by adding saccharose syrup (SS), glucose syrup (GS) and high fructose corn syrup (HFCS) into two different pure mulberry pekmez samples in the ratios of 0%, 10%, 30% and 50%. The δ(13)C ratio of the pure mulberry pekmez was determined as -26.60‰ on average, the saccharose syrup as -24.80‰, the glucose syrup as -11.20‰ and the high-fructose corn syrup as -11.40‰. In identifying the adulteration made to pekmez, especially with the high-fructose corn syrup, which is obtained from corn starch, and with the glucose syrup, the δ(13)C ratio comes into prominence. However it remains impossible identify the adulterations made with the saccharose, which is obtained from beet sugar, or invert sugar syrups. Copyright © 2014 Elsevier Ltd. All rights reserved.

7 CFR 1437.107 - Maple sap.

Code of Federal Regulations, 2014 CFR

2014-01-01

... Yield Coverage Using Actual Production History § 1437.107 Maple sap. (a) NAP assistance for maple sap is... maple sap. (g) The actual production history for maple sap shall be recorded on the basis of gallons of...

7 CFR 1437.107 - Maple sap.

Code of Federal Regulations, 2011 CFR

2011-01-01

... Yield Coverage Using Actual Production History § 1437.107 Maple sap. (a) NAP assistance for maple sap is... maple sap. (g) The actual production history for maple sap shall be recorded on the basis of gallons of...

The Halogen Demand of Commercial Beverage Powders, Drinks and Their Constituents

DTIC Science & Technology

1982-02-01

Corn Syrup . Best Foods,CPC International Inc., N.J. Ingredients: Light Corn Oil, Salt, Vanilla Fructose Corn Syrup . 67 Gatorade...Sucrose 58 Dextrose 59 d-Levulose 12 60 d-Xylose 61 Sorbitol 62 Mannitol 63 Sodium Saccharin 64 Sweet’n Low 65 Glucose Sucrose Syrup 66 Corn Syrup 9...Qt - - - - - - 64 Sweet’n Low 5 5.95 5.40 5.31 5.17 5.08 7.0 g/Qt - - - - - - 65 Glucose-Sucrose - - Syrup 85 g/Qt - - - - - - 66 Corn Syrup

21 CFR 184.1865 - Corn syrup.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 21 Food and Drugs 3 2011-04-01 2011-04-01 false Corn syrup. 184.1865 Section 184.1865 Food and... Substances Affirmed as GRAS § 184.1865 Corn syrup. (a) Corn syrup, commonly called “glucose sirup” or “glucose syrup,” is obtained by partial hydrolysis of corn starch with safe and suitable acids or enzymes...

21 CFR 184.1865 - Corn syrup.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 3 2010-04-01 2009-04-01 true Corn syrup. 184.1865 Section 184.1865 Food and... Substances Affirmed as GRAS § 184.1865 Corn syrup. (a) Corn syrup, commonly called “glucose sirup” or “glucose syrup,” is obtained by partial hydrolysis of corn starch with safe and suitable acids or enzymes...

21 CFR 168.140 - Maple sirup.

Code of Federal Regulations, 2010 CFR

2010-04-01

... § 168.140 Maple sirup. (a) Maple sirup is the liquid food derived by concentration and heat treatment of the sap of the maple tree (Acer) or by solution in water of maple sugar (mapel concrete) made from...

21 CFR 168.140 - Maple sirup.

Code of Federal Regulations, 2013 CFR

2013-04-01

... § 168.140 Maple sirup. (a) Maple sirup is the liquid food derived by concentration and heat treatment of the sap of the maple tree (Acer) or by solution in water of maple sugar (mapel concrete) made from...

21 CFR 168.140 - Maple sirup.

Code of Federal Regulations, 2014 CFR

2014-04-01

... § 168.140 Maple sirup. (a) Maple sirup is the liquid food derived by concentration and heat treatment of the sap of the maple tree (Acer) or by solution in water of maple sugar (mapel concrete) made from...

21 CFR 168.140 - Maple sirup.

Code of Federal Regulations, 2012 CFR

2012-04-01

... § 168.140 Maple sirup. (a) Maple sirup is the liquid food derived by concentration and heat treatment of the sap of the maple tree (Acer) or by solution in water of maple sugar (mapel concrete) made from...

21 CFR 168.140 - Maple sirup.

Code of Federal Regulations, 2011 CFR

2011-04-01

... § 168.140 Maple sirup. (a) Maple sirup is the liquid food derived by concentration and heat treatment of the sap of the maple tree (Acer) or by solution in water of maple sugar (mapel concrete) made from...

[Safety Evaluation of Rare Sugar Syrup: Single-dose Oral Toxicity in Rats, Reverse Mutation Assay, Chromosome Aberration Assay, and Acute Non-Effect Level for Diarrhea of a Single Dose in Humans].

PubMed

Yamada, Takako; Iida, Tetsuo; Takamine, Satoshi; Hayashi, Noriko; Okuma, Kazuhiro

2015-01-01

The safety of rare sugar syrup obtained from high-fructose corn syrup under slightly alkaline conditions was studied. Mutagenicity of rare sugar syrup was assessed by a reverse mutation assay using Salmonella typhimurium and Escherichia coli, and an in vitro chromosomal aberration assay using Chinese hamster lung cell line (CHL/IU). No mutagenicity of rare sugar syrup was detected under these experimental conditions. Oral administration of single dose (15,000 mg/kg) of rare sugar syrup to rats caused no abnormalities, suggesting no adverse effect of rare sugar syrup. In humans, the acute non-effect level of rare sugar syrup for causing diarrhea was estimated as 0.9 g/kg body weight as dry solid base in both males and females.

Process quality planning of quality function deployment for carrot syrup

NASA Astrophysics Data System (ADS)

Ekawati, Yurida; Noya, Sunday; Widjaja, Filemon

2017-06-01

Carrot products are rarely available in the market. Based on previous research that had been done using QFD to generate product design of carrots products, the research to produce the process quality planning had been carried out. The carrot product studied was carrot syrup. The research resulted in a process planning matrix for carrot syrup. The matrix gives information about critical process plan and the priority of the critical process plan. The critical process plan on the production process of carrot syrup consists of carrots sorting, carrots peeling, carrots washing, blanching process, carrots cutting, the making of pureed carrots, filtering carrot juice, the addition of sugar in carrot juice, the addition of food additives in carrot juice, syrup boiling, syrup filtering, syrup filling into the bottle, the bottle closure and cooling. The information will help the design of the production process of carrot syrup.

Observational study on the dispensing of cough syrups to children with acute cough by community pharmacists in France.

PubMed

Allaert, François-André; Villet, Stéphanie; Vincent, Stéphane; Sauve, Laurent

2018-04-01

Over-the-counter medicines may be proposed by pharmacists for children with acute cough. Study objectives were to describe the sociodemographic profile of children who were proposed a cough syrup by a pharmacist, the nature of the cough and type(s) of cough syrup proposed and to assess the evolution of the cough, tolerance and satisfaction with treatment. Observational, prospective, longitudinal, multicentre study with 157 pharmacies in France. Children who were proposed a cough syrup by a pharmacist were recruited. Questionnaires were completed by the pharmacists and/or parents at inclusion and by the parents after 5 days of treatment. Four hundred fourteen children were included (mean age: 6.0±2.9 years); 45.9% had a dry and 43.3% a productive cough. 30.4% were proposed an allopathic antitussive syrup, 28.3% an allopathic expectorant syrup and 23.7% a homeopathic syrup. Children with a dry cough were more likely to be given an allopathic antitussive (55.2%) or homeopathic (28.2%) syrup. Children with a productive cough or cough of several days duration were more likely to be given an allopathic expectorant syrup (70.1%). Cough disappearance was more frequent with homeopathic syrups compared to allopathic expectorants (P=0.002), or allopathic antitussives (P=0.042). Adverse events were most common with allopathic antitussive syrups (18.7%) (P<0.001). Two-thirds of parents were satisfied with the treatment their child received. Pharmacists play an important role in the management of acute cough in children. Homeopathic cough syrups may have an interest in terms of public health.

Living donor liver transplantation in maple syrup urine disease - Case series and world's youngest domino liver donor and recipient.

PubMed

Mohan, N; Karkra, S; Rastogi, A; Vohra, V; Soin, A S

2016-05-01

MSUD occurs due to deficiency of enzyme BCKAD required for metabolism of leucine, isoleucine, and valine leading to the accumulation of these and their ketoacids causing acute metabolic decompensation manifesting as encephalopathy or sudden death. The patient requires special protein-restricted diet to survive. As this enzyme is expressed in liver, liver transplantation has been successfully performed as a cure. We report two patients of MSUD who underwent LDLT while their livers were used as a domino graft for other biliary cirrhotic patients. A 22-month-old male child diagnosed as a case of classic MSUD underwent LDLT from an altruistic aunt as donor following which his serum leucine levels normalized on an unrestricted protein diet. His liver was used as a domino graft. A 38-month-old female child with diagnosed MSUD underwent LDLT from a swap donor, and her liver was used as a domino graft. Her DQ improved post-transplant. LDLT from non-heterozygous donors is a cure for classical MSUD. Their livers can be used as domino grafts for non-MSUD cases. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders

PubMed Central

Burrage, Lindsay C.; Nagamani, Sandesh C.S.; Campeau, Philippe M.; Lee, Brendan H.

2014-01-01

Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syrup urine disease (MSUD) for decades, treatment options for this disorder have been limited to dietary interventions. In recent years, the discovery of improved leucine tolerance after liver transplantation has resulted in a new therapeutic strategy for this disorder. Likewise, targeting the regulation of the BCKDC activity may be an alternative potential treatment strategy for MSUD. The regulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated in a new inborn error of metabolism characterized by autism, intellectual disability and seizures. Finally, there is a growing body of literature implicating BCAA metabolism in more common disorders such as the metabolic syndrome, cancer and hepatic disease. This review surveys the knowledge acquired on the topic over the past 50 years and focuses on recent developments in the field of BCAA metabolism. PMID:24651065

Heterozygous liver transplantation for maple syrup urine disease: First European reported case.

PubMed

Roilides, I; Xinias, I; Mavroudi, A; Ioannou, H; Savopoulou, P; Imvrios, G

2016-09-01

MSUD is an autosomal recessive metabolic disorder that results from a defect in the BCKDH enzyme. This enzyme is essential for the second step in the metabolism of the branched-chain amino acids, leucine, isoleucine, and valine. Patients with MSUD are subject to severe, irreversible neurologic injury unless closely managed with a specialized metabolic formula and a diet restricted in leucine throughout their lifetime. During times of illness, patients with MSUD can suffer from severe metabolic derangement, acute cerebral edema, and untimely death. Deceased donor liver transplant restores the ability to metabolize branched-chain amino acids, even on an unrestricted diet, and prevents metabolic derangements during times of illness. We report a successful case of living donor (parental) transplant for a child with MSUD. The donor was the child's father. This approach has been controversial as parents of children with MSUD are obligate heterozygotes for the condition and have diminished levels of BCKDH activity. If effective, living-related donor transplant provides a promising alternative for deceased donor liver transplant, which often requires a prolonged waiting period and may not be feasible in areas with limited medical resources. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Living donor liver transplantation from a heterozygous parent for classical maple syrup urine disease.

PubMed

Kadohisa, Masashi; Matsumoto, Shirou; Sawada, Hirotake; Honda, Masaki; Murokawa, Takahiro; Hayashida, Shintaro; Ohya, Yuki; Lee, Kwang-Jong; Yamamoto, Hidekazu; Mitsubuchi, Hiroshi; Endo, Fumio; Inomata, Yukihiro

2015-05-01

MSUD is a hereditary metabolic disorder that is characterized by impaired activity of the BCKADC. Liver transplantation has been approved as a treatment for some MSUD cases in which the control of BCAAs is insufficient. Although there have been several reports about DDLT for MSUD, few LDLT cases have been reported. Because either of parents who are heterozygote of this disease usually applies to be a candidate of donor in LDLT, the impairment of BCKADC activity of graft liver should be concerned. We performed LDLT for 10 month-old girl with a left lateral segment graft from her father. BCKADC activities of the patient and her parents were measured using lysates of lymphocytes isolated from peripheral blood specimen before the transplant. As a consequence, the activity of BCKADC of father was not inferior to a normal range. The patient tolerated the operation well. Postoperative course was uneventful and mixed milk was started at 8th POD. The serum BCAAs levels have remained within normal range. It should be necessary to follow the physical growth and mental development of the recipient in the future. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Analysis of 26 amino acids in human plasma by HPLC using AQC as derivatizing agent and its application in metabolic laboratory.

PubMed

Sharma, Gaurav; Attri, Savita Verma; Behra, Bijaylaxmi; Bhisikar, Swapnil; Kumar, Praveen; Tageja, Minni; Sharda, Sheetal; Singhi, Pratibha; Singhi, Sunit

2014-05-01

The present study reports the simultaneous analysis of 26 physiological amino acids in plasma along with total cysteine and homocysteine by high-performance liquid chromatography (HPLC) employing 6-aminoquinolyl-N-hydroxysuccinimidyl carbamate (AQC) as precolumn derivatizing reagent. Separations were carried out using Lichrospher 100 RP-18e (5 μm) 250 × 4.0 mm column connected to 100 CN 4.0 × 4.0 mm guard column on a quaternary HPLC system and run time was 53 min. Linearity of the peak areas for different concentrations ranging from 2.5 to 100 pmol/μL of individual amino acids was determined. A good linearity (R (2) > 0.998) was achieved in the standard mixture for each amino acid. Recovery of amino acids incorporated at the time of derivatization ranged from 95 to 106 %. Using this method we have established the normative data of amino acids in plasma, the profile being comparable to the range reported in literature and identified cases of classical homocystinuria, cobalamin defect/deficiency, non-ketotic hyperglycinemia, hyperprolinemia, ketotic hyperglycinemia, urea cycle defect and maple syrup urine disease.

Storage stability of a commercial hen egg yolk powder in dry and intermediate-moisture food matrices.

PubMed

Rao, Qinchun; Fisher, Mary Catherine; Guo, Mufan; Labuza, Theodore P

2013-09-11

Quality loss in intermediate-moisture foods (IMF) such as high-protein nutrition bars (HPNB) in the form of hardening, nonenzymatic browning, and free amino group loss is a general concern for the manufacturers. To measure the extent of quality loss over time in terms of these negative attributes, through changing the ratio by weight between two commercial spray-dried hen egg powders, egg white (DEW) and egg yolk (DEY), the storage stability of 10 IMF systems (water activity (aw) ∼ 0.6) containing 5% glycerol, 10% shortening, 35% protein, and 50% sweetener (either maltitol or 50% high-fructose corn syrup/50% corn syrup (HFCS/CS)) were studied. Additionally, the storage stability of the DEY powder itself was investigated. Overall, during storage at different temperatures (23, 35, and 45 °C), the storage stability of DEY in dry and IMF matrices was mainly controlled by the coaction of three chemical reactions (disulfide bond interaction, Maillard reaction, and lipid oxidation). The results showed that by replacing 25% of DEW in an IMF model system with DEY, the rate of bar hardening was significantly lower than that of the models with only DEW at all temperatures due to the softening effect of the fat in DEY. Furthermore, the use of maltitol instead of HFCS/CS in all bar systems not only resulted in decreased hardness but also drastically decreased the change in the total color difference (ΔE*). Interestingly, there was no significant loss of free amino groups in the maltitol systems at any DEW/DEY ratio.

Twenty-four-hour endocrine and metabolic profiles following consumption of high-fructose corn syrup-, sucrose-, fructose-, and glucose-sweetened beverages with meals.

PubMed

Stanhope, Kimber L; Griffen, Steven C; Bair, Brandi R; Swarbrick, Michael M; Keim, Nancy L; Havel, Peter J

2008-05-01

We have reported that, compared with glucose-sweetened beverages, consuming fructose-sweetened beverages with meals results in lower 24-h circulating glucose, insulin, and leptin concentrations and elevated triacylglycerol (TG). However, pure fructose and glucose are not commonly used as sweeteners. High-fructose corn syrup (HFCS) has replaced sucrose as the predominant sweetener in beverages in the United States. We compared the metabolic/endocrine effects of HFCS with sucrose and, in a subset of subjects, with pure fructose and glucose. Thirty-four men and women consumed 3 isocaloric meals with either sucrose- or HFCS-sweetened beverages, and blood samples were collected over 24 h. Eight of the male subjects were also studied when fructose- or glucose-sweetened beverages were consumed. In 34 subjects, 24-h glucose, insulin, leptin, ghrelin, and TG profiles were similar between days that sucrose or HFCS was consumed. Postprandial TG excursions after HFCS or sucrose were larger in men than in women. In the men in whom the effects of 4 sweeteners were compared, the 24-h glucose and insulin responses induced by HFCS and sucrose were intermediate between the lower responses during consumption of fructose and the higher responses during glucose. Unexpectedly, postprandial TG profiles after HFCS or sucrose were not intermediate but comparably high as after pure fructose. Sucrose and HFCS do not have substantially different short-term endocrine/metabolic effects. In male subjects, short-term consumption of sucrose and HFCS resulted in postprandial TG responses comparable to those induced by fructose.

Twenty-four Hour Endocrine and Metabolic Profiles Following Consumption of High Fructose Corn Syrup-, Sucrose- Fructose-, and Glucose-Sweetened Beverages with Meals

PubMed Central

Stanhope, Kimber L.; Griffen, Steven C.; Bair, Brandi R.; Swarbrick, Michael M.; Keim, Nancy L.; Havel, Peter J.

2011-01-01

Background We have reported that compared with glucose-sweetened beverages, consuming fructose-sweetened beverages with meals results in lower 24-h circulating glucose, insulin and leptin concentrations, and elevated triacylglycerol (TG). However, pure fructose and glucose are not commonly used as sweeteners. High fructose corn syrup (HFCS) has replaced sucrose as the predominant sweetener in beverages in the U.S. Objective We compared the metabolic/endocrine effects of HFCS with sucrose, and in a subset of subjects with pure fructose and glucose. Design 34 men and women consumed 3 isocaloric meals with either sucrose- or HFCS-sweetened beverages, and blood samples were collected over 24 hours. Eight of the male subjects were also studied when fructose- or glucose-sweetened beverages were consumed. Results In 34 subjects, 24-h glucose, insulin, leptin, ghrelin and TG profiles were similar between days that sucrose or HFCS were consumed. Postprandial TG excursions after HFCS or sucrose were larger in men than women. In the men in whom the effects of 4 sweeteners were compared, the 24-h glucose and insulin responses induced by HFCS and sucrose were intermediate between the lower responses during consumption of fructose and the higher responses during glucose. Unexpectedly, postprandial TG profiles after HFCS or sucrose were not intermediate, but comparably high as after pure fructose. Conclusions Sucrose and HFCS do not have substantially different short-term endocrine/metabolic effects. In male subjects, short-term consumption of sucrose and HFCS resulted in postprandial TG responses comparable to those induced by fructose. PMID:18469239

Effect of a traditional syrup from Citrus medica L. fruit juice on migraine headache: A randomized double blind placebo controlled clinical trial.

PubMed

Jafarpour, Mehrnaz; Yousefi, Gholamhossein; Hamedi, Azadeh; Shariat, Abdolhamid; Salehi, Alireza; Heydari, Mojtaba

2016-02-17

In Persian ethnomedicine several herbal remedies and functional foods have been used to treat migraine headache which are mostly summarized in Qarabadin-e-kabir (Aghili-Shirazi MH, 1773). One of them is Citron syrup (Sharbat-e-Balang) containing edible Citrus medica L. fruit juice and sugar. The present study was designed to assess the efficacy and safety of Citron syrup on patients with migraine headache. Citron syrup was prepared as described in Qarabadin-e-kabir. In this double blind randomized placebo-controlled clinical trial, ninety patients with migraine headache were allocated to three parallel groups (Citron syrup, propranolol or placebo). Patients received 15ml of Citron syrup, placebo syrup or 20mg of propranolol tablet three times daily after a meal for 4 weeks. Primary outcomes were obtained from three measures: the frequency (per month), mean duration (hour) and mean intensity (visual analogue scale "VAS" 0-10 score) of headache attacks evaluated prior to and following 4 weeks of the intervention. Citron syrup was superior to placebo in reduction of headache attacks intensity (P<0.01) and duration (p<0.0001) and as effective as propranolol in patients with migraine headache (P>0.05). However, unlike propranolol, Citron syrup could not significantly reduce the frequency of attacks compared to placebo. No indication of any serious side effects from Citron syrup was observed. According to obtained results, Citron syrup as a traditional Persian remedy can be suggested as an effective treatment for decreasing pain intensity and duration of attacks in migraine headache and the effectiveness is comparable to propranolol. However, the syrup did not show significant effect on frequency of attacks. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Factors associated with rapid mortality of sugar maple in Pennsylvania

Treesearch

Thomas J. Hall; James D. Unger; Thomas C. Bast; Bradley S. Regester

1999-01-01

Mortality of sugar maple and red maple was observed throughout Pennsylvania in 1995 following an outbreak in 1994 by forest tent caterpillar and elm spanworm on sugar maple and red maple, respectively. Symptoms of leaf anthracnose caused by Discula campestris (Pass.) were observed during the refoliation period from July through September 1994: the...

Correlation of maple sap composition with bacterial and fungal communities determined by multiplex automated ribosomal intergenic spacer analysis (MARISA).

PubMed

Filteau, Marie; Lagacé, Luc; LaPointe, Gisèle; Roy, Denis

2011-08-01

During collection, maple sap is contaminated by bacteria and fungi that subsequently colonize the tubing system. The bacterial microbiota has been more characterized than the fungal microbiota, but the impact of both components on maple sap quality remains unclear. This study focused on identifying bacterial and fungal members of maple sap and correlating microbiota composition with maple sap properties. A multiplex automated ribosomal intergenic spacer analysis (MARISA) method was developed to presumptively identify bacterial and fungal members of maple sap samples collected from 19 production sites during the tapping period. Results indicate that the fungal community of maple sap is mainly composed of yeast related to Mrakia sp., Mrakiella sp., Guehomyces pullulans, Cryptococcus victoriae and Williopsis saturnus. Mrakia, Mrakiella and Guehomyces peaks were identified in samples of all production sites and can be considered dominant and stable members of the fungal microbiota of maple sap. A multivariate analysis based on MARISA profiles and maple sap chemical composition data showed correlations between Candida sake, Janthinobacterium lividum, Williopsis sp., Leuconostoc mesenteroides, Mrakia sp., Rhodococcus sp., Pseudomonas tolaasii, G. pullulans and maple sap composition at different flow periods. This study provides new insights on the relationship between microbial community and maple sap quality. Copyright © 2011 Elsevier Ltd. All rights reserved.

Height diameter relations of maple street trees

Treesearch

David J. Nowak

1990-01-01

Height and diameter measurements were taken for silver, sugar and Norway maple street trees in Rochester and Syracuse, New York. Mature silver maples proved to be the tallest of the three species. Average sugar maple height was consistently taller than Norway maple height until diameters reached 28 inches. Average mature tree height for all three species level off in...

Impact of added nutrients in sweet sorghum syrup fermentation to produce ethanol

USDA-ARS?s Scientific Manuscript database

This work demonstrated that sweet sorghum syrup was efficiently converted to ethanol by yeast. Fermentation broth with sweet sorghum syrup performed better (at least faster) than with only pure sugars due to the pH-buffering effect of sweet sorghum syrup solutions. Sugar solutions containing up to 2...

21 CFR 184.1866 - High fructose corn syrup.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 21 Food and Drugs 3 2014-04-01 2014-04-01 false High fructose corn syrup. 184.1866 Section 184... as GRAS § 184.1866 High fructose corn syrup. (a) High fructose corn syrup, a sweet, nutritive... to the identity and specifications listed in the monograph entitled “High-Fructose Corn Syrup” in the...

Comparison of productivity of colonies of honey bees, Apis mellifera, supplemented with sucrose or high fructose corn syrup

USDA-ARS?s Scientific Manuscript database

Honey bee colony feeding trials were conducted to determine whether differential effects of carbohydrate feeding (sucrose syrup vs. high fructose corn syrups) were detected between colonies fed exclusively on these syrups. In one experiment, colonies installed within a closed arena had increased pr...

A Review of the Ingredients Contained in Over the Counter (OTC) Cough Syrup Formulations in Kenya. Are They Harmful to Infants?

PubMed Central

Kigen, Gabriel

2015-01-01

Background Cough syrups are widely used in the developing world, but safety of their use in infants and children less than two years has not been well documented. Some syrups contain multiple combinations of such drugs as promethazine, diphenhydramine and ephedrine; which are individually now contraindicated in children less than two years. Despite this, the syrups are available as over the counter drugs and may be dispensed to mothers who are unaware of the potentially hazardous effects to their infants. A descriptive cross-sectional study was used to investigate suitability of cough syrups sold within Eldoret municipality for use in children less than two years of age based on their formulations and available literature. Methods Two semi-structured questionnaires were administered to pharmacy attendants and mothers attending sick child clinic at a referral hospital to establish whether cough syrups containing more than one active ingredient of compounds, now contraindicated in children are administered to infants, and awareness of potential serious adverse effects. Data from labeled contents of cough syrups from retail pharmacies was recorded and corroborated with information from literature to determine those deemed to contain the ingredients. The second questionnaire was administered to mothers with children less than two years to ascertain whether they had used the identified syrups. A total of 260 mothers and 55 pharmacy attendants were interviewed. Results There was widespread use of the syrups in children, including infants, with 192 (74%) of the respondents having used identified syrups and over 90% of these on children less than 2 years including those less than three months.146 (76%) mothers had administered the syrup at double the recommended dose. Conclusion The regulatory authorities should make concerted efforts to discourage use of cough syrups containing ingredients that pose adverse events to infants, including campaigns to educate pharmacy workers and mothers. PMID:26540251

A Review of the Ingredients Contained in Over the Counter (OTC) Cough Syrup Formulations in Kenya. Are They Harmful to Infants?

PubMed

Kigen, Gabriel; Busakhala, Naftali; Ogaro, Francis; Chesire, Emily; Saat, Nathan; Too, Robert; Nyandiko, Winstone

2015-01-01

Cough syrups are widely used in the developing world, but safety of their use in infants and children less than two years has not been well documented. Some syrups contain multiple combinations of such drugs as promethazine, diphenhydramine and ephedrine; which are individually now contraindicated in children less than two years. Despite this, the syrups are available as over the counter drugs and may be dispensed to mothers who are unaware of the potentially hazardous effects to their infants. A descriptive cross-sectional study was used to investigate suitability of cough syrups sold within Eldoret municipality for use in children less than two years of age based on their formulations and available literature. Two semi-structured questionnaires were administered to pharmacy attendants and mothers attending sick child clinic at a referral hospital to establish whether cough syrups containing more than one active ingredient of compounds, now contraindicated in children are administered to infants, and awareness of potential serious adverse effects. Data from labeled contents of cough syrups from retail pharmacies was recorded and corroborated with information from literature to determine those deemed to contain the ingredients. The second questionnaire was administered to mothers with children less than two years to ascertain whether they had used the identified syrups. A total of 260 mothers and 55 pharmacy attendants were interviewed. There was widespread use of the syrups in children, including infants, with 192 (74%) of the respondents having used identified syrups and over 90% of these on children less than 2 years including those less than three months.146 (76%) mothers had administered the syrup at double the recommended dose. The regulatory authorities should make concerted efforts to discourage use of cough syrups containing ingredients that pose adverse events to infants, including campaigns to educate pharmacy workers and mothers.

Maple sirup production from bigleaf maple.

Treesearch

Robert H. Ruth; J. Clyde Underwood; Clark E. Smith; Hoya Y. Yang

1972-01-01

Bigleaf maple sap flow during the 1970-71 season ranged from none to 16.9 gallons per taphole and sugar content of the sap from 1.0 to 2.6 percent. Sugar content also varied seasonally, with the sweetest sap flowing in late January. The sirup was very flavorful, although not as strong in typical maple flavor as that made from eastern sugar maple. Sirup production...

Abnormal white matter integrity in chronic users of codeine-containing cough syrups: a tract-based spatial statistics study.

PubMed

Qiu, Y-W; Su, H-H; Lv, X-F; Jiang, G-H

2015-01-01

Codeine-containing cough syrups have become one of the most popular drugs of abuse in young people in the world. Chronic codeine-containing cough syrup abuse is related to impairments in a broad range of cognitive functions. However, the potential brain white matter impairment caused by chronic codeine-containing cough syrup abuse has not been reported previously. Our aim was to investigate abnormalities in the microstructure of brain white matter in chronic users of codeine-containing syrups and to determine whether these WM abnormalities are related to the duration of the use these syrups and clinical impulsivity. Thirty chronic codeine-containing syrup users and 30 matched controls were evaluated. Diffusion tensor imaging was performed by using a single-shot spin-echo-planar sequence. Whole-brain voxelwise analysis of fractional anisotropy was performed by using tract-based spatial statistics to localize abnormal WM regions. The Barratt Impulsiveness Scale 11 was surveyed to assess participants' impulsivity. Volume-of-interest analysis was used to detect changes of diffusivity indices in regions with fractional anisotropy abnormalities. Abnormal fractional anisotropy was extracted and correlated with clinical impulsivity and the duration of codeine-containing syrup use. Chronic codeine-containing syrup users had significantly lower fractional anisotropy in the inferior fronto-occipital fasciculus of the bilateral temporo-occipital regions, right frontal region, and the right corona radiata WM than controls. There were significant negative correlations among fractional anisotropy values of the right frontal region of the inferior fronto-occipital fasciculus and the right superior corona radiata WM and Barratt Impulsiveness Scale total scores, and between the right frontal region of the inferior fronto-occipital fasciculus and nonplan impulsivity scores in chronic codeine-containing syrup users. There was also a significant negative correlation between fractional anisotropy values of the right frontal region of the inferior fronto-occipital fasciculus and the duration of codeine-containing syrup use in chronic users. Chronic codeine-containing syrup abuse may be associated with disruptions in brain WM integrity. These WM microstructural deficits may be linked to higher impulsivity in chronic codeine-containing syrup users. © 2015 by American Journal of Neuroradiology.

76 FR 77480 - Honey From the People's Republic of China: Initiation of Anticircumvention Inquiry

Federal Register 2010, 2011, 2012, 2013, 2014

2011-12-13

... high fructose corn syrup were known to be mixed with honey, making them ``honey adulterants,'' and that... syrup blends in its discussion of artificial honey, while it did list refined sugar and high-fructose corn syrup, as evidence that honey-rice syrup blends were not contemplated at the time of the Order...

Acid-base status of upper rooting zone soil in declining and non-declining sugar maple (Acer saccharum Marsh) stands in Pennsylvania

Treesearch

William E. Sharpe; Troy L. Sunderland

1995-01-01

Sugar maple (Acer saccharum Marsh) is an important commercial tree species of the central hardwood region which is valued for its wood and maple sugar products. High elevation sugar maple stands in northcentral Pennsylvania have been in serious decline for about the last 15 years with more than 1,200 hectares of maple forest affected. The decline...

High Titer and Yields Achieved with Novel, Low-Severity Pretreatment Strategy

DOE Office of Scientific and Technical Information (OSTI.GOV)

NREL researchers obtained high concentration sugar syrups in enzymatic hydrolysis that are fermentable to ethanol and other advanced biofuels and intermediate products at high yields. The novel DMR process is simpler and bypasses all severe pretreatment methods, thus reducing the environmental impact. The results are unprecedented. Researchers achieved a high concentration of sugars (230g/L of monomeric sugar and 270 g/L total sugar) and this low toxicity, highly fermentable syrup yielded 86 g/L ethanol (> 90 percent conversion). In addition, the lignin streams from this process can readily be converted to jet or renewable diesel blendstocks through a hydrodeoxygenation step. Themore » NREL-developed, low severity DMR process may potentially replace higher severity chemical pretreatments and associated expensive reactors constructed of exotic alloys with a simpler process, using commercial-scale equipment commonly associated with the pulp and paper industry, to produce high concentration, low toxicity sugar streams and highly reactive lignin streams from non-food renewable biomass for biological and catalytic upgrading to advanced biofuels and chemicals. The simpler DMR process with black liquor recycling could reduce environmental and life-cycle impacts, and repurpose shuttered pulp and paper mills to help revitalize rural economies.« less

Norway maple displays greater seasonal growth and phenotypic plasticity to light than native sugar maple.

PubMed

Paquette, Alain; Fontaine, Bastien; Berninger, Frank; Dubois, Karine; Lechowicz, Martin J; Messier, Christian; Posada, Juan M; Valladares, Fernando; Brisson, Jacques

2012-11-01

Norway maple (Acer platanoides L), which is among the most invasive tree species in forests of eastern North America, is associated with reduced regeneration of the related native species, sugar maple (Acer saccharum Marsh) and other native flora. To identify traits conferring an advantage to Norway maple, we grew both species through an entire growing season under simulated light regimes mimicking a closed forest understorey vs. a canopy disturbance (gap). Dynamic shade-houses providing a succession of high-intensity direct-light events between longer periods of low, diffuse light were used to simulate the light regimes. We assessed seedling height growth three times in the season, as well as stem diameter, maximum photosynthetic capacity, biomass allocation above- and below-ground, seasonal phenology and phenotypic plasticity. Given the north European provenance of Norway maple, we also investigated the possibility that its growth in North America might be increased by delayed fall senescence. We found that Norway maple had significantly greater photosynthetic capacity in both light regimes and grew larger in stem diameter than sugar maple. The differences in below- and above-ground biomass, stem diameter, height and maximum photosynthesis were especially important in the simulated gap where Norway maple continued extension growth during the late fall. In the gap regime sugar maple had a significantly higher root : shoot ratio that could confer an advantage in the deepest shade of closed understorey and under water stress or browsing pressure. Norway maple is especially invasive following canopy disturbance where the opposite (low root : shoot ratio) could confer a competitive advantage. Considering the effects of global change in extending the potential growing season, we anticipate that the invasiveness of Norway maple will increase in the future.

[Adhesion loss of syrups in a metering glass which consists of a low surface free energy material].

PubMed

Yamamoto, Yoshihisa; Suzuki, Toyofumi; Hashizaki, Kaname; Ogura, Masao; Umeda, Yukiko; Hidaka, Shinji; Fukami, Toshiro; Tomono, Kazuo

2010-08-01

We previously reported a strong positive correlation between syrup viscosity and the rate of syrup loss due to adhesion to a glass metering device. In this study, we examined differences in the surface free energies of metering devices made of different polymeric materials, since reducing adhesion loss to metering devices could improve the efficiency of drug preparation involving highly viscous syrups. Among metering devices made of glass only, glass with a silicone coating (SLC), polypropylene (PP), and polymethylpentene (PMP) the surface free energy of the glass-only metering device was the highest (49.2 mN/m). The adhesion loss obtained for highly viscous syrups in the PP and PMP metering devices was significantly lower than that of the glass metering device. Measurements of syrup contact angles suggested that in metering devices made of PP and PMP, which have low surface free energies, a decrease in the spreading wetting of syrups was a factor in reducing the rate of adhesion loss. Thus irrespective of the syrup viscosity being measured, metering devices produced from materials with low surface free energies can reduce the time required to prepare prescriptions without compromising the accuracy of drug preparation.

Chapter 1:Red and silver maples : resource availability, utilization, and costs

Treesearch

Henry Spelter

2005-01-01

Red maple (Acer rubrum) is a medium-sized tree 15 to 20 meters (50 to 65 feet) high and 30 to 60 centimeters (12 to 24 inches) in diameter with a generally fairly clear trunk. This species and silver maple (Acer saccharinum) are often called “soft maples” to distinguish them from the higher density sugar and black maples that are commonly referred to...

Detection of honey adulteration with starch syrup by high performance liquid chromatography.

PubMed

Wang, Shaoqing; Guo, Qilei; Wang, Linlin; Lin, Li; Shi, Hailiang; Cao, Hong; Cao, Baosen

2015-04-01

According to saccharide profile comparison between starch syrups and pure honeys analysed through high performance liquid chromatography (HPLC), a characteristic peak was found at 15.25 min retention time in HPLC chromatogram of syrup, but no peak was observed at the same retention time in chromatogram of pure honeys. This characteristic peak for syrup was identified as an overlapping peak of oligosaccharides with more than 5 degree of polymerisation (DP) based on HPLC chromatogram comparison between starch syrup and a series of standard mono-, di- and oligosaccharides of 3-7 DP. Additionally syrup content correlated linearly with the height of the characteristic peak of syrup under different slope in two ranges 2.5-7.5% and 10-100%, respectively. Therefore, the characteristic peak at 15.25 min retention time can serve as a syrup indicator in HPLC analysis of the adulterated honeys. This new HPLC method for honey adulteration detection was further applied in an authenticity inspection on more than 100 commercial honeys. In addition to the improved accuracy of honey adulteration detection, the proposed HPLC method was simple, low cost and easy practice for honey product quality control by government department considering the popularity of HPLC device and technology. Copyright © 2014 Elsevier Ltd. All rights reserved.

Detection of adulteration in honey samples added various sugar syrups with 13C/12C isotope ratio analysis method.

PubMed

Tosun, Murat

2013-06-01

Honey can be adulterated in various ways. One of the adulteration methods is the addition of different sugar syrups during or after honey production. Starch-based sugar syrups, high fructose corn syrup (HFCS), glucose syrup (GS) and saccharose syrups (SS), which are produced from beet or canes, can be used for adulterating honey. In this study, adulterated honey samples were prepared with the addition of HFCS, GS and SS (beet sugar) at a ratio of 0%, 10%, 20%, 40% and 50% by weight. (13)C/(12)C analysis was conducted on these adulterated honey samples using an isotope ratio mass spectrometer in combination with an elemental analyser (EA-IRMS). As a result, adulteration using C(4) sugar syrups (HFCS and GS) could be detected to a certain extent while adulteration of honey using C(3) sugar syrups (beet sugar) could not be detected. Adulteration by using SS (beet sugar) still has a serious detection problem, especially in countries in which beet is used in manufacturing sugar. For this reason, practice and analysis methods are needed to meet this deficit and to detect the adulterations precisely in the studies that will be conducted. Copyright © 2012 Elsevier Ltd. All rights reserved.

A requirement for sucrose in xylem sap flow from dormant maple trees.

PubMed

Johnson, R W; Tyree, M T; Dixon, M A

1987-06-01

The response of excised stem segments of several tree species to freezing and thawing cycles was studied. All species studied (Thuja occidentalis, Fagus grandifolia, and Betula papyrifera) except maple (Acer spp.) exuded sap while freezing and absorbed on thawing. Maple stems absorbed sap while freezing and exuded sap during the thaw only when sucrose was present in the vessel solution. Increased concentration of sucrose in the vessel sap led to increased exudation. In the absence of sucrose, maple stems absorbed sap on thawing. The presence of sucrose enhanced sap absorption during freezing cycles in maples. In general, large sugars, disaccharides and larger, could substitute for sucrose in the maple exudation response while sugar hexoses could not. The results are discussed in relation to the O'Malley-Milburn model (1983 Can J Bot 61: 3100-3106) of sap flow in maples.

A Requirement for Sucrose in Xylem Sap Flow from Dormant Maple Trees 1

PubMed Central

Johnson, Robert W.; Tyree, Melvin T.; Dixon, Michael A.

1987-01-01

The response of excised stem segments of several tree species to freezing and thawing cycles was studied. All species studied (Thuja occidentalis, Fagus grandifolia, and Betula papyrifera) except maple (Acer spp.) exuded sap while freezing and absorbed on thawing. Maple stems absorbed sap while freezing and exuded sap during the thaw only when sucrose was present in the vessel solution. Increased concentration of sucrose in the vessel sap led to increased exudation. In the absence of sucrose, maple stems absorbed sap on thawing. The presence of sucrose enhanced sap absorption during freezing cycles in maples. In general, large sugars, disaccharides and larger, could substitute for sucrose in the maple exudation response while sugar hexoses could not. The results are discussed in relation to the O'Malley-Milburn model (1983 Can J Bot 61: 3100-3106) of sap flow in maples. Images Fig. 2 PMID:16665468

Bite of Arsenic, with Kathryn Cottingham. Interviewed by Ashley Ahearn.

PubMed

Cottingham, Kathryn

2012-05-01

Many organic foods and high-energy products are sweetened with brown rice syrup as an alternative to high-fructose corn syrup. Consumers who eat these products may be avoiding high-fructose corn syrup, but they also may be exposed to arsenic that's been absorbed by the rice plants from which the syrup is made. In this podcast, Kathryn Cottingham talks with host Ashley Ahearn about her recent market-basket study of products containing brown rice syrup and other rice-based ingredients. Arsenic was detected in all the products tested, although Cottingham cautions it's too soon to say what this means in terms of potential health effects.

Drought tolerance of sugar maple ecotypes

Treesearch

Richard J. Hauer; Jeffery O. Dawson

1995-01-01

Sugar maple declines periodically occur in rural and urban areas. These declines usually follow periods of below-average precipitation leading to the speculation that moisture deficiency is a primary cause of the decline. Sugar maple ecotypes with greater tolerance to drought should have greater longevity and vitality as a result of this tolerance. Sugar maple and...

Effects of Nitrogen Fertilization on Potato Leafhopper (Hemiptera: Cicadellidae) and Maple Spider Mite (Acari: Tetranychidae) on Nursery-Grown Maples.

PubMed

Prado, Julia; Quesada, Carlos; Gosney, Michael; Mickelbart, Michael V; Sadof, Clifford

2015-06-01

Although leaf nitrogen (N) has been shown to increase the suitability of hosts to herbivorous arthropods, the responses of these pests to N fertilization on susceptible and resistant host plants are not well characterized. This study determined how different rates of N fertilization affected injury caused by the potato leafhopper (Empoasca fabae Harris) and the abundance of maple spider mite (Oligonychus aceris (Shimer)) on 'Red Sunset' red maple (Acer rubrum) and 'Autumn Blaze' Freeman maple (Acer×freemanii) during two years in Indiana. N fertilization increased leaf N concentration in both maple cultivars, albeit to a lesser extent during the second year of the study. Overall, Red Sunset maples were more susceptible to E. fabae injury than Autumn Blaze, whereas Autumn Blaze maples supported higher populations of O. aceris. Differences in populations of O. aceris were attributed to differences between communities of stigmaeid and phytoseiid mites on each cultivar. Injury caused by E. fabae increased with N fertilization in a dose-dependent manner in both cultivars. Although N fertilization increased the abundance of O. aceris on both maple cultivars, there was no difference between the 20 and 40 g rates. We suggest the capacity of N fertilization to increase O. aceris on maples could be limited at higher trophic levels by the community of predatory mites. © The Authors 2015. Published by Oxford University Press on behalf of Entomological Society of America. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Red maple (Acer rubrum) leaf toxicosis in horses: a retrospective study of 32 cases.

PubMed

Alward, Ashley; Corriher, Candice A; Barton, Michelle H; Sellon, Debra C; Blikslager, Anthony T; Jones, Samuel L

2006-01-01

Ingestion of wilted red maple leaves by horses can result in severe hemolytic anemia and methemoglobinemia. Little is known about what factors influence the outcome of red maple leaf toxicosis in horses. Our hypothesis was that physical examination findings, clinicopathologic variables or therapeutic modalities may predict outcome in horses with red maple leaf toxicity. Horses with red maple leaf toxicosis presented to referral hospitals in the southeast region of the United States. A multi-institutional retrospective study was designed to identify factors that predict mortality in horses with red maple toxicosis. Thirty-two horses with red maple toxicosis were identified, 19 of which died. Twenty-nine horses presented with anemia and 24 had clinicopathologic evidence of systemic inflammation. Renal insufficiency was identified in 12/30 (41%) horses. Laminitis (9/28) and colic (13/30) also were identified in horses with red maple toxicosis, but development of these 2 conditions did not have a negative effect on short-term survival. Horses with red maple toxicosis that survived to discharge were likely to have developed pyrexia during hospitalization (P = .030). Horses that were treated with a corticosteroid had a significantly increased likelihood of death (P = .045). There was no significant relationship between initial serum hemoglobin concentration, methemoglobin concentration, or percentage methemoglobin and mortality in this horse series. This study suggests that information obtained on initial examination cannot be used to accurately predict survival in horses with red maple toxicosis, but horses that receive corticosteroids are unlikely to survive.

Rapid determination of amino acids in neonatal blood samples based on derivatization with isobutyl chloroformate followed by solid-phase microextraction and gas chromatography/mass spectrometry.

PubMed

Deng, Chunhui; Li, Ning; Zhang, Xiangmin

2004-01-01

The purpose of this study was to develop a simple, rapid and sensitive analytical method for determination of amino acids in neonatal blood samples. The developed method involves the employment of derivatization and a solid-phase microextraction (SPME) technique together with gas chromatography/mass spectrometry (GC/MS). Amino acids in blood samples were derivatized by a mixture of isobutyl chloroformate, methanol and pyridine, and the N(O,S)-alkoxycarbonyl alkyl esters thus formed were headspace extracted by a SPME fiber. Finally, the extracted analytes on the fiber were desorbed and detected by GC/MS in electron impact (EI) mode. L-Valine, L-leucine, L-isoleucine, L-phenylanaline and L-tyrosine in blood samples were quantitatively analyzed by measurement of the corresponding N(O,S)-alkoxycarbonyl alkyl esters using an external standard method. SPME conditions were optimized, and the method was validated. The method was applied to diagnosis of neonatal phenylkenuria (PKU) and maple syrup urine disease (MSUD) by the analyses of five amino acids in blood samples. The results showed that the proposed method is a potentially powerful tool for simultaneous screening for neonatal PKU and MSUD. Copyright (c) 2004 John Wiley & Sons, Ltd.

Heterozygote to homozygote related living donor liver transplantation in maple syrup urine disease: a case report.

PubMed

Patel, N; Loveland, J; Zuckerman, M; Moshesh, P; Britz, R; Botha, J

2015-05-01

Liver transplantation is an accepted treatment modality in the management of MSUD. To our knowledge, ours is only the second successful case to date of a patient with MSUD receiving an allograft from an RLD who is a heterozygous carrier for the disease. In view of the worldwide shortage of available organs for transplantation, heterozygote to homozygote transplantation in the setting of MSUD may provide a viable alternative for those awaiting transplantation. We report on the case of a two-yr-old infant with MSUD, who received a left lateral segment (segments II and III) liver transplant from his mother, a heterozygote carrier of one of the three abnormal genes implicated in MSUD. Post-operative BCAA levels normalized in our patient and remained so on an unrestricted protein diet and during times of physiological stress. To date, this is only the second case of a successful RLD liver transplant in a child with MSUD. Preliminary results indicate that RLD liver transplants are at least equivalent to deceased donor liver transplants in the treatment of MSUD, although longer term follow-up is required. Heterozygote to homozygote RLD transplant in patients with MSUD presents a new pool of potential liver donors. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Development of carrier testing for common inborn errors of metabolism in the Wisconsin Plain population.

PubMed

Kuhl, Ashley; van Calcar, Sandra; Baker, Mei; Seroogy, Christine M; Rice, Gregory; Scott Schwoerer, Jessica

2017-03-01

This community project is an initiative through the University of Wisconsin Biochemical Genetics Clinic and the Wisconsin Newborn Screening Program to identify members of the Plain population who are at risk for having children with maple syrup urine disease (MSUD) or propionic acidemia (PA) or who have PA. Because of the high prevalence of metabolic conditions in the Plain population and the importance of early intervention, a statewide outreach project was developed to provide targeted variant analysis of the common MSUD and PA pathogenic variants in this population through health-care provider distribution of blood spot testing kits. Awareness was achieved through outreach efforts with the state midwives guild and Plain population meetings. Eighty individuals were tested; diagnosis was confirmed for three adults with PA and one couple was identified as being at risk for having a child with PA. Genetic counseling was provided to those identified. Follow-up diagnostic testing was completed for the at-risk couple's children; none were found to be affected. This initiative successfully provided accessible clinical testing for MSUD and PA for a high-risk population. Early identification of at-risk couples sets the foundation for early care of at-risk neonates, thereby improving future clinical outcomes.Genet Med 19 3, 352-356.

Tolerability, pharmacokinetics, and bioequivalence of the tablet and syrup formulations of lacosamide in plasma, saliva, and urine: saliva as a surrogate of pharmacokinetics in the central compartment.

PubMed

Cawello, Willi; Bökens, Hilmar; Nickel, Brunhild; Andreas, Jens-Otto; Halabi, Atef

2013-01-01

To test for bioequivalence of 200 mg lacosamide oral tablet and syrup formulations. Additional objectives were to compare the pharmacokinetic profile of lacosamide in saliva and plasma, and to evaluate its tolerability. This open-label, randomized, two-way crossover trial was conducted in 16 healthy Caucasian male participants in Germany. The bioequivalence of 200 mg lacosamide tablet and syrup was evaluated using plasma to determine maximum measured concentration (C(max)) and area under the curve from zero to the last time point (AUC)(0-tz). Plasma and saliva samples for evaluation of pharmacokinetic parameters of lacosamide and the major metabolite O-desmethyl lacosamide (SPM 12809) were taken over 15 time points (0.5-72 h) and used to statistically compare bioavailability of the two. Urine samples were collected predose and over five time points (0-48 h) to evaluate the cumulative amount of unchanged drug and metabolite. Lacosamide median time to reach C(max) (t(max)) was 1 h for tablet and 0.5 h for syrup in plasma and saliva. Mean terminal half life (t(½)) for tablet and syrup was 12.5 and 12.4 h in plasma, and 13.1 and 13.3 h in saliva, respectively. Tablet and syrup mean plasma AUC(0-tz) was 84.5 and 83.3 μg/mL*h, respectively. Mean AUC(0-tz) in saliva was 93.2 μg/mL*h for tablet and syrup. Mean C(max) for tablet was 5.26 μg/mL in plasma and 5.63 μg/mL in saliva. Syrup mean C(max) was 5.14 and 8.32 μg/mL in plasma and saliva, respectively. Within 2 h of syrup administration, elevated lacosamide concentration in saliva compared to plasma was observed. The ratio of lacosamide syrup to tablet was 0.98 for C(max) and 0.99 for AUC(0-tz) in plasma, and 1.00 for AUC((0-tz)) in saliva; the 90% confidence intervals (CIs) for these parameters were within the range of 0.80-1.25, which meets accepted bioequivalence criteria. The syrup-to-tablet ratio for C(max) in saliva was 1.48, and the 90% CIs exceeded the accepted upper boundary for bioequivalence (1.32-1.66). Both formulations were well tolerated. Metabolite concentration versus time profiles for saliva were similar to plasma following tablet and syrup administration. The tablet and syrup formulations of lacosamide 200 mg were bioequivalent and well tolerated. Saliva samples were demonstrated to be a suitable surrogate to evaluate lacosamide tablet pharmacokinetics in the central compartment. Due to residual syrup in the buccal cavity, limitations exist when using saliva to evaluate the pharmacokinetics of lacosamide syrup <2 h after administration. Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.

High-Fructose Corn Syrup: What Are the Concerns?

MedlinePlus

Healthy Lifestyle Nutrition and healthy eating What is high-fructose corn syrup? What are the health concerns? Answers from Katherine Zeratsky, R.D., L.D. High-fructose corn syrup is a common sweetener in sodas and fruit- ...

BOILING HOUSE, INTERIOR, SECOND FLOOR, SYRUP TANKS IN RIGHT FOREGROUND, ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

BOILING HOUSE, INTERIOR, SECOND FLOOR, SYRUP TANKS IN RIGHT FOREGROUND, HIGH GRADE VACUUM PANS BEYOND THE SYRUP TANKS. VIEW FROM THE SOUTH - Kekaha Sugar Company, Sugar Mill Building, 8315 Kekaha Road, Kekaha, Kauai County, HI

Mercury from chlor-alkali plants: measured concentrations in food product sugar.

PubMed

Dufault, Renee; LeBlanc, Blaise; Schnoll, Roseanne; Cornett, Charles; Schweitzer, Laura; Wallinga, David; Hightower, Jane; Patrick, Lyn; Lukiw, Walter J

2009-01-26

Mercury cell chlor-alkali products are used to produce thousands of other products including food ingredients such as citric acid, sodium benzoate, and high fructose corn syrup. High fructose corn syrup is used in food products to enhance shelf life. A pilot study was conducted to determine if high fructose corn syrup contains mercury, a toxic metal historically used as an anti-microbial. High fructose corn syrup samples were collected from three different manufacturers and analyzed for total mercury. The samples were found to contain levels of mercury ranging from below a detection limit of 0.005 to 0.570 micrograms mercury per gram of high fructose corn syrup. Average daily consumption of high fructose corn syrup is about 50 grams per person in the United States. With respect to total mercury exposure, it may be necessary to account for this source of mercury in the diet of children and sensitive populations.

Determination of D-pinitol in carob syrup.

PubMed

Tetik, Nedim; Turhan, Irfan; Oziyci, Hatice R; Karhan, Mustafa

2011-09-01

Carob syrup is a traditional product native to the Mediterranean region, containing a high concentration of sugar, phenolic compounds and minerals. d-pinitol is a bioactive component extracted from legumes and has some beneficial effects on human metabolism. In this research, the d-pinitol content and sugar profile of 10 different carob syrup samples purchased from Turkish markets were determined. Mean d-pinitol, sucrose, glucose and fructose contents of samples were found to be 84.63 ± 10.73, 385.90 ± 45.07, 152.44 ± 21.72 and 162.03 ± 21.45 g/kg dry weight, respectively. Carob syrup has a considerable amount of d-pinitol compared with the other d-pinitol-including legumes. Consequently, this study showed that carob syrup may be a suitable source of d-pinitol for medical use and d-pinitol may be an indicator for the detection of any adulteration in carob syrup.

Effect of Sugar Maple Root Exudate on Seedlings of Northern Conifer Species

Treesearch

Carl H. Tubbs

1976-01-01

It has been shows that a root exudate of sugar maple reduces the growth of yellow birch. A laboratory test indicated that the growth of northern conifers is also reduced in sugar maple root exudate. Allelopathy may play an important role in survival of species on sites where sugar maple is abundant.

Health of eastern North American sugar maple forests and factors affecting decline

Treesearch

Stephen B. Horsley; Robert P. Long; Scott W. Bailey; Richard A. Hallett; Philip M. Wargo

2002-01-01

Sugar maple (Acer saccharum) is a keystone species in the forests of the northeastern and Midwestern United States and eastern Canada. Its sustained health is an important issue in both managed and unmanaged forests. While sugar maple generally is healthy throughout its range, decline disease of sugar maple has occurred sporadically during the past...

Calculus of One and More Variables with Maple

ERIC Educational Resources Information Center

Samkova, Libuse

2012-01-01

This is a guide to using Maple in teaching fundamental calculus of one, two and three variables (limits, derivatives, integrals, etc.), also suitable for Maple beginners. It outlines one of the ways to effective use of computers in the teaching process. It scans advantages and disadvantages of using Maple in relation to students and teacher. The…

Using Maple to Implement eLearning Integrated with Computer Aided Assessment

ERIC Educational Resources Information Center

Blyth, Bill; Labovic, Aleksandra

2009-01-01

Advanced mathematics courses have been developed and refined by the first author, using an action research methodology, for more than a decade. These courses use the computer algebra system (CAS) Maple in an "immersion mode" where all presentations and student work are done using Maple. Assignments and examinations are Maple files downloaded from…

Chapter 2:Basic properties of undervalued hardwoods

Treesearch

John I. Zerbe

2005-01-01

Among the most abundant of our undervalued hardwoods are the soft maples. However, other species that are also underutilized include some species of birch and some lower grades of the hard maples. This chapter covers physical, mechanical, and other important properties of different soft maples, hard maples, and yellow birch and compares them with the properties of...

Methyl gallate is a natural constituent of maple (Genus Acer) leaves.

PubMed

Abou-Zaid, Mamdouh M; Lombardo, Domenic A; Nozzolillo, Constance

2009-01-01

Methyl gallate was found in ethanolic extracts of red maple (Acer rubrum L.), silver maple (A. saccharinum L.) and sugar maple (A. saccharum Marsh) leaves, but more was present in methanolic extracts. The increased amount of methyl gallate in methanolic extracts was accompanied by a disappearance of m-digallate. It is concluded that only some of the methyl gallate detected in methanolic extracts is an artefact as a result of methanolysis of m-digallate. Its presence in ethanolic extracts is evidence that it is also a natural constituent of maple leaves.

Frequently Asked Questions about Sugar

MedlinePlus

... in “ose” (dextrose, fructose, glucose, lactose, maltose, sucrose), high-fructose corn syrup, fruit juice concentrate, honey, invert sugar, malt sugar, ... caloric sweeteners that are chemically manufactured (such as high fructose corn syrup). Some names for added sugars include agave syrup, ...

Laboratory experiments on fragmentation of highly-viscous bubbly syrup

NASA Astrophysics Data System (ADS)

Kurihara, H.; Kameda, M.; Ichihara, M.

2006-12-01

Fragmentation of vesicular magma by rapid decompression is a key process in explosive eruptions. To determine the fragmentation criteria, we carried out laboratory experiments on magma fragmentation using analogous materials. We used commercial syrup as an analogous material of magma, because the viscosity was widely altered by adding or subtracting water contents in the syrup. We made the bubbly syrup by adding hydrogen peroxide with manganese oxide in the syrup. The amount of hydrogen peroxide is proportional to the gas volume fraction in the syrup. We measured the rheological properties of the syrup. Zero shear viscosity η was measured by a rotating viscometer and a fiber elongation technique. Glass transition temperature was measured by differential scanning calorimetry. The measured data indicated that the temperature dependence of viscosity was described well using Williams-Landel-Ferry (WLF) equation. The solid content of syrup alters the viscosity as well as the glass transition temperature, though it may hardly affect the rigidity μ, which was measured by ultrasonic test in our previous work. We used a pressurized vertical tube with a large vacuum vessel to apply the rapid decompression on the material. An acrylic container, filled with the bubbly syrup, was placed in the bottom of the pressurized tube. By rupturing the diaphragms inserted between the tube and the vacuum vessel, the bubbly syrup is rapidly decompressed due to expansion of the pressurized gas in the tube. A high-speed video camera was used to obtain sequential images of the materials. Pressure transducers were mounted on the sidewall of the tube and the bottom of the container. The initial pressure was varied from 1 MPa to 5 MPa. The gas-volume fraction of the syrup under pressure was fixed as 2 % to 20%. The viscosity varied from 105 Pa·s to 108 Pa·s. We successfully observed three principal behaviors using the present analogous material; brittle fragmentation, partial fracture and ductile expansion without crack initiation. From all the experimental data, in conclusion, the fragmentation is observed when the pressure drop Δ p reaches a critical value within the order of relaxation time of syrup, which is defined as η/μ. Simultaneously, the initial gas volume fraction should be larger than a critical value, which decreases as the initial high-pressure is larger.

Evaluation of various organic fertilizer substrates and hydraulic retention times for enhancing anaerobic degradation of explosives-contaminated groundwater while using constructed wetlands at the Milan Army Ammunition Plant, Milan, Tennessee. Final report

DOE Office of Scientific and Technical Information (OSTI.GOV)

Behrends, L.L.; Almond, R.A.; Kelly, D.A.

1998-05-01

This document describes studies conducted at the Milan Army Ammunition Plant (MAAP) to improve the design, operation, and cost of gravel-based anaerobic cells when phytoremediating explosives-contaminated groundwater. To conduct this study, small-scale anaerobic test cells were used to determine: (1) If the hydraulic retention time of a large demonstration-scale anaerobic cell at MAAP could be reduced, and (2) if other carbon sources could be used as an anaerobic feedstock. The study results indicated that: (1) The existing anaerobic cell`s 7.5-day retention time should not be reduced since residual explosive by-products were present in the effluent of treatments with a 3.5-daymore » retention time. (2) Daily application of a relatively soluble substrate, such as molasses syrup, will provide better explosives removal than periodic application of less soluble substrates like milk replacement starter and sewage sludge. (3) Molasses syrup could be, and should be, used as a substitute for milk replacement power. The recommendation to use molasses syrup was based on: (1) The lower cost of molasses syrup as compared to milk replacement starter, (2) molasses syrup`s higher solubility (which makes it easier to apply), and (3) molasses syrup`s ability to provide enhanced explosives removal.« less

Root-Crown Relations of Young Sugar Maple and Yellow Birch

Treesearch

Carl H. Tubbs

1977-01-01

Young forest-grown sugar maple and yellow birch (1 to 6 inches d.b.h.) crowns were mapped and roots excavated. Crown dimensions were compared. Sugar maple roots usually terminated within a few feet of the crown perimeter. Yellow birch roots frequently terminated well outside crown perimeters and roots of birch were more irregularly distributed than those of maple....

Dynamics of the sugar maple component of a white oak-yellow-poplar community

Treesearch

Richard C. Schlesinger

1989-01-01

The sugar maple (Acer saccharum Marsh) component in a southern Illinois forest increased over a 48-year period. By 1983 sugar maple was three times greater in importance than any other species in the stand. If the observed rate of increase were to continue, sugar maple would exclude all other species within another 50 to 60 years.

An Experimental Study on the aerodynamic and aeroacoustic performances of Maple-Seed-Inspired UAV Propellers

NASA Astrophysics Data System (ADS)

Hu, Hui; Ning, Zhe

2016-11-01

Due to the auto-rotating trait of maple seeds during falling down process, flow characteristics of rotating maple seeds have been studied by many researchers in recent years. In the present study, an experimental investigation was performed to explore maple-seed-inspired UAV propellers for improved aerodynamic and aeroacoustic performances. Inspired by the auto-rotating trait of maple seeds, the shape of a maple seed is leveraged for the planform design of UAV propellers. The aerodynamic and aeroacoustic performances of the maple-seed-inspired propellers are examined in great details, in comparison with a commercially available UAV propeller purchased on the market (i.e., a baseline propeller). During the experiments, in addition to measuring the aerodynamic forces generated by the maple-seed-inspired propellers and the baseline propeller, a high-resolution Particle Image Velocimetry (PIV) system was used to quantify the unsteady flow structures in the wakes of the propellers. The aeroacoustic characteristics of the propellers are also evaluated by leveraging an anechoic chamber available at the Aerospace Engineering Department of Iowa State University. The research work is supported by National Science Foundation under Award Numbers of OSIE-1064235.

Maple

NASA Astrophysics Data System (ADS)

Nicolaides, Roy A.; Walkington, Noel J.

1996-06-01

A knowledge of one or more high level symbolic mathematics programs is rapidly becoming a necessity for mathematics users from all fields of science. The aim of this book is to provide a solid grounding in Maple, one of the best known of these programs. The authors combine efficiency and economy of exposition with a complete coverage of Maple. The book has twelve chapters, of which eight are completely accessible to anyone who has completed calculus and linear sequences as taught in American universities. These chapters cover the great majority of Maple's capabilities. There are also three chapters on Maple programming that can be read without prior programming experience, although knowledge of a high level programming language (Basic, Fortran, C etc.) will help. There is also a chapter on some relevant aspects of algebra. Above all, the book allows the reader to extract value from Maple without wasting time and effort in the learning process. It is the fastest track to expertise for Maple users in mathematics and computer science.

75 FR 62520 - Combined Notice of Filings #1

Federal Register 2010, 2011, 2012, 2013, 2014

2010-10-12

... Thursday, October 7, 2010. Docket Numbers: ER10-2541-001. Applicants: Maple Analytics, LLC. Description: Maple Analytics, LLC submits tariff filing per 35: Maple Analytics, LLC Compliance Filing to be...

A guide to sugarbush stocking. Based on the crown diameter/D.b.h. relationship of open-grown sugar maples

Treesearch

H. Clay Smith; Carter B. Gibbs; Carter B. Gibbs

1970-01-01

Sugarbush managers have long needed a guide for determining the stocking of their sugar maple stands. The question is: for desirable sugar maple sap production, how many trees per acre are needed? To provide information about stocking, the USDA Forest Service's sugar maple sap production project at Burlington, Vermont, has made a regionwide study of the...

Measuring changes in stress and vitality indicators in limed sugar maple on the Allegheny Plateau in north-central Pennsylvania

Treesearch

Philip M. Wargo; Rakesh Minocha; Betty Wong; Robert P. Long; Stephen B. Horsley; Thomas J. Hall

2002-01-01

A study established in 1985 in north-central Pennsylvania to determine effects of lime fertilization on declining sugar maple (Acer saccharum Marsh.) was evaluated in 1993 and showed that liming positively affected growth and crown vitality in sugar maple. This effect of lime on sugar maple offered an opportunity to assess other indicators of tree...

Population dynamics of sugar maple through the southern portion of its range: implications for range migration

Treesearch

Justin L. Hart; Christopher M. Oswalt; Craig M. Turberville

2014-01-01

The range of sugar maple (Acer saccharum Marsh.) is expected to shift northward in accord with changing climate. However, a pattern of increased sugar maple abundance has been reported from sites throughout the eastern US. The goal of our study was to examine the stability of the sugar maple southern range boundary by analyzing its demography through...

Sap sugar parameters of silver maple provenances and clones grown on upland and bottomland sites

Treesearch

J. J. Zaczek; A. D. Carver; K. W. J. Williard; J. K. Buchheit; J. E. Preece; J. C. Mangun

2003-01-01

Sap sugar concentration (SSC), sap volume, and stem diameter were measured for 49 different silver maple clones representing a range-wide collection of 13 provenances within replicated upland and bottomland plantations in southern Illinois during the winter of 2001. For comparison, 42 sugar maple trees were sampled in a local sugarbush. Silver maple SSC averaged 1.51...

Replication of Holograms with Corn Syrup by Rubbing

PubMed Central

Mejias-Brizuela, Nildia Y.; Olivares-Pérez, Arturo; Ortiz-Gutiérrez, Mauricio

2012-01-01

Corn syrup films are used to replicate holograms in order to fabricate micro-structural patterns without the toxins commonly found in photosensitive salts and dyes. We use amplitude and relief masks with lithographic techniques and rubbing techniques in order to transfer holographic information to corn syrup material. Holographic diffraction patterns from holographic gratings and computer Fourier holograms fabricated with corn syrup are shown. We measured the diffraction efficiency parameter in order to characterize the film. The versatility of this material for storage information is promising. Holographic gratings achieved a diffraction efficiency of around 8.4% with an amplitude mask and 36% for a relief mask technique. Preliminary results using corn syrup as an emulsion for replicating holograms are also shown in this work.

Foliar Nutrient Distribution Patterns in Sympatric Maple Species Reflect Contrasting Sensitivity to Excess Manganese.

PubMed

Fernando, Denise R; Marshall, Alan T; Lynch, Jonathan P

2016-01-01

Sugar maple and red maple are closely-related co-occurring tree species significant to the North American forest biome. Plant abiotic stress effects including nutritional imbalance and manganese (Mn) toxicity are well documented within this system, and are implicated in enhanced susceptibility to biotic stresses such as insect attack. Both tree species are known to overaccumulate foliar manganese (Mn) when growing on unbuffered acidified soils, however, sugar maple is Mn-sensitive, while red maple is not. Currently there is no knowledge about the cellular sequestration of Mn and other nutrients in these two species. Here, electron-probe x-ray microanalysis was employed to examine cellular and sub-cellular deposition of excessively accumulated foliar Mn and other mineral nutrients in vivo. For both species, excess foliar Mn was deposited in symplastic cellular compartments. There were striking between-species differences in Mn, magnesium (Mg), sulphur (S) and calcium (Ca) distribution patterns. Unusually, Mn was highly co-localised with Mg in mesophyll cells of red maple only. The known sensitivity of sugar maple to excess Mn is likely linked to Mg deficiency in the leaf mesophyll. There was strong evidence that Mn toxicity in sugar maple is primarily a symplastic process. For each species, leaf-surface damage due to biotic stress including insect herbivory was compared between sites with acidified and non-acidified soils. Although it was greatest overall in red maple, there was no difference in biotic stress damage to red maple leaves between acidified and non-acidified soils. Sugar maple trees on buffered non-acidified soil were less damaged by biotic stress compared to those on unbuffered acidified soil, where they are also affected by Mn toxicity abiotic stress. This study concluded that foliar nutrient distribution in symplastic compartments is a determinant of Mn sensitivity, and that Mn stress hinders plant resistance to biotic stress.

Foliar Nutrient Distribution Patterns in Sympatric Maple Species Reflect Contrasting Sensitivity to Excess Manganese

PubMed Central

Fernando, Denise R.; Marshall, Alan T.; Lynch, Jonathan P.

2016-01-01

Sugar maple and red maple are closely-related co-occurring tree species significant to the North American forest biome. Plant abiotic stress effects including nutritional imbalance and manganese (Mn) toxicity are well documented within this system, and are implicated in enhanced susceptibility to biotic stresses such as insect attack. Both tree species are known to overaccumulate foliar manganese (Mn) when growing on unbuffered acidified soils, however, sugar maple is Mn-sensitive, while red maple is not. Currently there is no knowledge about the cellular sequestration of Mn and other nutrients in these two species. Here, electron-probe x-ray microanalysis was employed to examine cellular and sub-cellular deposition of excessively accumulated foliar Mn and other mineral nutrients in vivo. For both species, excess foliar Mn was deposited in symplastic cellular compartments. There were striking between-species differences in Mn, magnesium (Mg), sulphur (S) and calcium (Ca) distribution patterns. Unusually, Mn was highly co-localised with Mg in mesophyll cells of red maple only. The known sensitivity of sugar maple to excess Mn is likely linked to Mg deficiency in the leaf mesophyll. There was strong evidence that Mn toxicity in sugar maple is primarily a symplastic process. For each species, leaf-surface damage due to biotic stress including insect herbivory was compared between sites with acidified and non-acidified soils. Although it was greatest overall in red maple, there was no difference in biotic stress damage to red maple leaves between acidified and non-acidified soils. Sugar maple trees on buffered non-acidified soil were less damaged by biotic stress compared to those on unbuffered acidified soil, where they are also affected by Mn toxicity abiotic stress. This study concluded that foliar nutrient distribution in symplastic compartments is a determinant of Mn sensitivity, and that Mn stress hinders plant resistance to biotic stress. PMID:27391424

Experimental Testing and Computational Fluid Dynamics Simulation of Maple Seeds and Performance Analysis as a Wind Turbine

NASA Astrophysics Data System (ADS)

Holden, Jacob R.

Descending maple seeds generate lift to slow their fall and remain aloft in a blowing wind; have the wings of these seeds evolved to descend as slowly as possible? A unique energy balance equation, experimental data, and computational fluid dynamics simulations have all been developed to explore this question from a turbomachinery perspective. The computational fluid dynamics in this work is the first to be performed in the relative reference frame. Maple seed performance has been analyzed for the first time based on principles of wind turbine analysis. Application of the Betz Limit and one-dimensional momentum theory allowed for empirical and computational power and thrust coefficients to be computed for maple seeds. It has been determined that the investigated species of maple seeds perform near the Betz limit for power conversion and thrust coefficient. The power coefficient for a maple seed is found to be in the range of 48-54% and the thrust coefficient in the range of 66-84%. From Betz theory, the stream tube area expansion of the maple seed is necessary for power extraction. Further investigation of computational solutions and mechanical analysis find three key reasons for high maple seed performance. First, the area expansion is driven by maple seed lift generation changing the fluid momentum and requiring area to increase. Second, radial flow along the seed surface is promoted by a sustained leading edge vortex that centrifuges low momentum fluid outward. Finally, the area expansion is also driven by the spanwise area variation of the maple seed imparting a radial force on the flow. These mechanisms result in a highly effective device for the purpose of seed dispersal. However, the maple seed also provides insight into fundamental questions about how turbines can most effectively change the momentum of moving fluids in order to extract useful power or dissipate kinetic energy.

Soil base saturation combines with Beech Bark Disease to influence composition and structure of Sugar Maple-Beech forests in an acid rain-impacted region

USGS Publications Warehouse

Lawrence, Gregory B.; McDonnell, Todd C.; Sullivan, Timothy J.; Dovciak, Martin; Bailey, Scott W.; Antidormi, Michael; Zarfos, Michael R.

2018-01-01

Sugar maple, an abundant and highly valued tree species in eastern North America, has experienced decline from soil calcium (Ca) depletion by acidic deposition, while beech, which often coexists with sugar maple, has been afflicted with beech bark disease (BBD) over the same period. To investigate how variations in soil base saturation combine with effects of BBD in influencing stand composition and structure, measurements of soils, canopy, subcanopy, and seedlings were taken in 21 watersheds in the Adirondack region of NY (USA), where sugar maple and beech were the predominant canopy species and base saturation of the upper B horizon ranged from 4.4 to 67%. The base saturation value corresponding to the threshold for Al mobilization (16.8%) helped to define the species composition of canopy trees and seedlings. Canopy vigor and diameter at breast height (DBH) were positively correlated (P < 0.05) with base saturation for sugar maple, but unrelated for beech. However, beech occupied lower canopy positions than sugar maple, and as base saturation increased, the average canopy position of beech decreased relative to sugar maple (P < 0.10). In low-base saturation soils, soil-Ca depletion and BBD may have created opportunities for gap-exploiting species such as red maple and black cherry, whereas in high-base saturation soils, sugar maple dominated the canopy. Where soils were beginning to recover from acidic deposition effects, sugar maple DBH and basal area increased progressively from 2000 to 2015, whereas for beech, average DBH did not change and basal area did not increase after 2010.

Seed production and advance regeneration in Allegheny hardwood forests

Treesearch

John C. Bjorkbom

1979-01-01

The production of flowers and seeds of black cherry, red maple, sugar maple, and white ash and their relation to advance regeneration were observed from 1971 through 1976 on the Allegheny Plateau of northwestern Pennsylvania. There were good seed crops 1 year out of 2 for black cherry, 1 year out of 3 for red maple, and 1 year out of 6 for sugar maple. No white ash...

Use of damage surveys and field inventories to evaluate oak and sugar maple health in the northern United States

Treesearch

Randall S Morin; Christopher W. Woodall; Jim Steinman; Charles H. Perry

2009-01-01

Oak species (Quercus spp.) and sugar maple (Acer saccharum) are substantial components of the forest ecosystems in the 24-state region spanning the northern U.S. During recent decades, both damage surveys and forest inventories have documented declines of sugar maple and oak health. In order to more fully assess the status of oak and sugar maple health, we examined...

An Investigation of bat durability by wood species

Treesearch

Eric Ruggiero; James Sherwood; Patrick Drane; David Kretschmann

2012-01-01

Northern white ash had been the wood of choice for Major League Baseball (MLB) bats until the introduction of hard maple in the late 1990s. Since the introduction of maple to the game, there has been a perceived increase in the rate of bats to exhibit multi-piece failures (MPF)—both ash and maple. Lab and field data indicate that while a maple bat is as...

Widespread sugar maple decline and regeneration failure in the Adirondacks

Treesearch

Jerry C. Jenkins; Elizabeth Moffett; Daphne Ross

1999-01-01

Over large areas of the Adirondacks, hardwood stands whose canopies are dominated by or contain abundant mature sugar maple (Acer saccharum Marsh.) have almost no sugar maple saplings or seedlings in the understory.

Sugar maple ecology and health: proceedings of an international symposium

Treesearch

Stephen B. Horsley; Robert P. Long; eds.

1999-01-01

Contains 28 papers and abstracts on sugar maple history and ecology; recent sugar maple declines; nutrient and belowground dynamics in northeastern forests; and interactions of forest health with biotic and abiotic stressors.

After the Maples--What Then?

ERIC Educational Resources Information Center

Trisler, Carmen E.

1994-01-01

Uses models to illustrate the possible "migration route" of the sugar maple in response to predicted global climate change. Curriculum activities for students are provided that specifically address the sugar maple forests of the Great Lakes regions. (ZWH)

21 CFR 520.452 - Clenbuterol syrup.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 6 2010-04-01 2010-04-01 false Clenbuterol syrup. 520.452 Section 520.452 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS ORAL DOSAGE FORM NEW ANIMAL DRUGS § 520.452 Clenbuterol syrup. (a...

21 CFR 520.452 - Clenbuterol syrup.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 21 Food and Drugs 6 2013-04-01 2013-04-01 false Clenbuterol syrup. 520.452 Section 520.452 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS ORAL DOSAGE FORM NEW ANIMAL DRUGS § 520.452 Clenbuterol syrup. (a...

21 CFR 520.452 - Clenbuterol syrup.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 21 Food and Drugs 6 2014-04-01 2014-04-01 false Clenbuterol syrup. 520.452 Section 520.452 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS ORAL DOSAGE FORM NEW ANIMAL DRUGS § 520.452 Clenbuterol syrup. (a...

21 CFR 520.452 - Clenbuterol syrup.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 21 Food and Drugs 6 2012-04-01 2012-04-01 false Clenbuterol syrup. 520.452 Section 520.452 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS ORAL DOSAGE FORM NEW ANIMAL DRUGS § 520.452 Clenbuterol syrup. (a...

21 CFR 520.452 - Clenbuterol syrup.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 21 Food and Drugs 6 2011-04-01 2011-04-01 false Clenbuterol syrup. 520.452 Section 520.452 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS ORAL DOSAGE FORM NEW ANIMAL DRUGS § 520.452 Clenbuterol syrup. (a...

7 CFR 1435.200 - Information reporting.

Code of Federal Regulations, 2011 CFR

2011-01-01

... on the production, consumption, and trade of high fructose corn syrup in Mexico and publish the data..., cane sugar refiner, and importer of sugar, syrup, and molasses shall report, by the 20th of each month..., sugarcane yields and sugarcane planted acres. (e) Importers of sugars, syrups, or molasses to be used for...

7 CFR 1435.200 - Information reporting.

Code of Federal Regulations, 2012 CFR

2012-01-01

... on the production, consumption, and trade of high fructose corn syrup in Mexico and publish the data..., cane sugar refiner, and importer of sugar, syrup, and molasses shall report, by the 20th of each month..., sugarcane yields and sugarcane planted acres. (e) Importers of sugars, syrups, or molasses to be used for...

7 CFR 1435.200 - Information reporting.

Code of Federal Regulations, 2010 CFR

2010-01-01

... on the production, consumption, and trade of high fructose corn syrup in Mexico and publish the data..., cane sugar refiner, and importer of sugar, syrup, and molasses shall report, by the 20th of each month..., sugarcane yields and sugarcane planted acres. (e) Importers of sugars, syrups, or molasses to be used for...

7 CFR 1435.200 - Information reporting.

Code of Federal Regulations, 2013 CFR

2013-01-01

... on the production, consumption, and trade of high fructose corn syrup in Mexico and publish the data..., cane sugar refiner, and importer of sugar, syrup, and molasses shall report, by the 20th of each month..., sugarcane yields and sugarcane planted acres. (e) Importers of sugars, syrups, or molasses to be used for...

7 CFR 1435.200 - Information reporting.

Code of Federal Regulations, 2014 CFR

2014-01-01

... on the production, consumption, and trade of high fructose corn syrup in Mexico and publish the data..., cane sugar refiner, and importer of sugar, syrup, and molasses shall report, by the 20th of each month..., sugarcane yields and sugarcane planted acres. (e) Importers of sugars, syrups, or molasses to be used for...

Bioavailability of paracetamol, phenylephrine hydrochloride and guaifenesin in a fixed-combination syrup versus an oral reference product.

PubMed

Janin, Annick; Monnet, Joelle

2014-04-01

The primary objective of this study was to compare the bioavailability of paracetamol, phenylephrine hydrochloride and guaifenesin in a new oral syrup with an established oral reference product. The secondary objective was to compare the safety of the new syrup and the reference product. This was a single-centre, open-label, randomized, reference-replicated, crossover study. Healthy adult volunteers received one dose of syrup and two separate doses of a reference oral liquid formulation in a randomized sequence over three study periods, with a washout interval of ≥ 7 days between study periods. Blood samples were taken regularly postdose and analysed for paracetamol, phenylephrine hydrochloride and guaifenesin concentrations; adverse events were recorded. This study enrolled 45 subjects. For paracetamol and guaifenesin, the syrup and reference product were considered to be bioequivalent. Bioequivalence was not shown for phenylephrine hydrochloride. All adverse events were mild or moderate, most of which were considered formulation related. The syrup did not reach bioequivalence with the reference product, as bioequivalence could not be shown for phenylephrine hydrochloride. This may be due to differences in the excipients between the two products. Both the syrup and the reference product had a good safety profile and were well tolerated.

Sugar Profile, Mineral Content, and Rheological and Thermal Properties of an Isomerized Sweet Potato Starch Syrup

PubMed Central

Dominque, Brunson; Gichuhi, Peter N.; Rangari, Vijay; Bovell-Benjamin, Adelia C.

2013-01-01

Currently, corn is used to produce more than 85% of the world's high fructose syrup (HFS). There is a search for alternative HFS substrates because of increased food demand and shrinking economies, especially in the developing world. The sweet potato is a feasible, alternative raw material. This study isomerized a high glucose sweet potato starch syrup (SPSS) and determined its sugar profile, mineral content, and rheological and thermal properties. Rheological and thermal properties were measured using a rheometer and DSC, respectively. Sweet potato starch was hydrolyzed to syrup with a mean fructose content of 7.6 ± 0.4%. The SPSS had significantly higher (P < 0.05) mineral content when compared to commercial ginger and pancake syrups. During 70 days of storage, the SPSS acted as a non-Newtonian, shear-thinning liquid in which the viscosity decreased as shear stress increased. Water loss temperature of the SPSS continually decreased during storage, while pancake and ginger syrups' peak water loss temperature decreased initially and then increased. Further and more detailed studies should be designed to further enhance the fructose content of the syrup and observe its stability beyond 70 days. The SPSS has the potential to be used in human food systems in space and on Earth. PMID:26904593

Sugar Profile, Mineral Content, and Rheological and Thermal Properties of an Isomerized Sweet Potato Starch Syrup.

PubMed

Dominque, Brunson; Gichuhi, Peter N; Rangari, Vijay; Bovell-Benjamin, Adelia C

2013-01-01

Currently, corn is used to produce more than 85% of the world's high fructose syrup (HFS). There is a search for alternative HFS substrates because of increased food demand and shrinking economies, especially in the developing world. The sweet potato is a feasible, alternative raw material. This study isomerized a high glucose sweet potato starch syrup (SPSS) and determined its sugar profile, mineral content, and rheological and thermal properties. Rheological and thermal properties were measured using a rheometer and DSC, respectively. Sweet potato starch was hydrolyzed to syrup with a mean fructose content of 7.6 ± 0.4%. The SPSS had significantly higher (P < 0.05) mineral content when compared to commercial ginger and pancake syrups. During 70 days of storage, the SPSS acted as a non-Newtonian, shear-thinning liquid in which the viscosity decreased as shear stress increased. Water loss temperature of the SPSS continually decreased during storage, while pancake and ginger syrups' peak water loss temperature decreased initially and then increased. Further and more detailed studies should be designed to further enhance the fructose content of the syrup and observe its stability beyond 70 days. The SPSS has the potential to be used in human food systems in space and on Earth.

Maple Flooring - How Architects View it

Treesearch

Gary R. Lindell

1971-01-01

In a study of architects'' opinions of maple flooring versus vinyl asbestos tile for school gymnasuims, resiliency and durability were found to be important factors favoring maple whereas vinyl asbestos tile was felt to be less costly and more dimesionally stable.

Mold-Ripened Soft Cheeses Fortified with Date Palm Fruit Product as Functional Dairy Products.

PubMed

Al-Otaibi, Mutlag M; Haddadin, Jamal S; Haddadin, Malik S Y

2016-01-01

Date fruit based products are gaining popularity among the consumers in almost all date growing countries due to its added nutritional value. Therefore, novel products were developed by combining two types of foods i.e., soft ripened cheeses and date fruit syrups or date powder. This study is the first to report the surface mold-ripened cheese production with date syrup and date powder. Model cheeses were prepared from pasteurized milk inoculated with Streptococcus thermophilus, Penicillium camemberti and Geotrichum candidum. Date syrup-1, date syrup-2, date powder or the date mixture were added at the stage of curdling. Based on the kinetic growth of the microbial groups in all the treatments, there was no change in the growth of these in various date palm product. On the contrary It may be said that addition of the date fruit product supports their growth. After 35 days, the amounts of total poly phenols were 128.3 ± 1.01, 81.8 ± 1.11, 33.5 ± 2.19, 156.23 ± 1.27 mg GAE/100 g in the cheeses support with date syrup-1, date syrup-2, date powder or the date mixture, respectively. Antioxidant activity of date fruits ranged from 80.13 IC50 (date syrup-2) to 82.23 IC50 (date syrup-1). Based on the chemical characteristics and sensory analysis, the study results showed the potential for innovative application of date products for developing new functional dairy products as an ideal medium for the delivery of biological active compounds with beneficial health effects over.

Analysis of Dextromethorphan in Cough Drops and Syrups: A Medicinal Chemistry Laboratory

ERIC Educational Resources Information Center

Hamilton, Todd M.; Wiseman, Frank L., Jr.

2009-01-01

Fluorescence spectroscopy is used to determine the quantity of dextromethorphan hydrobromide (DM) in over-the-counter (OTC) cough drops and syrups. This experiment is appropriate for an undergraduate medicinal chemistry laboratory course when studying OTC medicines and active ingredients. Students prepare the cough drops and syrups for analysis,…

78 FR 146 - Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-01-02

... OFFICE OF THE UNITED STATES TRADE REPRESENTATIVE Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of Chile, Morocco, Costa Rica, the Dominican Republic... the determination of the trade surplus in certain sugar and syrup goods and sugar containing products...

Qualitative and quantitative control of pediatric syrups using Nuclear Magnetic Resonance and chemometrics.

PubMed

Alves Filho, Elenilson G; Silva, Lorena Mara A; Araújo, Nathália V P; Alves, Elenilson G; Lião, Luciano M; Alcantara, Glaucia B

2018-05-10

Several flavoring and sweetening agents added to excipient of pediatric syrups are not declared in the package leaflet. Therefore, the aim of this study was to develop a non-target, simple, and precise method for qualitative and quantitative evaluation of pediatric syrups using NMR spectroscopy combined with chemometrics. This approach allowed the identification of several added compounds as citric acid, cyclamate, ethanol, glycerol, propylene glycol, saccharin, sorbitol, fructose, glucose, and sucrose. Among the sugared syrups, sucrose was the main carbohydrate with approximately 59.1%, and for sweetened syrups, glycerol with 25.5%. The ethanol was found with highest concentration of 4.0%, approximately. In addition, some syrups presented both sugar and sweetener, which is inconsistent according to the purpose of the addition. Consequently, institutional structures of countries as Brazil that are in charge of public health should put additional compliance pressure on pharmaceutical companies to clearly declare in package leaflet the presence and exact amount of the main compounds (at least) existent in the pediatric excipients. Copyright © 2018 Elsevier B.V. All rights reserved.

PECASE: Multi-Spectral Photon Detection in Polymer/Nanoparticle Composites-Toward IR Photodectors and Solar Cells Applicable to Unmanned Vehicles

DTIC Science & Technology

2016-03-31

in Polymer/Nanoparticle Composites-Toward IR Photodectors and Solar Cells Applicable to Sb. GRANT NUMBER Unmanned Vehicles N00014-1 0-1-0481 Sc...photodetectors and solar cells deposited by RIR-MAPLE, and developing a simulation tool for optoelectronic device performance that accounts for RIR...MAPLE film properties. 1S. SUBJECT TERMS Hybrid nanocomposites, MAPLE, RIR-MAPLE, intraband absorption, mid-IR photodetectors, organic solar cells

Detection of adulterated honey produced by honeybee (Apis mellifera L.) colonies fed with different levels of commercial industrial sugar (C₃ and C₄ plants) syrups by the carbon isotope ratio analysis.

PubMed

Guler, Ahmet; Kocaokutgen, Hasan; Garipoglu, Ali V; Onder, Hasan; Ekinci, Deniz; Biyik, Selim

2014-07-15

In the present study, one hundred pure and adulterated honey samples obtained from feeding honeybee colonies with different levels (5, 20 and 100 L/colony) of various commercial sugar syrups including High Fructose Corn Syrup 85 (HFCS-85), High Fructose Corn Syrup 55 (HFCS-55), Bee Feeding Syrup (BFS), Glucose Monohydrate Sugar (GMS) and Sucrose Sugar (SS) were evaluated in terms of the δ(13)C value of honey and its protein, difference between the δ(13)C value of protein and honey (Δδ(13)C), and C4% sugar ratio. Sugar type, sugar level and the sugar type*sugar level interaction were found to be significant (P<0.001) regarding the evaluated characteristics. Adulterations could not be detected in the 5L/colony syrup level of all sugar types when the δ(13)C value of honey, Δδ(13)C (protein-honey), and C4% sugar ratio were used as criteria according to the AOAC standards. However, it was possible to detect the adulteration by using the same criteria in the honeys taken from the 20 and 100 L/colony of HFCS-85 and the 100L/colony of HFCS-55. Adulteration at low syrup level (20 L/colony) was more easily detected when the fructose content of HFCS syrup increased. As a result, the official methods (AOAC, 978.17, 1995; AOAC, 991.41, 1995; AOAC 998.12, 2005) and Internal Standard Carbon Isotope Ratio Analysis could not efficiently detect the indirect adulteration of honey obtained by feeding the bee colonies with the syrups produced from C3 plants such as sugar beet (Beta vulgaris) and wheat (Triticium vulgare). For this reason, it is strongly needed to develop novel methods and standards that can detect the presence and the level of indirect adulterations. Copyright © 2014 Elsevier Ltd. All rights reserved.

An automated system for evaluation of the potential functionome: MAPLE version 2.1.0

PubMed Central

Takami, Hideto; Taniguchi, Takeaki; Arai, Wataru; Takemoto, Kazuhiro; Moriya, Yuki; Goto, Susumu

2016-01-01

Metabolic and physiological potential evaluator (MAPLE) is an automatic system that can perform a series of steps used in the evaluation of potential comprehensive functions (functionome) harboured in the genome and metagenome. MAPLE first assigns KEGG Orthology (KO) to the query gene, maps the KO-assigned genes to the Kyoto Encyclopedia of Genes and Genomes (KEGG) functional modules, and then calculates the module completion ratio (MCR) of each functional module to characterize the potential functionome in the user’s own genomic and metagenomic data. In this study, we added two more useful functions to calculate module abundance and Q-value, which indicate the functional abundance and statistical significance of the MCR results, respectively, to the new version of MAPLE for more detailed comparative genomic and metagenomic analyses. Consequently, MAPLE version 2.1.0 reported significant differences in the potential functionome, functional abundance, and diversity of contributors to each function among four metagenomic datasets generated by the global ocean sampling expedition, one of the most popular environmental samples to use with this system. MAPLE version 2.1.0 is now available through the web interface (http://www.genome.jp/tools/maple/) 17 June 2016, date last accessed. PMID:27374611

Maple sap as a rich medium to grow probiotic lactobacilli and to produce lactic acid.

PubMed

Cochu, A; Fourmier, D; Halasz, A; Hawari, J

2008-12-01

To demonstrate the feasibility of growing lactobacilli and producing lactic acid using maple sap as a sugar source and to show the importance of oligosaccharides in the processes. Two maple sap samples (Cetta and Pinnacle) and purified sucrose were used as carbon sources in the preparation of three culture media. Compared with the sucrose-based medium, both maple sap-based media produced increased viable counts in two strains out of five by a factor of four to seven. Maple sap-based media also enhanced lactic acid production in three strains. Cetta sap was found to be more efficient than Pinnacle sap in stimulating lactic acid production and, was also found to be richer in various oligosaccharides. The amendment of the Pinnacle-based medium with trisaccharides significantly stimulated Lactobacillus acidophilus AC-10 to grow and produce lactic acid. Maple sap, particularly if rich in oligosaccharides, represents a good carbon source for the growth of lactobacilli and the production of lactic acid. This study provides a proof-of-concept, using maple sap as a substrate for lactic acid production and for the development of a nondairy probiotic drink.

Seasonal and regional diversity of maple sap microbiota revealed using community PCR fingerprinting and 16S rRNA gene clone libraries.

PubMed

Filteau, Marie; Lagacé, Luc; LaPointe, Gisèle; Roy, Denis

2010-04-01

An arbitrary primed community PCR fingerprinting technique based on capillary electrophoresis was developed to study maple sap microbial community characteristics among 19 production sites in Québec over the tapping season. Presumptive fragment identification was made with corresponding fingerprint profiles of bacterial isolate cultures. Maple sap microbial communities were subsequently compared using a representative subset of 13 16S rRNA gene clone libraries followed by gene sequence analysis. Results from both methods indicated that all maple sap production sites and flow periods shared common microbiota members, but distinctive features also existed. Changes over the season in relative abundance of predominant populations showed evidence of a common pattern. Pseudomonas (64%) and Rahnella (8%) were the most abundantly and frequently represented genera of the 2239 sequences analyzed. Janthinobacterium, Leuconostoc, Lactococcus, Weissella, Epilithonimonas and Sphingomonas were revealed as occasional contaminants in maple sap. Maple sap microbiota showed a low level of deep diversity along with a high variation of similar 16S rRNA gene sequences within the Pseudomonas genus. Predominance of Pseudomonas is suggested as a typical feature of maple sap microbiota across geographical regions, production sites, and sap flow periods.

Time domain-nuclear magnetic resonance study of chars from southern hardwoods

Treesearch

Thomas Elder; Nicole Labbe; David Harper; Timothy Rials

2006-01-01

Chars from the thermal degradation of silver maple (Acer saccharinum), red maple (Acer rubrum), sugar maple (Acer saccharum), and white oak (Quercus spp.), performed at temperatures from 250 to 350 oC, were examined using time domain-nuclear magnetic resonance...

Altered Acer Rubrum Fecundity Induced By Chemical Climate Change

NASA Astrophysics Data System (ADS)

Deforest, J. L.; Peters, A.

2014-12-01

Red maple (Acer rubrum L.) is becoming the most dominating tree in North American eastern deciduous forests. Concurrently, human activities have altered the chemical climate of terrestrial ecosystems via acidic deposition, which increases the available of nitrogen (N), while decreasing phosphorus (P) availability. Once a minor forest component prior to European settlement, the abundance of red maple may be a symptom of the modern age. The current paradigm explaining red maple's rise to prominence concerns fire suppression that excludes competitors. However, this still does not explain why red maple is unique compared to other functionally similar trees. The objective of this study was to investigate the interactive influence of acid rain mitigation on the fecundity of red maple. Objectives were achieved by measuring flowering, seed production, germination, and growth from red maple on plots that have been experimentally manipulated to increase soil pH, P, or both in three unglaciated eastern deciduous hardwood forests. At least 50% of the red maple population is seed bearing in our control soils, however the median percent of seed-bearing trees declined to zero when mitigating soils from acidic deposition. This can be explained by the curious fact that red maple is polygamodioecious, or has labile sex-expression, in which an individual tree can change its sex-expression in response to the environment. Furthermore, seed-bearing trees in the mitigated plots grew less, produced less seeds, and germinated at lower rates than their counterparts in control soils. Our results provide evidence that chemical climate change could be the primary contributing factor accelerating the dominance of red maple in eastern North American forests. Our observations can provide a boarder conceptual framework for understanding how nutrient limitations can be applied beyond plant productivity towards explaining distribution changes in vegetation.

75 FR 77042 - Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar Containing Products of...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-10

... OFFICE OF THE UNITED STATES TRADE REPRESENTATIVE Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar Containing Products of Chile, Morocco, Costa Rica, the Dominican Republic... notice of its determination of the trade surplus in certain sugar and syrup goods and sugar-containing...

76 FR 81005 - Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-12-27

... OFFICE OF THE UNITED STATES TRADE REPRESENTATIVE Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of Chile, Morocco, Costa Rica, the Dominican Republic...) is providing notice of its determination of the trade surplus in certain sugar and syrup goods and...

78 FR 73234 - Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-12-05

... OFFICE OF THE UNITED STATES TRADE REPRESENTATIVE Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of Chile, Morocco, Costa Rica, the Dominican Republic... (USTR) is providing notice of its determination of the trade surplus in certain sugar and syrup goods...

77 FR 74726 - Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-12-17

... OFFICE OF THE UNITED STATES TRADE REPRESENTATIVE Determination of Trade Surplus in Certain Sugar and Syrup Goods and Sugar-Containing Products of Chile, Morocco, Costa Rica, the Dominican Republic... (USTR) is providing notice of its determination of the trade surplus in certain sugar and syrup goods...

21 CFR 184.1866 - High fructose corn syrup.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 21 Food and Drugs 3 2011-04-01 2011-04-01 false High fructose corn syrup. 184.1866 Section 184... Listing of Specific Substances Affirmed as GRAS § 184.1866 High fructose corn syrup. (a) High fructose... entitled “High-Fructose Corn Syrup” in the Food Chemicals Codex, 4th ed. (1996), pp. 191-192, which is...

21 CFR 173.357 - Materials used as fixing agents in the immobilization of enzyme preparations.

Code of Federal Regulations, 2012 CFR

2012-04-01

... manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter. Cellulose triacetate... glucose isomerase enzyme preparations for use in the manufacture of high fructose corn syrup, in... enzyme preparations for use in the manufacture of high fructose corn syrup, in accordance with § 184.1372...

21 CFR 173.357 - Materials used as fixing agents in the immobilization of enzyme preparations.

Code of Federal Regulations, 2013 CFR

2013-04-01

... manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter. Cellulose triacetate... glucose isomerase enzyme preparations for use in the manufacture of high fructose corn syrup, in... enzyme preparations for use in the manufacture of high fructose corn syrup, in accordance with § 184.1372...

21 CFR 184.1866 - High fructose corn syrup.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 21 Food and Drugs 3 2013-04-01 2013-04-01 false High fructose corn syrup. 184.1866 Section 184... Listing of Specific Substances Affirmed as GRAS § 184.1866 High fructose corn syrup. (a) High fructose... entitled “High-Fructose Corn Syrup” in the Food Chemicals Codex, 4th ed. (1996), pp. 191-192, which is...

21 CFR 184.1866 - High fructose corn syrup.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 3 2010-04-01 2009-04-01 true High fructose corn syrup. 184.1866 Section 184.1866... Listing of Specific Substances Affirmed as GRAS § 184.1866 High fructose corn syrup. (a) High fructose... entitled “High-Fructose Corn Syrup” in the Food Chemicals Codex, 4th ed. (1996), pp. 191-192, which is...

21 CFR 173.357 - Materials used as fixing agents in the immobilization of enzyme preparations.

Code of Federal Regulations, 2011 CFR

2011-04-01

... manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter. Cellulose triacetate... glucose isomerase enzyme preparations for use in the manufacture of high fructose corn syrup, in... enzyme preparations for use in the manufacture of high fructose corn syrup, in accordance with § 184.1372...

21 CFR 173.357 - Materials used as fixing agents in the immobilization of enzyme preparations.

Code of Federal Regulations, 2010 CFR

2010-04-01

... manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter. Cellulose triacetate... glucose isomerase enzyme preparations for use in the manufacture of high fructose corn syrup, in... enzyme preparations for use in the manufacture of high fructose corn syrup, in accordance with § 184.1372...

21 CFR 184.1866 - High fructose corn syrup.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 21 Food and Drugs 3 2012-04-01 2012-04-01 false High fructose corn syrup. 184.1866 Section 184... Listing of Specific Substances Affirmed as GRAS § 184.1866 High fructose corn syrup. (a) High fructose... entitled “High-Fructose Corn Syrup” in the Food Chemicals Codex, 4th ed. (1996), pp. 191-192, which is...

78 FR 12591 - Amendment to the Standards of Identity for Distilled Spirits

Federal Register 2010, 2011, 2012, 2013, 2014

2013-02-25

... ``Cacha[ccedil]a'' any spirits that use corn or corn syrup in the fermentation process. Some product... have been manufactured using a small quantity of corn or corn syrup in the fermentation process. Since... standard for Cacha[ccedil]a would not allow for the use of corn or corn syrup in the fermentation process...

Effect of conventional and sugar free pediatric syrup formulations on primary tooth enamel hardness: An in vitro study.

PubMed

Mali, Gaurao Vasant; Dodamani, Arun Suresh; Karibasappa, Gundabaktha Nagappa; Kumar, Prashanth Vishwakarma; Jain, Vardhaman Mulchand

2015-01-01

To assess and compare the effect of conventional and sugar free pediatric syrup formulations on primary tooth enamel hardness over a period of 14 days. An in vitro study was done on 40 noncarious deciduous teeth. 10 teeth in each group were dipped in 4 pediatric medicinal syrups (1 sugarfree and 3 conventional) for 1 min thrice daily for 14 days and the enamel surface micro hardness was checked at baseline, 7 th day and 14 th day by Vickers hardness testing machine. The pH, titratable acidity and buffering capacity of the syrups were assessed. The pH of syrups were above critical pH for demineralization of the tooth but tiratable acidity and buffering capacity differed. ANOVA test indicated that the reduction in mean micro hardness was maximum in Group D (Conventional Analgesic syrup) and least in Group A (Sugarfree cough syrup) on 7 th and 14 th day. On intergroup comparison there was no difference (P > 0.05) in micro hardness between Group B (Conventional Cough syrup) and Group C (Conventional Antibiotic). However, highly significant (P < 0.01) difference between the either pair of Group B with Group D, and Group C with Group D on 14 th day. The percentage reduction in micro hardness on 14 th day was maximum for Group D (24.4 ± 2.2) and minimum for Group A (14.0 ± 1.3) which was statistically significant (P < 0.01). Sugar free pediatric medicines can be effective in reducing dental erosion and efforts should be made to incorporate sugar substitutes in formulation of pediatric medicines.

The Maple Sugar Festival

ERIC Educational Resources Information Center

Johnston, Basil

1978-01-01

Describing the Iroquoi's Maple Sugar Festival, this article details the symbolism of renewal, becoming, and regeneration celebrated by the Iroquoi as the sap from the maple trees begins to flow each year. The symbolic role of woman, the sweet sap itself, and man's fellow creatures are described. (JC)

Effect of concentration temperature on some bioactive compounds and antioxidant proprieties of date syrup.

PubMed

Abbès, Fatma; Besbes, Souhail; Brahim, Bchir; Kchaou, Wissal; Attia, Hamadi; Blecker, Christophe

2013-08-01

The effect of the concentration temperature on the antioxidant activity, carotenoid and phenolic compounds of date syrup was investigated. Date juice was concentrated at 100  and at "60  in vacuum". After concentration, total phenolic, tannin, non-tannin, flavonoid and carotenoid content were determined spectrophotometrically and high-performance liquid chromatography was used for determination of 5-Hydroxymethyl-2-furfuraldehyde content. The antioxidant activity of date syrup was evaluated by various antioxidant methods including total antioxidant, 2,2-diphenyl-1-picrylhydrazyl free radical scavenging test, ferric reducing antioxidant power and β-carotene bleaching. All date syrups showed strong antioxidant activity accompanied by high total phenolic contents. Results showed that concentration at 100  significantly enhanced the antioxidant activities and total phenolic contents of date syrups compared to vacuum concentration at 60 . A good correlation between the antioxidant activity and total phenolic content and flavonoid was observed.

Non-nutritive sweeteners are not super-normal stimuli

PubMed Central

Antenucci, Rachel G.; Hayes, John E.

2014-01-01

Background It is often claimed that non-nutritive sweeteners (NNS) are ‘sweeter than sugar’, with the implicit implication high potency sweeteners are super-normal stimuli that encourage exaggerated responses. This study aimed to investigate the perceived sweetness intensity of a variety of nutritive (Sucrose, Maple Syrup, and Agave Nectar) and NNS (Acesulfame-K (AceK), Rebaudioside A (RebA), Aspartame, and Sucralose) in a large cohort of untrained participants using contemporary psychophysical methods. Methods Participants (n=401 total) rated the intensity of sweet, bitter, and metallic sensations for nutritive and NNS in water using the general labeled magnitude scale (gLMS). Results Sigmoidal Dose-Response functions were observed for all stimuli except AceK. That is, sucrose follows a sigmoidal function if the data are not artifactually linearized via prior training. More critically, there is no evidence that NNS have a maximal sweetness (intensity) greater than sucrose; indeed, the maximal sweetness for AceK, RebA and Sucralose were significantly lower than for concentrated sucrose. For these sweeteners, mixture suppression due to endogenous dose-dependent bitter or metallic sensations appears to limit maximal perceived sweetness. Conclusions In terms of perceived sweetness, non-nutritive sweeteners cannot be considered super-normal stimuli. These data do not support the view that non-nutritive sweeteners hijack or over-stimulate sweet receptors to product elevated sweet sensations. PMID:24942868

Developmental Defects of Caenorhabditis elegans Lacking Branched-chain α-Ketoacid Dehydrogenase Are Mainly Caused by Monomethyl Branched-chain Fatty Acid Deficiency.

PubMed

Jia, Fan; Cui, Mingxue; Than, Minh T; Han, Min

2016-02-05

Branched-chain α-ketoacid dehydrogenase (BCKDH) catalyzes the critical step in the branched-chain amino acid (BCAA) catabolic pathway and has been the focus of extensive studies. Mutations in the complex disrupt many fundamental metabolic pathways and cause multiple human diseases including maple syrup urine disease (MSUD), autism, and other related neurological disorders. BCKDH may also be required for the synthesis of monomethyl branched-chain fatty acids (mmBCFAs) from BCAAs. The pathology of MSUD has been attributed mainly to BCAA accumulation, but the role of mmBCFA has not been evaluated. Here we show that disrupting BCKDH in Caenorhabditis elegans causes mmBCFA deficiency, in addition to BCAA accumulation. Worms with deficiency in BCKDH function manifest larval arrest and embryonic lethal phenotypes, and mmBCFA supplementation suppressed both without correcting BCAA levels. The majority of developmental defects caused by BCKDH deficiency may thus be attributed to lacking mmBCFAs in worms. Tissue-specific analysis shows that restoration of BCKDH function in multiple tissues can rescue the defects, but is especially effective in neurons. Taken together, we conclude that mmBCFA deficiency is largely responsible for the developmental defects in the worm and conceivably might also be a critical contributor to the pathology of human MSUD. © 2016 by The American Society for Biochemistry and Molecular Biology, Inc.

Climate and hydrological changes in the northeastern United States: recent trends and implications for forested and aquatic ecosystems

USGS Publications Warehouse

Huntington, Thomas G.; Richardson, Andrew D.; McGuire, Kevin J.; Hayhoe, Katharine

2009-01-01

We review twentieth century and projected twenty-first century changes in climatic and hydrologic conditions in the northeastern United States and the implications of these changes for forest ecosystems. Climate warming and increases in precipitation and associated changes in snow and hydrologic regimes have been observed over the last century, with the most pronounced changes occurring since 1970. Trends in specific climatic and hydrologic variables differ in their responses spatially (e.g., coastal vs. inland) and temporally (e.g., spring vs. summer). Trends can differ depending on the period of record analyzed, hinting at the role of decadal-scale climatic variation that is superimposed over the longer-term trend. Model predictions indicate that continued increases in temperature and precipitation across the northeastern United States can be expected over the next century. Ongoing increases in growing season length (earlier spring and later autumn) will most likely increase evapotranspiration and frequency of drought. In turn, an increase in the frequency of drought will likely increase the risk of fire and negatively impact forest productivity, maple syrup production, and the intensity of autumn foliage coloration. Climate and hydrologic changes could have profound effects on forest structure, composition, and ecological functioning in response to the changes discussed here and as described in related articles in this issue of the Journal.

Experience of the Manitoba Perinatal Screening Program, 1965-85.

PubMed Central

Fox, J G

1987-01-01

The Manitoba Perinatal Screening Program is guided by a committee of medical specialists with skills in the diagnosis and management of disorders of metabolism in the newborn. The program is voluntary and is centralized at Cadham Provincial Laboratory, in Winnipeg. A filter card blood specimen is collected from newborns on discharge from hospital, and a filter card urine sample is collected and mailed to the laboratory by the mother when the infant is about 2 weeks of age. The overall compliance rates for the blood and urine specimens are approximately 100% and 84% respectively. The blood specimen is screened for phenylalanine and other amino acids, thyroxine, galactose, galactose-1-phosphate and biotinidase. The urine specimen is screened for amino acids, including cystine, as well as methylmalonic acid and homocystine. Between 1965 and 1985, 83 cases of metabolic disorders were detected, including 23 cases of primary hypothyroidism, 14 of classic phenylketonuria, 5 of galactosemia variants, 3 of galactosemia, 2 of maple syrup urine disease and 1 of hereditary tyrosinemia. The direct cost per infant screened is $5.50, and the cost:benefit ratio is approximately 7.5:1. Maternal serum alpha-fetoprotein screening is being made available as the necessary supporting clinical facilities become available. On the basis of this experience, the author outlines the components that are important for an effective screening program. PMID:3676929

The first case of domino-split-liver transplantation in maple syrup urine disease.

PubMed

Herden, Uta; Li, Jun; Fischer, Lutz; Brinkert, Florian; Blohm, Martin; Santer, René; Nashan, Bjoern; Grabhorn, Enke

2017-09-01

The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet. The organ was allocated to a 14-year-old girl (55 kg) with primary sclerosing cholangitis. Due to excellent organ quality and suitable anatomy, a backward split for a girl of 3 months (5 kg) with decompensated liver cirrhosis due to biliary atresia was performed. The postoperative course was without relevant complications, and the three recipients were discharged on postoperative days 28, 29, and 45, respectively, with good organ function. BCAAs in plasma were normal in the two domino graft recipients, and the MSUD patient showed mildly elevated but stable BCAA concentrations despite an unrestricted diet. Split-domino LTX enabled successful transplantation of three patients of the waiting list with only one deceased donor graft. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Living donor domino liver transplantation using a maple syrup urine disease donor: A case series of three children - The first report from Japan.

PubMed

Matsunami, Masatoshi; Fukuda, Akinari; Sasaki, Kengo; Uchida, Hajime; Shigeta, Takanobu; Hirata, Yoshihiro; Kanazawa, Hiroyuki; Horikawa, Reiko; Nakazawa, Atsuko; Suzuki, Tatsuya; Mizuta, Koichi; Kasahara, Mureo

2016-08-01

As the priority of LD-Domino LT is the safety of the first recipient, limitations and technical difficulties in the second recipient often occur. The most technically challenging part of LD-Domino LT is the reconstruction of the vessels. For the reconstruction of HVs, the native HVs were exteriorized as far as possible using a CUSA because longer extensive HVs are essential for facilitating the reconstruction. At the back table, the HVs of the domino graft were sutured together, and the single cuff of the HVs was anastomosed to the IVC by joining the orifices. The HAs, the presence of insufficient length, and multiple vessels in the whole liver rendered the reconstruction more difficult. We determined the dividing sites of the vessels according to the preoperative 3D-CT findings obtained in two institutions. This is the first case series using grafts in DLT obtained from LDLT for patients with MSUD between two institutions. In conclusion, LD-Domino LT is a safe and feasible therapeutic option to expand the donor pool by technical refinement in the reconstruction of the second recipient. Further studies with a greater accumulation of patients and a longer follow-up will be necessary to establish LD-Domino LT using an MSUD donor. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Newborn screening by matrix-assisted laser desorption/ionization mass spectrometry based on parylene-matrix chip.

PubMed

Kim, Jo-Il; Noh, Joo-Yoon; Kim, Mira; Park, Jong-Min; Song, Hyun-Woo; Kang, Min-Jung; Pyun, Jae-Chul

2017-08-01

Newborn screening for diagnosis of phenylketonuria, homocystinuria, and maple syrup urine disease have been conducted by analyzing the concentration of target amino acids using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-ToF MS) based on parylene-matrix chip. Parylene-matrix chip was applied to MALDI-ToF MS analysis reducing the matrix peaks significantly at low mass-to-charge ratio range (m/z < 500). Reproducibility of inter-spot and intra-spot analyses of amino acids was less than 10%. Methanol extraction was adopted for simple and rapid sample preparation of serum before mass spectrometric analysis showing 13.3 to 45% of extraction efficiency. Calibration curves for diagnosis of neonatal metabolic disorders were obtained by analyzing methanol-extracted serum spiked with target amino acids using MALDI-ToF MS. They showed good linearity (R 2  > 0.98) and the LODs were ranging from 9.0 to 22.9 μg/mL. Effect of proteins in serum was estimated by comparing MALDI-ToF mass spectra of amino acids-spiked serum before and after the methanol extraction. Interference of other amino acids on analysis of target analyte was determined to be insignificant. From these results, MALDI-ToF MS based on parylene-matrix chip could be applicable to medical diagnosis of neonatal metabolic disorders. Copyright © 2017 Elsevier Inc. All rights reserved.

Using Math With Maple Sugaring.

ERIC Educational Resources Information Center

Christenson, Gary

1984-01-01

Suggest several math activities using the simple technique of tapping a sugar maple tree for sap. Information and activities presented are useful in tapping one or two trees on school property, helping students who tap trees at home, or leading a field trip to a nearby maple sugaring site. (ERB)

77 FR 50464 - Honey From the People's Republic of China: Affirmative Final Determination of Circumvention of...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-21

... Determination \\1\\ of this anticircumvention inquiry, and determined that blends of honey and rice syrup are... are blends of honey and rice syrup, regardless of the percentage of honey they contain, from the PRC... of blends of honey and rice syrup in the antidumping duty order on honey from the PRC.\\3\\ The ITC...

21 CFR 173.357 - Materials used as fixing agents in the immobilization of enzyme preparations.

Code of Federal Regulations, 2014 CFR

2014-04-01

... immobilization of glucose isomerase enzyme preparations for use in the manufacture of high fructose corn syrup... for use in the manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter... use in the manufacture of high fructose corn syrup, in accordance with § 184.1372 of this chapter. 2...

Technical Protocol for Using Soluble Carbohydrates to Enhance Reductive Dechlorination of Chlorinated Aliphatic Hydrocarbons

DTIC Science & Technology

2002-12-19

High -Density Polyethylene HFCS High Fructose Corn Syrup HRC Hydrogen Release Compound HAS Hollow Stem...subsurface injection of a soluble electron donor solution (typically comprised of a carbohydrate such as molasses, whey, high fructose corn syrup (HFCS...whey, high fructose corn syrup (HFCS), glucose, lactate, butyrate, benzoate). Other approaches to enhanced anaerobic bioremediation exist, but

Principles and Practices of Enhanced Anaerobic Bioremediation of Chlorinated Solvents

DTIC Science & Technology

2004-09-01

high - fructose corn syrup (HFCS), whey, bark mulch and compost, chitin, and gaseous hydrogen. Table 1.2...Benzoate Injection wells or circulation systems Dissolved in water Continuous to monthly Molasses, High Fructose Corn Syrup Injection wells...to 0.35 High (> 100) Refined Sugars ( high fructose corn syrup ) 0.25 to 0.30 Moderate (> 20) Soluble substrates may be used for source

Principles and Practices of Enhanced Anaerobic Bioremediation of Chlorinated Solvents

DTIC Science & Technology

2004-08-01

high - fructose corn syrup - xi - 022/738863/28.doc...systems Dissolved in water Continuous to monthly Molasses, High Fructose Corn Syrup Injection wells Dissolved in water Continuous to monthly Viscous...7 to 90 days Suthersan et al., 2002; Appendices E.2 and E.11 High Fructose Corn Syrup Diluted to 1 to 10 percent by weight. 50 to 500 mg/L

21 CFR 201.308 - Ipecac syrup; warnings and directions for use for over-the-counter sale.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 4 2010-04-01 2010-04-01 false Ipecac syrup; warnings and directions for use for over-the-counter sale. 201.308 Section 201.308 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT... poisonings, ipecac syrup is considered the emetic of choice. The immediate availability of this drug for use...

77 FR 25382 - Proposed Amendment to the Standards of Identity for Distilled Spirits

Federal Register 2010, 2011, 2012, 2013, 2014

2012-04-30

... manufactured using a small quantity of corn or corn syrup in the fermentation process. Since these products do... include as ``Cacha[ccedil]a'' any spirits that use corn or corn syrup in the fermentation process. TTB has... the use of corn or corn syrup in the fermentation process. As such, under the terms of the proposed...

Changes in electrical energy requirements to operate an ice cream freezer as a function of sweeteners and gums

DOE Office of Scientific and Technical Information (OSTI.GOV)

Smith, D.E.; Bakshi, A.S.; Gay, S.A.

1985-01-01

Changes in electrical energy required to operate a continuous freezer were monitored for various ice cream formulae. Ice cream formulae consisted of nine different combinations of sucrose, 36 DE corn syrup, and 42 high fructose corn syrup as well as two ratios of guar gum to locust bean gum. Within the same sweetening system, a mix high in locust bean gum tended to have a lower energy demand than mix with large amounts of guar gum. This was especially pronounced in mixes with 50% 42 high fructose corn syrup and/or 50% 36 DE corn syrup solids.

Clinical assessment of the warming sensation accompanying flavor 316282 in a cold and cough syrup containing paracetamol, phenylephrine hydrochloride, and guaifenesin

PubMed Central

Monnet, Joëlle

2014-01-01

Objective: The primary objective was to assess the warming sensation caused by flavor 316282 in a cold and cough product in the target population. Methods: A single-cohort, single-treatment arm, open-label study. Subjects received one 30-mL dose of syrup containing flavor 316282, paracetamol, phenylephrine hydrochloride, and guaifenesin and recorded onset and disappearance of any warming sensation in the mouth/throat. Subjects’ assessment of strength and appeal of the sensation, taste, texture, and acceptability of the product as a cold and cough remedy was investigated using questionnaires. Results: A total of 51 subjects were included; 47 (92.1%) experienced a warming sensation. The median duration of the warming sensation was 100 s (95% confidence interval = 82 s, 112 s). The majority of subjects rated the syrup as excellent, good, or fair for treatment of cough and cold symptoms (96.1%), taste (80.4%), and texture (98.0%). There were no safety concerns, and the syrup was well tolerated. Most subjects liked the warming sensation. Conclusions: Flavor 316282 in a cold and cough syrup is associated with a warming sensation. The syrup is well tolerated, safe, and palatable. PMID:26770699

A randomised controlled trial of oral chloral hydrate vs. intranasal dexmedetomidine before computerised tomography in children.

PubMed

Yuen, V M; Li, B L; Cheuk, D K; Leung, M K M; Hui, T W C; Wong, I C; Lam, W W; Choi, S W; Irwin, M G

2017-10-01

Chloral hydrate is commonly used to sedate children for painless procedures. Children may recover more quickly after sedation with dexmedetomidine, which has a shorter half-life. We randomly allocated 196 children to chloral hydrate syrup 50 mg.kg -1 and intranasal saline spray, or placebo syrup and intranasal dexmedetomidine spray 3 μg.kg -1 , 30 min before computerised tomography studies. More children resisted or cried after drinking chloral hydrate syrup than placebo syrup, 72 of 107 (67%) vs. 42 of 87 (48%), p = 0.009, but there was no difference after intranasal saline vs. dexmedetomidine, 49 of 107 (46%) vs. 40 of 87 (46%), p = 0.98. Sedation was satisfactory in 81 of 107 (76%) children after chloral hydrate and 64 of 87 (74%) children after dexmedetomidine, p = 0.74. Of the 173 children followed up for at least 4 h after discharge, 38 of 97 (39%) had recovered normal function after chloral hydrate and 32 of 76 (42%) after dexmedetomidine, p = 0.76. Six children vomited after chloral hydrate syrup and placebo spray vs. none after placebo syrup and dexmedetomidine spray, p = 0.03. © 2017 The Association of Anaesthetists of Great Britain and Ireland.

What's a sugar maple worth?

Treesearch

George L. Cook

1991-01-01

What is a sugar maple worth? This is an interesting question and not one which is easy to answer. I have some thoughts on this subject to share with you. Many of these have been discussed with county and district foresters, sugarmakers and people from the community who appreciate the maple tree very much.

Chemical control of bigleaf maple trees and stump sprouts.

Treesearch

Carl M. Berntsen

1960-01-01

Control of bigleaf maple (Acer macrophyllum) has become an important consideration in many western Oregon areas that are managed primarily for Douglas-fir (Pseudotsuga menziesii) and associated conifers. The problem is acute on areas where damaged and defective maples are left after harvest of commercial timber. They sprout...

Sapstreak Disease of Sugar Maple Found is Wisconsin for the First Time

Treesearch

Kenneth J. Jr. Kessler

1972-01-01

The first occurrence in Wisconsin of sapstreak disease of sugar maple is described. The outbreak of the disease was traced to past logging activities. To prevent further disease development, sugar maples should not be cut for a few years in areas contiguos to diseased trees

Maple Explorations, Perfect Numbers, and Mersenne Primes

ERIC Educational Resources Information Center

Ghusayni, B.

2005-01-01

Some examples from different areas of mathematics are explored to give a working knowledge of the computer algebra system Maple. Perfect numbers and Mersenne primes, which have fascinated people for a very long time and continue to do so, are studied using Maple and some questions are posed that still await answers.

75 FR 57016 - Maple Analytics, LLC; Supplemental Notice That Initial Market-Based Rate Filing Includes Request...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-09-17

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER10-2541-000] Maple Analytics, LLC; Supplemental Notice That Initial Market- Based Rate Filing Includes Request for Blanket... proceeding of Maple Analytics, LLC's application for market-based rate authority, with an accompanying rate...

America's Native Sweet: Chippewa Treaties and the Right to Harvest Maple Sugar.

ERIC Educational Resources Information Center

Keller, Robert H.

1989-01-01

Argues in favor of a Chippewa right to harvest maple sap from trees on federal land. Discusses the history of Indian production of and trade in maple sugar, examines relevant treaties, and draws parallels with tribal rights to fish and harvest wild rice. Contains 91 references. (SV)

Maple Sugar Harvesting/Wild Rice Harvesting.

ERIC Educational Resources Information Center

Minneapolis Public Schools, MN.

Comprised of two separate booklets, this resource unit assists elementary teachers in explaining how the Ojibwe people harvest maple sugar and wild rice. The first booklet explains the procedure of tapping the maple trees for sap, preparation for boiling the sap, and the three forms the sugar is made into (granulated, "molded," and…

An automated system for evaluation of the potential functionome: MAPLE version 2.1.0.

PubMed

Takami, Hideto; Taniguchi, Takeaki; Arai, Wataru; Takemoto, Kazuhiro; Moriya, Yuki; Goto, Susumu

2016-07-03

Metabolic and physiological potential evaluator (MAPLE) is an automatic system that can perform a series of steps used in the evaluation of potential comprehensive functions (functionome) harboured in the genome and metagenome. MAPLE first assigns KEGG Orthology (KO) to the query gene, maps the KO-assigned genes to the Kyoto Encyclopedia of Genes and Genomes (KEGG) functional modules, and then calculates the module completion ratio (MCR) of each functional module to characterize the potential functionome in the user's own genomic and metagenomic data. In this study, we added two more useful functions to calculate module abundance and Q-value, which indicate the functional abundance and statistical significance of the MCR results, respectively, to the new version of MAPLE for more detailed comparative genomic and metagenomic analyses. Consequently, MAPLE version 2.1.0 reported significant differences in the potential functionome, functional abundance, and diversity of contributors to each function among four metagenomic datasets generated by the global ocean sampling expedition, one of the most popular environmental samples to use with this system. MAPLE version 2.1.0 is now available through the web interface (http://www.genome.jp/tools/maple/) 17 June 2016, date last accessed. © The Author 2016. Published by Oxford University Press on behalf of Kazusa DNA Research Institute.

Relative resistance or susceptibility of maple (Acer) species, hybrids and cultivars to six arthropod pests of production nurseries.

PubMed

Seagraves, Bonny L; Redmond, Carl T; Potter, Daniel A

2013-01-01

Maples (Acer spp.) in production nurseries are vulnerable to numerous arthropod pests that can stunt or even kill the young trees. Seventeen cultivars representing various Acer species and hybrids were evaluated for extent of infestation or injury by shoot and trunk borers (Proteoteras aesculana, Chrysobothris femorata), potato leafhopper (Empoasca fabae), Japanese beetle (Popillia japonica), maple spider mite (Oligonychus aceris) and calico scale (Eulecanium cerasorum). Evaluations were done in replicated field plots in central and western Kentucky. All of the maples were susceptible, to varying degrees, to one or more key pest(s). Red maples (A. rubrum) were relatively vulnerable to potato leafhopper injury and borers but nearly free of Japanese beetle feeding and spider mites. Sugar maples sustained conspicuous Japanese beetle damage but had very low mite populations, whereas the opposite was true for Freeman maples (A. × freemanii). A. campestre was heavily infested by calico scale. Within each species or hybrid there were cultivar differences in degree of infestation or damage by particular pests. The results should help growers to focus pest management efforts on those plantings at greatest risk from particular pests, and to choose cultivars requiring fewer insecticide inputs to produce a quality tree. Copyright © 2012 Society of Chemical Industry.

A Monte-Carlo maplet for the study of the optical properties of biological tissues

NASA Astrophysics Data System (ADS)

Yip, Man Ho; Carvalho, M. J.

2007-12-01

Monte-Carlo simulations are commonly used to study complex physical processes in various fields of physics. In this paper we present a Maple program intended for Monte-Carlo simulations of photon transport in biological tissues. The program has been designed so that the input data and output display can be handled by a maplet (an easy and user-friendly graphical interface), named the MonteCarloMaplet. A thorough explanation of the programming steps and how to use the maplet is given. Results obtained with the Maple program are compared with corresponding results available in the literature. Program summaryProgram title:MonteCarloMaplet Catalogue identifier:ADZU_v1_0 Program summary URL:http://cpc.cs.qub.ac.uk/summaries/ADZU_v1_0.html Program obtainable from: CPC Program Library, Queen's University, Belfast, N. Ireland Licensing provisions: Standard CPC licence, http://cpc.cs.qub.ac.uk/licence/licence.html No. of lines in distributed program, including test data, etc.:3251 No. of bytes in distributed program, including test data, etc.:296 465 Distribution format: tar.gz Programming language:Maple 10 Computer: Acer Aspire 5610 (any running Maple 10) Operating system: Windows XP professional (any running Maple 10) Classification: 3.1, 5 Nature of problem: Simulate the transport of radiation in biological tissues. Solution method: The Maple program follows the steps of the C program of L. Wang et al. [L. Wang, S.L. Jacques, L. Zheng, Computer Methods and Programs in Biomedicine 47 (1995) 131-146]; The Maple library routine for random number generation is used [Maple 10 User Manual c Maplesoft, a division of Waterloo Maple Inc., 2005]. Restrictions: Running time increases rapidly with the number of photons used in the simulation. Unusual features: A maplet (graphical user interface) has been programmed for data input and output. Note that the Monte-Carlo simulation was programmed with Maple 10. If attempting to run the simulation with an earlier version of Maple, appropriate modifications (regarding typesetting fonts) are required and once effected the worksheet runs without problem. However some of the windows of the maplet may still appear distorted. Running time: Depends essentially on the number of photons used in the simulation. Elapsed times for particular runs are reported in the main text.

Study of Polymer Crystallization by Physical Vapor Deposition

NASA Astrophysics Data System (ADS)

Jeong, Hyuncheol

When a polymer is confined under the submicron length scale, confinement size and interfaces can significantly impact the crystallization kinetics and resulting morphology. The ability to tune the morphology of confined polymer systems is of critical importance for the development of high-performance polymer microelectronics. The wisdom from the research on confined crystallization suggests that it would be beneficial to have a processing route in which the crystallization of polymers is driven by interface and temperature effects at a nanometer-scale confinement. In practice, for atomic and small-molecular systems, physical vapor deposition (PVD) has been recognized as the most successful processing route for the precise control of the film structure at surface utilizing confinement effects. While standard PVD technologies are not generally applicable to the deposition of the chemically fragile macromolecules, the development of matrix-assisted pulsed laser evaporation (MAPLE) now enables the non-destructive PVD of high-molecular weight polymers. In this thesis work, we investigated the use of MAPLE for the precise control of the crystallization of polymer films at a molecular level. We also sought to decipher the rules governing the crystallization of confined polymers, by using MAPLE as a tool to form confined polymer systems onto substrates with a controlled temperature. We first explored the early stages of film growth and crystallization of poly(ethylene oxide) (PEO) at the substrate surface formed by MAPLE. The unique mechanism of film formation in MAPLE, the deposition of submicron-sized polymer droplets, allowed for the manifestation of confinement and substrate effects in the crystallization of MAPLE-deposited PEO. Furthermore, we also focused on the property of the amorphous PEO film formed by MAPLE, showing the dependence of polymer crystallization kinetics on the thermal history of the amorphous phase. Lastly, we probed how MAPLE processing affected the semi-crystalline structure in MAPLE-deposited polyethylene (PE) films. Depositing PE at various temperatures remarkably allowed for the tunability of the melting temperature and crystallinity of the PE films, thus manipulating the semi-crystalline structure. By comparing the structure of PE formed by different processing routes, i.e., MAPLE and melt-crystallization, we discussed how processing routes affect the development of semi-crystalline phase in polymer films.

The Abuse of Dextromethorphan-Based Cough Syrup: A Pilot Study of the Community of Waynesboro, Pennsylvania.

ERIC Educational Resources Information Center

Momodou, N. Darboe; And Others

1996-01-01

Discusses the emergence of a new type of abused drug, dextromethorphan, which is used in cough syrup. Presents the results of the first phase of a comprehensive investigation of this phenomenon in the Waynesboro, Pa. school district. Survey data indicate abuse of cough syrup has increased over the years and is increasingly perceived as a problem…

Agribusiness Industry

DTIC Science & Technology

2007-01-01

corn supply has been used as animal feed and to produce high fructose corn syrup . In 2007, 25% of the US corn harvest is expected to be... high fructose corn syrup for existing corn . Thus, higher prices for corn caused by the ethanol demand is causing a rise in cost for high fructose ... corn syrup and animal feed, driving up consumer prices for chicken, pork, beef, and products, such as soft drinks, made

Dietary Supplements and Military Divers: A Synopsis for Undersea Medical Officers

DTIC Science & Technology

2004-01-01

High Fructose Corn Syrup Na: 55 mg K: 55 mg Niacinamide, Calcium Pantothenate... Fructose Na: 120 mg K: 20 mg None Hydrade 55 10 High Fructose Corn Syrup Na: 91 mg K: 77 mg Vitamin C, Glycerol, Metabolol Endurance 133 16 Maltodextrin...sports bars are exceptional for maintaining performance standards over a longer time period. Powerade 72 19 High Fructose , Corn Syrup ,

New Biocatalysts: Essential Tools for a Sustainable 21st Century Chemical Industry

DTIC Science & Technology

2005-01-01

ethanol, high fructose corn syrup , citric acid, and amino acids also employ microbes or en- zymes. However, the inroads into commodity chemical...for manufacture of aspartame, and is illustrative of two types of biocatalyst selectivity: chemical and stereoselectivity. High - fructose corn syrup ...Current applications of biocatalysts include the production of high fruc- tose corn syrup , aspartame, semi-synthetic penicillins and award-winning cancer

Pharmacokinetics of promethazine hydrochloride after administration of rectal suppositories and oral syrup to healthy subjects.

PubMed

Strenkoski-Nix, L C; Ermer, J; DeCleene, S; Cevallos, W; Mayer, P R

2000-08-15

The pharmacokinetics of promethazine hydrochloride after administration of rectal suppositories at three dosage strengths and oral syrup were studied. The study had an open-label, randomized, crossover design. At intervals of five to nine days, healthy volunteers were given two 12.5-mg promethazine rectal suppositories, one 25-mg suppository, one 50-mg suppository, or 50 mg (10 mL) of promethazine oral syrup. Blood samples were collected before each dose and at intervals from 0.5 to 48 hours afterward. Promethazine concentration was determined by high-performance liquid chromatography, and pharmacokinetic values were calculated with noncompartmental methods. Thirty-six subjects (18 men and 18 women) completed the study. Absorption was highly variable for all the formulations. On average, absorption was more rapid and the maximum plasma concentration (Cmax) higher for the syrup than for the suppositories. Cmax was significantly lower for the 50-mg suppository (mean, 9.04 ng/mL) than for the syrup (19.3 ng/mL). The time to Cmax (tmax) was significantly shorter for the syrup (mean, 4.4 hours) than for the suppositories (6.7-8.6 hours). There were no significant differences in dose-normalized Cmax among the three suppository treatments. Area under the concentration-versus-time curve (AUC) was comparable between the syrup and the 50-mg suppository and between the treatments with two 12.5-mg suppositories and the 25-mg suppository. Elimination profiles were similar among all treatments (mean half-life [t1/2], 16-19 hours). There were no significant differences in pharmacokinetics on the basis of sex or race. The mean relative bioavailability for the three suppository treatments ranged from 70% to 97%. Individual relative bioavailabilities ranged from 4% to 343%. The pharmacokinetics of promethazine administered in oral syrup and rectal suppositories were highly variable, but, in general, the suppositories produced a lower Cmax and later tmax than the syrup. All formulations were comparable in terms of dose-normalized AUC and t1/2, and the three suppository treatments were comparable in terms of dose-normalized Cmax.

Monitoring the health of sugar maple, Acer saccharum

NASA Astrophysics Data System (ADS)

Carlson, Martha

The sugar maple, Acer saccharum, is projected to decline and die in 88 to 100 percent of its current range in the United States. An iconic symbol of the northeastern temperate forest and a dominant species in this forest, the sugar maple is identified as the most sensitive tree in its ecosystem to rising temperatures and a warming climate. This study measures the health of sugar maples on 12 privately owned forests and at three schools in New Hampshire. Laboratory quantitative analyses of leaves, buds and sap as well as qualitative measures of leaf and bud indicate that record high beat in 2012 stressed the sugar maple. The study identifies several laboratory and qualitative tests of health which seem most sensitive and capable of identifying stress early when intervention in forest management or public policy change might counter decline of the species. The study presents evidence of an unusual atmospheric pollution event which defoliated sugar maples in 2010. The study examines the work of citizen scientists in Forest Watch, a K-12 school program in which students monitor the impacts of ozone on white pine, Pinus strobus, another keystone species in New Hampshire's forest. Finally, the study examines three simple measurements of bud, leaf and the tree's acclimation to light. The findings of these tests illuminate findings in the first study. And they present examples of what citizen scientists might contribute to long-term monitoring of maples. A partnership between science and citizens is proposed to begin long-term monitoring and to report on the health of sugar maples.

7 CFR 1437.107 - Maple sap.

Code of Federal Regulations, 2010 CFR

2010-01-01

... produced from trees that: (1) Are located on land the producer controls by ownership or lease; (2) Are... a maximum of 4 taps per tree according to the tree's diameter. (b) The crop year for maple sap... coverage in accordance with § 1437.6, tree acreage containing trees from which maple sap is produced or is...

Controlling taphole depth in maple sap production research

Treesearch

Melvin R. Koelling; Barton M. Blum

1967-01-01

Because bark thickness of sugar maple trees varies considerably, the depth of tapholes for collecting maple sap should be varied accordingly to get the taphole depth that will produce the best sap flow. A system of removable collars on the drill bit is recommended as a means of regulating taphole depth in research studies.

HOW to Identify and Control Sugar Maple Borer

Treesearch

William H. Hoffard; Philip T. Marshall

1978-01-01

The sugar maple borer, Glycobius speciosus (Say), a long-horned wood boring beetle, is a common pest of sugar maple (the only known host) throughout the range of the tree. Although borer-caused mortality is rare, infestations lead to value loss through lumber defect caused by larval galleries, discoloration, decay, and twisted grain.

Effect of manganese on endomycorrhizal sugar maple seedlings

Treesearch

George A. Schier; Carolyn J. McQuattie

2002-01-01

Manganese (Mn) toxicity may play an important role in the poor survival of seedlings in declining sugar maple (Acer saccharum Marsh.) stands in northern Pennsylvania. To determine the effect of Mn on the growth of sugar maple seedlings, 1-year-old seedlings inoculated with vesicular-arbuscular mycorrhizal (VAM) fungi and growing in sand-vermiculite-...

Sugar maple: its characteristics and potentials

Treesearch

Ralph D. Nyland

1999-01-01

Sugar maple dominates the northern hardwood forest, but grows over a broader geographic area. Conditions of soil and climate largely limit its distribution, and account for its less continuous cover along fringes of the range. Sugar maple regenerates readily following a wide range of overstory treatments. Success depends upon its status as advance regeneration,...

Factors associated with the decline disease of sugar maple on the Allegheny Plateau

Treesearch

Stephen B. Horsley; Robert P. Long; Scott W. Bailey; Richard A. Hallett; Thomas J. Hall

2000-01-01

Mortality of sugar maple (Acer saccharum Marsh.) has reached unusually high levels across northern Pennsylvania since the early to mid-1980s. We evaluated the influence of glaciation, topographic position, foliage chemistry, defoliation history, and stand characteristics (species composition, structure, density) on the health of sugar maple in 43...

Infrared and colorimetric characterization of discolored kiln-dried hard maple lumber

Treesearch

Benjamin E. Dawson-Andoh; Michael Wiemann; Laurent Matuana; John Baumgras

2004-01-01

Discoloration of hard maple lumber commonly occurs during kiln-drying. In this study, discolored andnondiscolored kiln-dried hard maple lumber boards were characterized using a colorimetric method and Attenuated Total Reflectance-Fourier Transform Infrared Spectroscopy (ATR-FTIR). Colorimetric measurements (L*, a*, b*) were found to be in good agreement with visual...

Decay in yellow-poplar, maple, black gum, and ash in the central hardwood region

Treesearch

Frederick H. Berry

1977-01-01

In a study of decay in yellow-poplar (Liriodendron tulipifera L.), red maple (Acer rubrum L.), sugar maple (Acer saccharum Marsh.), black gum (Nyssa sylvatica Marsh.), and ash (Fraxinus spp.) in the central hardwood region, decay was found in 57 of 148 study trees. Extent of...

Comparison of oak and sugar maple distribution and regeneration in central Illinois upland oak forests

Treesearch

Peter J. Frey; Scott J. Meiners

2014-01-01

Changes in disturbance frequencies, habitat fragmentation, and other biotic pressures are allowing sugar maple (Acer saccharum) to displace oak (Quercus spp.) in the upland forest understory. The displacement of oaks by sugar maples represents a major management concern throughout the region. We collected seedling microhabitat data...

Preliminary silvicultural recommendations and an updated annotated bibliography for birdseye sugar maple

Treesearch

Don C. Bragg

2008-01-01

The birdseye grain abnormality in sugar maple can greatly enhance its commercial appeal. However, birdseye has been opportunistically exploited, without exploring management strategies that can improve its potential. Even though the initiation and development processes of birdseye maple are still largely unknown, useful silvicultural advice can still be provided for...

7 CFR 1437.107 - Maple sap.

Code of Federal Regulations, 2012 CFR

2012-01-01

... a maximum of 4 taps per tree according to the tree's diameter. (b) The crop year for maple sap... produced from trees that: (1) Are located on land the producer controls by ownership or lease; (2) Are... coverage in accordance with § 1437.6, tree acreage containing trees from which maple sap is produced or is...

7 CFR 1437.107 - Maple sap.

Code of Federal Regulations, 2013 CFR

2013-01-01

... a maximum of 4 taps per tree according to the tree's diameter. (b) The crop year for maple sap... produced from trees that: (1) Are located on land the producer controls by ownership or lease; (2) Are... coverage in accordance with § 1437.6, tree acreage containing trees from which maple sap is produced or is...

RIR-MAPLE deposition of conjugated polymers and hybrid nanocomposites for application to optoelectronic devices

DOE Office of Scientific and Technical Information (OSTI.GOV)

Stiff-Roberts, Adrienne D.; Pate, Ryan; McCormick, Ryan

2012-07-30

Resonant infrared matrix-assisted pulsed laser evaporation (RIR-MAPLE) is a variation of pulsed laser deposition that is useful for organic-based thin films because it reduces material degradation by selective absorption of infrared radiation in the host matrix. A unique emulsion-based RIR-MAPLE approach has been developed that reduces substrate exposure to solvents and provides controlled and repeatable organic thin film deposition. In order to establish emulsion-based RIR-MAPLE as a preferred deposition technique for conjugated polymer or hybrid nanocomposite optoelectronic devices, studies have been conducted to demonstrate the value added by the approach in comparison to traditional solution-based deposition techniques, and this workmore » will be reviewed. The control of hybrid nanocomposite thin film deposition, and the photoconductivity in such materials deposited using emulsion-based RIR-MAPLE, will also be reviewed. The overall result of these studies is the demonstration of emulsion-based RIR-MAPLE as a viable option for the fabrication of conjugated polymer and hybrid nanocomposite optoelectronic devices that could yield improved device performance.« less

MAPLE deposition of nanomaterials

NASA Astrophysics Data System (ADS)

Caricato, A. P.; Arima, V.; Catalano, M.; Cesaria, M.; Cozzoli, P. D.; Martino, M.; Taurino, A.; Rella, R.; Scarfiello, R.; Tunno, T.; Zacheo, A.

2014-05-01

The matrix-assisted pulsed laser evaporation (MAPLE) has been recently exploited for depositing films of nanomaterials by combining the advantages of colloidal inorganic nanoparticles and laser-based techniques. MAPLE-deposition of nanomaterials meeting applicative purposes demands their peculiar properties to be taken into account while planning depositions to guarantee a congruent transfer (in terms of crystal structure and geometric features) and explain the deposition outcome. In particular, since nanofluids can enhance thermal conductivity with respect to conventional fluids, laser-induced heating can induce different ablation thermal regimes as compared to the MAPLE-treatment of soft materials. Moreover, nanoparticles exhibit lower melting temperatures and can experience pre-melting phenomena as compared to their bulk counterparts, which could easily induce shape and or crystal phase modification of the material to be deposited even at very low fluences. In this complex scenario, this review paper focuses on examples of MAPLE-depositions of size and shape controlled nanoparticles for different applications highlights advantages and challenges of the MAPLE-technique. The influence of the deposition parameters on the physical mechanisms which govern the deposition process is discussed.

Molecular weight dependent structure and charge transport in MAPLE-deposited poly(3-hexylthiophene) thin films

DOE PAGES

Dong, Ban Xuan; Smith, Mitchell; Strzalka, Joseph; ...

2018-02-06

In this work, poly(3-hexylthiophene) (P3HT) films prepared using the matrix-assisted pulsed laser evaporation (MAPLE) technique are shown to possess morphological structures that are dependent on molecular weight (MW). Specifically, the structures of low MW samples of MAPLE-deposited film are composed of crystallites/aggregates embedded within highly disordered environments, whereas those of high MW samples are composed of aggregated domains connected by long polymer chains. Additionally, the crystallite size along the side-chain (100) direction decreases, whereas the conjugation length increases with increasing molecular weight. This is qualitatively similar to the structure of spin-cast films, though the MAPLE-deposited films are more disordered. In-planemore » carrier mobilities in the MAPLE-deposited samples increase with MW, consistent with the notion that longer chains bridge adjacent aggregated domains thereby facilitating more effective charge transport. The carrier mobilities in the MAPLE-deposited simples are consistently lower than those in the solvent-cast samples for all molecular weights, consistent with the shorter conjugation length in samples prepared by this deposition technique.« less

Molecular weight dependent structure and charge transport in MAPLE-deposited poly(3-hexylthiophene) thin films

DOE Office of Scientific and Technical Information (OSTI.GOV)

Dong, Ban Xuan; Smith, Mitchell; Strzalka, Joseph

In this work, poly(3-hexylthiophene) (P3HT) films prepared using the matrix-assisted pulsed laser evaporation (MAPLE) technique are shown to possess morphological structures that are dependent on molecular weight (MW). Specifically, the structures of low MW samples of MAPLE-deposited film are composed of crystallites/aggregates embedded within highly disordered environments, whereas those of high MW samples are composed of aggregated domains connected by long polymer chains. Additionally, the crystallite size along the side-chain (100) direction decreases, whereas the conjugation length increases with increasing molecular weight. This is qualitatively similar to the structure of spin-cast films, though the MAPLE-deposited films are more disordered. In-planemore » carrier mobilities in the MAPLE-deposited samples increase with MW, consistent with the notion that longer chains bridge adjacent aggregated domains thereby facilitating more effective charge transport. The carrier mobilities in the MAPLE-deposited simples are consistently lower than those in the solvent-cast samples for all molecular weights, consistent with the shorter conjugation length in samples prepared by this deposition technique.« less

Quantification of metal loading to Silver Creek through the Silver Maple Claims area, Park City, Utah, May 2002

USGS Publications Warehouse

Kimball, Briant A.; Johnson, Kevin K.; Runkel, Robert L.; Steiger, Judy I.

2004-01-01

The Silver Maple Claims area along Silver Creek, near Park City, Utah, is administered by the Bureau of Land Management. To quantify possible sources of elevated zinc concentrations in Silver Creek that exceed water-quality standards, the U.S. Geological Survey conducted a mass-loading study in May 2002 along a 1,400-meter reach of Silver Creek that included the Silver Maple Claims area. Additional samples were collected upstream and downstream from the injection reach to investigate other possible sources of zinc and other metals to the stream. Many metals were investigated in the study, but zinc is of particular concern for water-quality standards. The total loading of zinc along the study reach from Park City to Wanship, Utah, was about 49 kilograms per day. The Silver Maple Claims area contributed about 38 percent of this load. The Silver Creek tailings discharge pipe, which empties just inside the Silver Maple Claims area, contributed more than half the load of the Silver Maple Claims area. Substantial zinc loads also were added to Silver Creek downstream from the Silver Maple Claims area. Ground-water discharge upstream from the waste-water treatment plant contributed 20 percent of the total zinc load, and another 17 percent was contributed near the waste-water treatment plant. By identifying the specific areas where zinc and other metal loads are contributed to Silver Creek, it is possible to assess the needs of a remediation plan. For example, removing the tailings from the Silver Maple Claims area could contribute to lowering the zinc concentration in Silver Creek, but without also addressing the loading from the Silver Creek tailings discharge pipe and the ground-water discharge farther downstream, the zinc concentration could not be lowered enough to meet water-quality standards. Additional existing sources of zinc loading downstream from the Silver Maple Claims area could complicate the process of lowering zinc concentration to meet water-quality standards.

The effect of sea-water and fresh-water soaking on the quality of Eucheuma sp. syrup and pudding

NASA Astrophysics Data System (ADS)

Novianty, H.; Herandarudewi, S. M. C.

2018-04-01

Eucheuma alvarezii is one of marine commodity with great opportunities to be developed in Indonesia. This seaweed can be used as an additional material in cosmetic and pharmaceutical products or for syrup and pudding. Post-harvest technique conducted by the seaweed farmers will affects the quality of dried and processed products. The purpose of this study was to observe the effect of post harvest technique on the quality of dried seaweed and hedonic test (favorable test) of processed product (syrup and pudding). This study was conducted using descriptive method. The study compared dried, syrup, and puddings from two differents post-harvest technique, between salt and fresh-water draining products. The results showed that fresh-water draining technique obtained better quality results organoleptic test. The results showed that panelist prefered the fresh-water drained products of syrup and pudding. The hedonic scores were much higher for the fresh-water drained products in all three catagories of color, taste, and smell.

Ultrafiltration and thermal processing effects on Maillard reaction products and biological properties of date palm sap syrups (Phoenix dactylifera L.).

PubMed

Makhlouf-Gafsi, Ines; Krichen, Fatma; Mansour, Riadh Ben; Mokni, Abir; Sila, Assad; Bougatef, Ali; Blecker, Christophe; Attia, Hamadi; Besbes, Souhail

2018-08-01

The effect of ultrafiltration process and temperature concentration on MRPs content and antioxidant, antimicrobial and cytotoxic properties of date palm sap syrups were investigated. MRPs were analyzed by HPLC. Antioxidant activity was evaluated by reducing power and DPPH free radical and H 2 O 2 scavenging activities. Antimicrobial activity was evaluated by the agar disk diffusion method. In vitro cytotoxic activity was examined by cell proliferation assay. Date sap syrups displayed strong antioxidant activities which are correlated 5HMF and 2F contents. In addition, concentration at 100 °C, unlike ultrafiltration process, enhanced significantly the antioxidant activities sap syrups and total phenolic contents. The antimicrobial activities showed marked activity against S. enterica, P. aeruginosa, S. aureus, L. monocytogenes with an inhibition zone of 21, 34, 27 and 34 mm respectively. Cytotoxicity assays showed that sap syrups can inhibit the proliferation of HeLa cell lines at high concentration. Published by Elsevier Ltd.

U.S.-Mexico Economic Relations: Trends, Issues, and Implications

DTIC Science & Technology

2010-03-31

resolved a long-standing trade dispute in 2006 involving sugar and high fructose corn syrup . Mexico argued that the sugar side letter negotiated under...Research Service 23 complained that imports of high fructose corn syrup (HFCS) sweeteners from the United States constituted dumping, and it imposed anti...made with corn syrup sweeteners to aid the ailing domestic cane sugar industry, and subsequently extended the tax annually despite U.S. objections

Mexico-U.S. Relations: Issues for Congress

DTIC Science & Technology

2009-04-14

highways beyond the commercial zone. Sugar and High Fructose Corn Syrup The United States and Mexico resolved a long standing trade dispute involving...sugar and high fructose corn syrup in 2006. Mexico argued that the sugar side letter negotiated under NAFTA entitled it to ship net sugar surplus to...complained that imports of high fructose corn syrup (HFCS) sweeteners from the United States constituted dumping, and it imposed anti-dumping duties

Mexico-U.S. Relations: Issues for Congress

DTIC Science & Technology

2008-12-18

States and Mexico recently resolved a long standing trade dispute involving sugar and high fructose corn syrup . Mexico argued that the sugar side...sugar side letter limited Mexican shipments of sugar. Mexico also complained that imports of high fructose corn syrup (HFCS) sweeteners from the...Mexican Congress imposed a 20% tax on soft drinks made with corn syrup sweeteners to aid the ailing domestic cane sugar industry, and subsequently

Landmine Detection by Scatter Radiation Radiography.

DTIC Science & Technology

1987-07-02

and sucrose. The explosive substitute is solidified Karo TMLih Corn Syrup . While this material is not sucrose, it has similar elemental composition and...photon interaction characteristics. Upon heating, a portion of the fructose contained in the syrup is converted to sucrose. A number of test batches...solidified Karo TMLight Corn Syrup are each subjected to the four spectra: 80, 100, 150, and 200 KVp, each filtered by 4.75 mm of beryllium inherent

Dibasic Ammonium Phosphate Application Enhances Aromatic Compound Concentration in Bog Bilberry Syrup Wine.

PubMed

Wang, Shao-Yang; Li, Yi-Qing; Li, Teng; Yang, Hang-Yu; Ren, Jie; Zhang, Bo-Lin; Zhu, Bao-Qing

2016-12-29

A nitrogen deficiency always causes bog bilberry syrup wine to have a poor sensory feature. This study investigated the effect of nitrogen source addition on volatile compounds during bog bilberry syrup wine fermentation. The syrup was supplemented with 60, 90, 120 or 150 mg/L dibasic ammonium phosphate (DAP) before fermentation. Results showed that an increase of DAP amounts accelerated fermentation rate, increased alcohol content, and decreased sugar level. Total phenol and total flavonoid content were also enhanced with the increase of DAP amounts. A total of 91 volatile compounds were detected in the wine and their concentrations were significantly enhanced with the increase of DAP. Ethyl acetate, isoamyl acetate, phenethyl acetate, ethyl butanoate, ethyl hexanoate, ethyl octanoate, ethyl decanoate, isobutanol, isoamyl alcohol, levo -2,3-butanediol, 2-phenylethanol, meso -2,3-butanediol, isobutyric acid, hexanoic acid, and octanoic acid exhibited a significant increase of their odor activity value (OAV) with the increase of DAP amounts. Bog bilberry syrup wine possessed fruity, fatty, and caramel flavors as its major aroma, whereas a balsamic note was the least present. The increase of DAP amounts significantly improved the global aroma attributes, thereby indicating that DAP supplementation could promote wine fermentation performance and enhance the sensory quality of bog bilberry syrup wine.

Beet sugar syrup and molasses as low-cost feedstock for the enzymatic production of fructo-oligosaccharides.

PubMed

Ghazi, Iraj; Fernandez-Arrojo, Lucia; Gomez De Segura, Aranzazu; Alcalde, Miguel; Plou, Francisco J; Ballesteros, Antonio

2006-04-19

Sugar syrup and molasses from beet processing containing 620 and 570 mg/mL sucrose, respectively, were assayed as low-cost and available substrates for the enzymatic synthesis of fructo-oligosaccharides (FOSs). A commercial pectinase (Pectinex Ultra SP-L, from Aspergillus aculeatus) characterized by the presence of a transfructosylating activity was used as a biocatalyst. The FOS production increased when lowering the initial pH value of syrup (7.5) and molasses (8.9) to 5.5. Sugar syrup and molasses were diluted in order to reduce substrate viscosity; interestingly, the percentage of FOS with regards to total sugars remained almost constant, which indicated a high transferase-to-hydrolase ratio for this enzyme. Kinetics of FOS production was analyzed. Using approximately 10 U transfructosylating activity per g sucrose, the FOS concentration reached a maximum of 388 mg/mL after 30 h using syrup and 235 mg/mL in 65 h with molasses. These values corresponded to approximately 56 and 49% (w/w), respectively, of the total amount of carbohydrates in the mixture. The enzyme was also covalently immobilized on an epoxy-activated polymethacrylate-based polymer (Sepabeads EC-EP5). We found that immobilized Pectinex Ultra SP-L can be efficiently applied to the synthesis of FOS using syrup and molasses as substrates.

Palm tree syrup: nutritional composition of a natural edulcorant.

PubMed

Luis, G; Rubio, C; Gutiérrez, A J; Hernández, C; González-Weller, D; Revert, C; Castilla, A; Abreu, P; Hardisson, A

2012-01-01

Palm syrup is a typical product from the Canary Islands, traditionally produced from the sap of the tropical palm tree Phoenix canariensis. Its high caloric content has led to its increasing use as a health food supplement for athletes, children and elderly. Furthermore, demand for this natural syrup is continuously increasing due also to its medicinal uses in homeopathic medicine. Palm Tree syrup samples prepared with palm sap from primary producers in La Gomera island (Canary Islands, Spain) were analyzed for their nutritional composition (moisture, ash, sugars, fat, vitamins and minerals). 35 syrup samples from five different producing regions in La Gomera island were analyzed. High-performance liquid chromatography (HPLC) was used to determine sugars and vitamins and Flame Atomic Absorption Spectrophotometry (FAAS) was used to analyze the minerals. Major carbohydrates were sucrose (37.8%), glucose (9.50%) and fructose (4.80%), respectively. The presence of arabinose could not be confirmed. Niacin was the water-soluble vitamin with the highest concentration with an average content of 0.003%. Fat content was found to be under 0.20%. Potassium was the mineral with highest contents (0.45%). Results suggest that palm tree syrup can play an important role as a sugar and mineral source in human nutrition, suggesting that future applications for this product could be developed.

Turbulent flow through channels in a viscously deforming matrix

NASA Astrophysics Data System (ADS)

Meyer, Colin; Hewitt, Ian; Neufeld, Jerome

2017-11-01

Channels of liquid melt form within a surrounding solid matrix in a variety of natural settings, for example, lava tubes and water flow through glaciers. Channels of water on the underside of glaciers, known as Rothlisberger (R-) channels, are essential components of subglacial hydrologic systems and can control the rate of glacier sliding. Water flow through these channels is turbulent, and dissipation melts open the channel while viscous creep of the surrounding closes the channel leading to the possibility of a steady state. Here we present an analogous laboratory experiment for R-channels. We pump warm water from the bottom into a tank of corn syrup and a channel forms. The pressure is lower in the water than in the corn syrup, therefore the syrup creeps inward. At the same time, the water ablates the corn syrup through dissolution and shear erosion, which we measure by determining the change in height of the syrup column over the course of the experiment. We find that the creep closure is much stronger than turbulent ablation which leads to traveling solitary waves along the water-syrup interface. These waves or `magmons' have been previously observed in experiments and theory for laminar magma melt conduits. We compliment our experiments with numerical simulations. David Crighton Fellowship.

Characterization and optimization of schizophyllan production from date syrup.

PubMed

Jamshidian, Hajar; Shojaosadati, Seyed Abbas; Vilaplana, Francisco; Mousavi, Seyed Mohammad; Soudi, Mohammad Reza

2016-11-01

This study demonstrates the efficient utilization of low-cost agricultural substrates, particularly date syrup, by Schizophyllum commune ATCC 38548 for schizophyllan production. Initially, one factor-at-a-time method was used to find the best carbon and nitrogen sources for schizophyllan production. Subsequently, response surface methodology was employed to optimize the level of culture medium components to maximize substrate conversion yield and schizophyllan production in submerged culture. Maximum product yield (0.12g schizophyllan/g date syrup) and schizophyllan production (8.5g/l) were obtained at concentrations of date syrup and corn steep liquor, inoculum size and agitation rate at 7.02%w/v, 0.10%w/v, 7.68%v/v and 181rpm, respectively. Sugar composition analysis, FTIR, NMR and molar mass determination revealed the purity and molecular properties of recovered schizophyllan produced from date syrup as glycosidic linkage analysis showed three main schizophyllan characteristic peaks arising from the 3-linked, 3,6-linked and terminal glucose residues. Finally, process economic analysis suggested that use of date syrup and corn steep liquor as nutrients would result in approximately 6-fold reduction in cost of raw materials for schizophyllan production as compared to conventional carbon and nitrogen sources such as sucrose and malt extract. Copyright © 2016 Elsevier B.V. All rights reserved.

Sugar and Multivitamin Diet Effects on The Longevity and Mating Capacity of Laboratory-Reared Male Anopheline Mosquitoes.

PubMed

Phasomkusolsil, Siriporn; Pantuwatana, Kanchana; Tawong, Jaruwan; Khongtak, Weeraphan; Kertmanee, Yossasin; Monkanna, Nantaporn; Khaosanorh, Sakon; Wanja, Elizabeth W; Davidson, Silas A

2017-09-01

Successful mating by male mosquitoes is dependent on several factors, with sugar feeding being particularly important. The effect of ingested vitamins on adult male mosquitoes is poorly understood. This laboratory study used 3 anopheline species, Anopheles campestris, An. dirus, and An. sawadwongporni, to study the effect of sugar and vitamins on male longevity, copulation, and fecundity. Males were fed 1 of 5 diets containing different combinations of sugar and vitamins: 10% glucose, 10% sucrose, 10% multivitamin syrup, 10% multivitamin syrup + 10% glucose, and 10% multivitamin syrup + 10% sucrose. The longevity of males was measured for a period of 15 days. Forced mating was used to simulate copulation, and fecundity was measured by counting the number of eggs oviposited and the hatch rate of larvae. The longevity of An. campestris and An. dirus was greatest when fed a diet of 10% multivitamin syrup + 10% glucose, and the longevity of An. sawadwongporni was greatest when fed a diet of 10% multivitamin syrup + 10% sucrose. The 1st mating routinely produced the most viable eggs when males were mated with several females. The diet of 10% multivitamin syrup + 10% sucrose produced numerically greater egg production and larval emergence for all 3 species, although this was not always statistically significant due to variability and small sample size. These results indicate that the addition of multivitamin syrup to sucrose may produce healthier and more fit male anophelines. This has potential implications for increasing insectary operations and improving the fitness of laboratory-reared male mosquitoes that will be released for mosquito and disease-pathogen control studies.

Development of lotus root fermented sugar syrup as a functional food supplement/condiment and evaluation of its physicochemical, nutritional and microbiological properties.

PubMed

Shukla, Shruti; Park, Juyeon; Park, Jung Hyun; Lee, Jong Suk; Kim, Myunghee

2018-02-01

Lotus ( Nelumbo nucifera ) root has been used as an edible vegetable in East Asia for thousands of years. The present research was aimed to explore the physicochemical, nutritional and microbiological safety of lotus root fermented sugar syrup as a fermented food supplement or condiment for human health benefits. In this study, the physicochemical, nutritional and microbiological safety properties of lotus root syrup fermented with 57° Brix brown sugar at different time periods until 6 months (180 days) was investigated. There was a significant improvement as compared to 57° Brix brown sugar broth (as a control) in the total acceptability and physicochemical properties of lotus root sugar syrup samples such as pH and color improvement. The red color values of 180 days lotus root fermented sugar syrup samples were significantly enhanced (6.85 ± 0.58) when compared with the control (0.20 ± 0.15). In addition, the total protein content was increased from 8.27 ± 0.86 to 392.33 ± 7.19 μg/mL, along with the increase in fermentation time reaching to the level of consumption acceptability. All the lotus root fermented sugar syrup samples were subjected to microbiological analysis. It was found that the coliform, Bacillus cereus , Escherichia coli , Salmonella and Staphylococcus aureus counts were not detected in majority of the samples, confirming the high degree of hygiene processing of lotus root fermented sugar syrup samples for its use as a food supplement or condiment.

Identification of hydrolyzed inulin syrup and high-fructose corn syrup in apple juice by capillary gas chromatography: PVM 4:1999.

PubMed

Low, N H; McLaughlin, M A; Page, S W; Canas, B J; Brause, A R; Low, N H

2001-01-01

A peer-verified, gas chromatographic (GC) method is presented for the identification of hydrolyzed inulin syrup (HIS) and high-fructose corn syrup (HFCS) in apple juice. The procedure involves determining the Brix value of the apple juice or apple juice concentrate and preparing a dilution of the test sample to 5.5 degrees Brix. A 100 microL aliquot of the 5.5 degrees Brix test solution is then freeze-dried in a GC autosampler vial. The sugars in the freeze-dried residue are converted to trimethylsilyl derivatives, by the addition of an appropriate silylation reagent, and the vial is heated at 75 degrees C for 30 min. After derivatization, the solution is introduced into a gas chromatograph where the analytes are separated on a 30 m, 0.25 mm id DB-5 column. The method can use hydrogen, helium, or nitrogen as the carrier gas. The analytes and marker compounds are measured by use of a flame ionization detecone of the 2 syrups at 2 levels. Dilution was ascertained by the presence of retrograde sugar markers found in the 2 sugar syrups. All 3 laboratories involved in the study were able to identify the correct diluent in the blind, randomly coded, apple juice test portions. The levels of dilution in the test portions were 0, 6.9% (HIS), 16.0% (HIS), 8.1% (HFCS), and 17.0% (HFCS). No false positive results were reported. Quantitative conclusions can be drawn when the same syrup is used for dilution and as a reference standard.

Use of compounded dispersing media for extemporaneous pediatric syrups with candesartan cilexetil and valsartan.

PubMed

Musko, Monika; Sznitowska, Malgorzata

2014-12-01

Available tablets or capsules for adults are often used to prepare extemporaneously formulated medicines appropriate for children. The most acceptable drug forms in pediatric population are oral liquids and pharmacists use commercial dispersing media to compound syrups from an active substance or from tablets available on the market. In many countries ready-to-use dispersing media are not available or refunded, but pharmacists can use other compounded media, providing their compatibility and stability are proven. The aim of this study was to formulate and evaluate the stability of syrups with candesartan cilexetil (1 mg mL-1) and valsartan (4 mg mL-1) extemporaneously prepared using commercial tablets (Diovan® and Atacand®). The following three different suspending media, which could be easily made in a pharmacy, were investigated: V1 - with xanthan gum (0.5 %), V2 - the USP/NF vehicle for oral solution and V3 - the medium based on a simple sucrose syrup. The stability of preparations was studied during 35 days of storage in a dark place at controlled temperature of 25 and 4 °C. During the study, microscopic observation was carried out and pH, viscosity, and concentration of candesartan cilexetil and valsartan were analyzed. Syrups with valsartan prepared with V2 and V3 media were stable for 3 or 4 weeks when stored at 25 °C, while syrups with candesartan were stable for as long as 35 days. For syrups prepared using V1 medium, the 14-day expiry date was not achieved because of microbial deterioration.

The bitterness intensity of clarithromycin evaluated by a taste sensor.

PubMed

Tanigake, Atsu; Miyanaga, Yohko; Nakamura, Tomoko; Tsuji, Eriko; Matsuyama, Kenji; Kunitomo, Masaru; Uchida, Takahiro

2003-11-01

The purpose of this study was to evaluate the ability of a quantitative prediction method using a taste sensor to determine the bitterness of clarithromycin powder suspensions of various concentrations and of a commercial clarithromycin dry syrup product (Clarith dry syrup, Taisho Pharmaceutical Co., Ltd., Tokyo) containing aminoalkyl methacrylate polymer as a taste-masker. The bitterness of the clarithromycin dry syrup product dissolved in various beverages was also evaluated in gustatory sensation tests and using the taste sensor. In the sensor measurements, three variables were used to predict bitterness in single and multiple regression analysis: relative sensor output (R), the change of membrane potential caused by adsorption (CPA), and CPA/R ratio. The CPA values for channel 3 of the sensor predicted well the bitterness of clarithromycin powder suspensions and their filtered solutions. For Clarith dry syrup, the sensor output was small, suggesting that aminoalkyl methacrylate polymer was successful in almost complete masking of the bitter taste of the dry syrup product. When the bitterness intensities of mixtures of 1 g of Clarith dry syrup with 25 ml of water, coffee, tea, green tea, cocoa, milk, and a sports drink were examined, a good correlation was obtained between the results from human taste tests and the predicted values calculated on the basis of multiple regression analysis using CPA data from channel 4, and the CPA/R ratio from channel 3 of the taste sensor (r(2)=0.963, p<0.005). Co-administration of 1 g of Clarith dry syrup with an acidic sports drink was found to be the most bitter using either method.

Chromatographic separation of fructose from date syrup.

PubMed

Al Eid, Salah M

2006-01-01

The objective of this study is to provide a process for separating fructose from a mixture of sugars containing essentially fructose and glucose, obtained from date palm fruits. The extraction procedure of date syrup from fresh dates gave a yield of 86.5% solids after vacuum drying. A process for separating fructose from an aqueous solution of date syrup involved adding the date syrup solutions (20, 30 and 40% by weight) to a chromatographic column filled with Dowex polystyrene strong cation exchange gel matrix resin Ca2 + and divinylbenzene, a functional group, sulfonic acid, particle size 320 microm, with a flow rate of 0.025 and 0.05 bed volume/min, under 30 and 70 degrees C column temperature. After the date sugar solution batch, a calculated quantity of water was added to the column. Glucose was retained by the resin more weakly than fructose and proceeded faster into the water batch flowing ahead. Three fractions were collected: a glucose-rich fraction, a return fraction, and a fructose-rich fraction. The return fraction is based on when the peaks of fructose and glucose were reached, which could be determined by means of an analyzer (polarimeter) based on the property of glucose and fructose solutions to turn the polarization level of polarized light. A high yield of fructose is obtained at 70 degrees C column temperature with a flow rate of 0.025 bed volume/min and date syrup solution containing 40% sugar concentration. The low recovery by weight obtained using date syrup solutions having a sugar concentration of 20 and 30%, encourages the use of a concentration of 40%. However, with the 40% date syrup supply the average concentrations of glucose and fructose in the return fractions were more than 40%, which can be used for diluting the thick date syrup solution extracted from dates.

Sapstreak disease of sugar maple

Treesearch

James W. Walters

1992-01-01

Sapstreak is a fatal disease of sugar maple that usually enters the tree through basal trunk scars or root wounds. The disease most often affects large, wounded trees left after logging. The fungus causing sapstreak readily infects stumps or cut logs during the summer months. So, wounding sugar maples during this time will increase the potential for disease spread. In...

Monitoring the Health of Sugar Maple, "Acer Saccharum"

ERIC Educational Resources Information Center

Carlson, Martha

2013-01-01

The sugar maple, "Acer saccharum," is projected to decline and die in 88 to 100 percent of its current range in the United States. An iconic symbol of the northeastern temperate forest and a dominant species in this forest, the sugar maple is identified as the most sensitive tree in its ecosystem to rising temperatures and a warming…

Passive Maple-Seed Robotic Fliers for Education, Research and Entrepreneurship

ERIC Educational Resources Information Center

Aslam, D. M.; Abu-Ageel, A.; Alfatlawi, M.; Varney, M. W.; Thompson, C. M.; Aslam, S. K.

2014-01-01

As inspirations from flora and fauna have led to many advances in modern technology, the concept of drawing ideas from nature for design should be reflected in engineering education. This paper focuses on a maple-seed robotic flier (MRF) with various complexities, a robotic platform modeled after the samaras of maple or ash trees, to teach STEM…

History of sugar maple decline

Treesearch

David R. Houston

1999-01-01

Only a few episodes of sugar maple dieback or decline were recorded during the first half of the 20th Century. In contrast, the last 50 years have provided numerous reports of both urban and forest dieback/decline. In the late 1950s, a defoliation-triggered decline, termed maple blight, that occurred in Wisconsin prompted the first comprehensive, multidisciplinary...

Optimum drying temperature to prevent oxidative stain in soft maple

Treesearch

Michael C. Wiemann; Mark Knaebe; Scott A. Bowe

2011-01-01

The objective of this paper is to determine the kiln conditions necessary to avoid interior enzymatic oxidative discoloration in soft maple. Three drying chambers were designed and constructed to control temperature and relative humidity of maple test samples. The tests showed that drying as soon as possible after harvest at temperatures below 42°C will...

Characterization of Nanostructured Polymer Films

DTIC Science & Technology

2014-12-23

discovered that polymer films with exceptional thermal and kinetic stability could be formed by Matrix Assisted Pulsed Laser Evaporation ( MAPLE ) onto...thermal properties of amorphous polymer nanoglobules fabricated via Matrix-Assisted Pulsed Laser Deposition ( MAPLE ). We discovered that stability in... MAPLE , Glass Transition Temperature 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF ABSTRACT 18. NUMBER OF PAGES 19a. NAME OF RESPONSIBLE PERSON

Some effects of forest preservation

Treesearch

William B. Leak

1974-01-01

Long-term preservation (no cutting) of a deciduous forest stand in New Hampshire is leading toward stable populations of beech, sugar maple, striped maple, mountain maple, and hobblebush, coupled with a decline or complete disappearance of other woody species. The humus has stabilized at a depth no greater than that of cut stands. Nitrate discharge in the streams is...

Survival of striped maple following spring prescribed fires in pennsylvania

Treesearch

Patrick H. Brose; Gary W. Miller; Kurt W. Gottschalk

2007-01-01

Survival of striped maple (Acer pensylvanicum L.) was assessed after three spring prescribed fires in Pennsylvania mixed oak (Quercus spp.) stands. Portions of two stands were prescribe-burned in spring 2002 and the part of a third in spring 2004. Following the fires, each stand was divided into burned and unburned units. Striped maple sapling...

History and range of Norway maple

Treesearch

David J. Nowak; Rowan A. Rowntree

1990-01-01

The Norway maple, Acer platanoldes, was introduced into the United States about 1756 after being cultivated in England since 1683. This species remained in relative obscurity in the U.S. until the mid- to late-1800's, but is now one of the most popular urban trees. In certain regions of the United States, Norway maples dominate street tree...

MAPLE: reflected light from exoplanets with a 50-cm diameter stratospheric balloon telescope

NASA Astrophysics Data System (ADS)

Marois, Christian; Bradley, Colin; Pazder, John; Nash, Reston; Metchev, Stanimir; Grandmont, Frédéric; Maire, Anne-Lise; Belikov, Ruslan; Macintosh, Bruce; Currie, Thayne; Galicher, Raphaël.; Marchis, Franck; Mawet, Dimitri; Serabyn, Eugene; Steinbring, Eric

2014-08-01

Detecting light reflected from exoplanets by direct imaging is the next major milestone in the search for, and characterization of, an Earth twin. Due to the high-risk and cost associated with satellites and limitations imposed by the atmosphere for ground-based instruments, we propose a bottom-up approach to reach that ultimate goal with an endeavor named MAPLE. MAPLE first project is a stratospheric balloon experiment called MAPLE-50. MAPLE-50 consists of a 50 cm diameter off-axis telescope working in the near-UV. The advantages of the near-UV are a small inner working angle and an improved contrast for blue planets. Along with the sophisticated tracking system to mitigate balloon pointing errors, MAPLE-50 will have a deformable mirror, a vortex coronograph, and a self-coherent camera as a focal plane wavefront-sensor which employs an Electron Multiplying CCD (EMCCD) as the science detector. The EMCCD will allow photon counting at kHz rates, thereby closely tracking telescope and instrument-bench-induced aberrations as they evolve with time. In addition, the EMCCD will acquire the science data with almost no read noise penalty. To mitigate risk and lower costs, MAPLE-50 will at first have a single optical channel with a minimum of moving parts. The goal is to reach a few times 109 contrast in 25 h worth of flying time, allowing direct detection of Jovians around the nearest stars. Once the 50 cm infrastructure has been validated, the telescope diameter will then be increased to a 1.5 m diameter (MAPLE-150) to reach 1010 contrast and have the capability to image another Earth.

Loading Rates and Impacts of Substrate Delivery for Enhanced Anaerobic Bioremediation

DTIC Science & Technology

2010-02-01

High Fructose Corn Syrup Food service companies Viscous fluid at 60 to 80 percent by weight Dissolved in water. Slow-Release Substrate...as sulfur, sulfate, and metals that may be of potential concern. Higher grades of molasses or high fructose corn syrup (HFCS) may be used in...ubiquitous in processed foods and beverages. Many confuse pure “ fructose ” with “ high fructose corn syrup ,” a sweetener that never contains fructose

Mexico-U.S. Relations: Issues for Congress

DTIC Science & Technology

2009-05-13

zone. Sugar and High Fructose Corn Syrup The United States and Mexico resolved a long standing trade dispute involving sugar and high fructose ... high fructose corn syrup (HFCS) sweeteners from the United States constituted dumping, and it imposed anti-dumping duties for some time, until NAFTA... corn syrup in 2006. Mexico argued that the sugar side letter negotiated under NAFTA entitled it to ship net sugar surplus to the United States duty free

U.S.-Mexico Economic Relations: Trends, Issues, and Implications

DTIC Science & Technology

2012-01-25

States and Mexico resolved a long-standing trade dispute in 2006 involving sugar and high fructose corn syrup . Mexico argued that the sugar side letter...sugar side letter limited Mexican shipments of sugar. Mexico also complained that imports of high fructose corn syrup (HFCS) sweeteners from the United...Mexican Congress imposed a 20% tax on soft drinks made with corn syrup sweeteners to aid the ailing domestic cane sugar industry, and subsequently

In-situ Substrate Addition to Create Reactive Zones for Treatment of Chlorinated Aliphatic Hydrocarbons: Hanscom Air Force Base

DTIC Science & Technology

2003-04-04

processes in the subsurface. This substrate is typically molasses although these substrates can include high fructose corn syrup , whey, etc. (Suthersan...typically comprised of a carbohydrate such as molasses, whey, high fructose corn syrup , lactate, butyrate, or benzoate). Through periodic subsurface...this purpose; other carbohydrates such as high fructose corn syrup and whey can also be effective. This approach has been accepted by regulators and

Ray tissues as an indirect measure of relative sap-sugar concentration in sugar maple

Treesearch

Peter W. Garrett; Kenneth R. Dudzik; Kenneth R. Dudzik

1989-01-01

Attempts to correlate ray tissue as a percentage of total wood volume with sap-sugar concentrations of sugar maple progenies were unsuccessful. These results raise doubts about our ability to use a relatively constant value such as ray-tissue volume in a selection program designed to increase the sap-sugar concentration of sugar maple seedlings.

Chapter 8:Properties of red maple laminated veneer lumber

Treesearch

Robert J. Ross; Xiping Wang; Brian K. Brashaw; John W. Forsman; John R. Erickson

2005-01-01

The purpose of the work reported in this chapter was to examine the flexural properties of LVL manufactured from ultrasonically rated red maple veneer sheets. The specific objectives were: 1. to determine the flatwise and edgewise bending properties of ½ inch-(1.3-cm-) thick red maple LVL billets, and 2. to examine the relationship between nondestructive parameters of...

Influence of nutrition and stress on sugar maple at a regional scale

Treesearch

Richard A. Hallett; Scott W. Bailey; Stephen B. Horsley; Robert P. Long

2006-01-01

Sugar maple (Acer saccharum Marsh.) decline disease on the Allegheny Plateau (region 1) resulted in high levels of mortality during the 1990s. Sugar maple was predisposed to decline because of an imbalance in Mg, Ca, and Mn nutrition and incited to decline by repeated defoliation. We sampled 33 stands in New York, Vermont, and New Hampshire (region 2...

Exploring methods for prevention of oxidative stain in soft maple

Treesearch

Michael C. Wiemann; Richard D. Bergman; Mark Knaebe; Scott A. Bowe

2009-01-01

Interior gray enzymatic oxidative stain for white woods such as maple has plagued the wood industry for many years because methods that have been found to reduce stain are hard to scale up to industrial levels. We examined possible alternative treatments to eliminate stain in soft maple (Acer rubrum L.), and found that exposure to sulfur dioxide gas eliminates interior...

Maturation of sugar maple seed

Treesearch

Clayton M., Jr. Carl; Albert G., Jr. Snow; Albert G. Snow

1971-01-01

The seeds of a sugar maple tree (Acer saccharum Marsh.) do not mature at the same time every year. And different trees mature their seeds at different times. So time of year is not a reliable measure of when seeds are ripe. Better criteria are needed. In recent studies we have found that moisture content and color are the best criteria for judging when sugar maple...

Long-term impact of liming on growth and vigor of northern hardwoods

Treesearch

Robert P. Long; Stephen B. Horsley; Thomas J. Hall

2011-01-01

Sugar maple (Acer saccharum Marsh.) is a keystone species in the northern hardwood forest, and decline episodes have negatively affected the growth and health of sugar maple in portions of its range over the past 50+ years. Crown health, growth, survival, and flower and seed production of sugar maple were negatively affected by a widespread decline...

A sugar maple planting study in Vermont

Treesearch

Harry W. Yawney; Clayton M., Jr. Carl; Clayton M. Carl

1970-01-01

Past attempts to establish sugar maple (Acer saccharum Marsh.) by planting have generally met with little success. The failures have been blamed mainly on competition by other vegetation and on damage done by animals. Finding an effective way to establish sugar maple seedlings is a key part in the research being carried on in Vermont by the USDA Forest Service to...

Effects of defoliation and drought on root food reserves in sugar maple seedlings

Treesearch

Johnson Parker; Johnson Parker

1970-01-01

The artificial defoliation of sugar maple (Acer. saccharum Marsh.) can cause a marked decline in root food reserves, especially starch, and an increase in the levels of the reducing sugars, fructose and glucose. Defoliation can also bring on the dieback-decline syndrome in sugar maples (Parker and Houston 1968). Two experiments designed to examine this question were...

The Influence of Residual Stand Densities on Regeneration in Sugar Maple Stands

Treesearch

Carl H. Tubbs

1968-01-01

Studies of regeneration 2, 5, and 10 years after cutting mature and overmature sugar maple stands to several residual densities show that (1) sugar maple is still the predominant species under all stand densities (2) nearly all regeneration reaching larger size classes became established before cutting (3) heavier cuttings (30, 50, and 70 square feet) are more rapidly...