Acid beta-glucocerebrosidase (N-acylsphingosyl-1-O-beta-D-glucoside:glucohydrolase) is a lysosomal glycoprotein that catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide. Inadequate levels of this enzyme underly the pathophysiology of Gaucher's disease. Cerezyme (Genzyme Corporation, Cambridge, MA, USA) is a partially ...

... 5 1 Fatty Acids Synthesis 2 4 Glycolysis and Gluconeogenesis 4 6 Mitochondrial LC-Fatty Acid Beta-Oxidation 1 3 Molecular Function ...

Reversibly methylated aspartyl residues in human erythrocyte membrane proteins are shown to be in the "unnatural" D configuration. This is demonstrated by treatment of proteolytically derived aspartic acid beta-[3H]methyl ester with L- and D-amino-acid oxidases and by the resolution of diastereomeric L-leucyl dipeptides containing either L- or D-aspartic ...

Two new new diterpene glycosides, 13-[(2-O-(6-O-beta-D-glucopyranosyl)-beta-D-glucopyranosyl-beta-D-glucopyranosyl)oxy] kaur-16-en-18-oic acid beta-D-glucopyranosyl ester (1) and 13-[(2-O-beta-D-glucopyranosyl-3-O-beta-D-fructofuranosyl-beta-D-glucopyranosyl)oxy] kaur-16-en-18-oic acid beta-D-glucopyranosyl ester ...

Six green leafy vegetables and herbs - spinach, amaranth, bengal gram, cauliflower, mint, coriander and carrots - were analyzed for moisture, protein, ascorbic acid, beta-carotene, total iron, ionizable iron (as % of total iron) in vitro iron (% of total iron), copper, manganese and zinc. Moisture content of the leaves and carrots varied from 75.1 percent ...

Under the conditions of disynchronization by the manipulation of both the alternation of light and darkness and the availability and unavailability of food, circadian rhythms characterize the excretion of several amino acids by inbred LOU rats bearing an immunocytoma. Large amplitude rhythms can be demonstrated for urinary beta-aminoisobutyric acid, ...

From the commercial extract of the leaves of Stevia rebaudiana, two additional new diterpenoid glycosides were isolated and their structures were characterized as 13-[(2-O-beta-glucopyranosyl-3-O-beta-D-xylopyranosyl-beta-D-glucopyranosyl)oxy] ent-kaur-16-en-19-oic acid beta-D-glucopyranosyl ester (1) and ...

The biochemical mechanism of action of antimony (Sb) in pentavalent form complexed to gluconic acid (sodium stibogluconate)-the drug of choice for the leishmaniases-has been only slightly investigated. We recently reported that, in stibogluconate-exposed ...

The biochemical mechanisms of action of antimony (Sb) in pentavalent form complexed to gluconic acid (sodium stibogluconate)-the drug of choice for the leishmaniases-has been only slightly investigated. The authors recently reported that, in stibogluconat...

Fatty acids are a major fuel source used to sustain contractile function in heart and oxidative skeletal muscle. To meet the energy demands of these muscles, the uptake and beta-oxidation of fatty acids must be coordinately regulated in order to ensure an adequate, but not excessive, supply for mitochondrial beta-oxidation. However, imbalance between fatty acid uptake and beta-oxidation has the ...

Small-subunit rRNA sequences were obtained for two saturated fatty acid-{beta}-oxidizing syntrophic bacteria, Syntrophomonas sapovorans and Syntrophomonas wolfei LYB, and sequence analysis confirmed their classification as members of the family Syntrophomonadaceae. S.wolfei LYB was closely related to S.wolfei subsp. solfei, but S. sapovorans did not cluster with the other ...

A method for determining Th is outlined which can detect as little as 10 mu g of Th. The method comprises adding 3-(6sulfo-2-naphthylazo)-chromotropic acid ( BETA -SNADNS-6) to the buffered acidic Th solution, adding small increments of EDTA in steps, and measuring the absorbance at 590 m mu between steps. (D.L.C.)

for the observed result, we conducted analyses including betacarotene, retinol, vitamins BB 2, B6, B12, C, D, E supplements?'' with options for vitamins A, C, D, E, B group, folic acid, beta-carotene, and other vitamins, vitamin B12, vitamin C, vitamin D, vitamin E, and fiber. In the subanalyses excluding women who reported

The neurotoxic amino acids Beta-N-oxalylamino-L-alanine (BOAA) and Beta-N-methylamino-L-alanine (BMAA) were evaluated for possible effects on spontaneous and stimulus-evoked release of L-glutamate (L-Glu) and dynorphin A(1-8)-like immunoreactivity (LI) fr...

The isolation is reported of a coenzyme form of pseudovitamin /sub 12/ which is required fur the decomposition of glutamate by particle-free extracts of Clostridium tetranomorphum. The coenzyme is involved in the rearrangement of the carbon of glutamate to form a branched chain amino acid, beta -methylaspartate. (C. H.)

The paraherquamides are potent anthelmintic alkaloids isolated from various Penicillium sp. These substances have attracted considerable attention due to their molecular complexity, intriguing biogenesis, and potential as antiparasitic drugs. The most potent member of this family is paraherquamide A (1), which contains the unusual amino acid, ...

The concentrations of alpha-acids, beta-acids, desmethylxanthohumol, and xanthohumol were monitored in the hop varieties Admiral (A), Wye Challenger (WC), and First Gold (FG) during the harvest seasons of 2003 through 2005. Hops grown under an organic regimen were compared to plants grown conventionally in hop fields in close vicinity. The concentrations ...

All the nonprotein amino acids found in the Murchison meteorite are products of the action of electric discharge on a mixture of methane, nitrogen, and water with traces of ammonia. These amino acids include alpha-amino-n-butyric acid, alpha-aminoisobutyric acid, norvaline, isovaline, pipecolic acid, beta-alanine, beta-amino-n-butyric ...

The authors describe four new mutations in the [beta]-galactosidase gene. These are the first mutations causing infantile and juvenile GM[sub 1]-gangliosidosis to be described in American patients. Cell lines from two patients with juvenile and from six patients with infantile GM[sub 1]-gangliosidosis were analyzed. Northern blot analysis showed the acid ...

Several mouse models for mitochondrial fatty acid beta-oxidation (FAO) defects have been developed. So far, these models have contributed little to our current understanding of the pathophysiology. The objective of this study was to explore differences between murine and human FAO. Using a combination of analytical, biochemical and molecular methods, we ...

Important secondary metabolites, present in hops (Humulus lupulus L.), include alpha-acids and beta-acids, which are essential for the brewing of beer, as well as the prenylated chalcones, desmethylxanthohumol, and xanthohumol, which exhibit interesting bioactive properties. Their formation and accumulation in five selected hop varieties, Wye Challenger, Wye Target, Golding, Admiral, and Whitbread ...

Germinated spores of Bacillus megaterium QM B1551 were irradiated with ultraviolet light, and spore-forming survivors were screened for germination requirements. Spore strains which failed to germinate in a variety of defined solutions germinative for spores of the parent strain were obtained. Mutant spores germinated readily in solutions containing yeast extract or one of numerous complex ...

A new masking agent in complexometry, beta-dithiocarbaminopropionic acid (beta-DTCPA) is proposed. beta-DTCPA forms very stable water-soluble complexes with a number of metal ions. Zinc can be titrated selectively with EDTA at pH 5-6 in the presence of large amounts of cadmium, which is masked effectively as a cadmium-beta-DTCPA complex. Correct results ...

Thirteen constituents were identified from Girardinia palmata ssp ciliata C. J. Chen by using GC-MS, which amounted to 84.5% of the total peaks, the main constituent were linoleic acid (52.01%) and dimethyl fumarate (10.05%). The dissociated amino acids and KNO(3) in the sample amounted to 43.30 mg/100 g and 3.0%. Five compounds were isolated and elucidated as octadecane, hexadecylic ...

Phytochemical investigation of the seeds of Althea officinalis L. (Malvaceae) led to the isolation of three new phytoconstituents, identified as n-hexacos-2-enyl-1,5-olide (altheahexacosanyl lactone), 2beta-hydroxycalamene (altheacalamene) and 5,6-dihydroxycoumarin-5-dodecanoate-6beta-D-glucopyranoside (altheacoumarin glucoside), along with the known phytoconstituents lauric ...

A method for determining the beta-amino-isobutyric acid in urine using two-dimensional chromatography was developed. Previous observations indicate that the excretion rate of this amino acid would be related to exposure. A statistical survey was made on t...

We have used mice with inborn errors of mitochondrial fatty acid beta-oxidation to test the concept of synergistic heterozygosity. We postulated that clinical disease can result from heterozygous mutations in more than one gene in single or related metabolic pathways. Mice with combinations of mutations in mitochondrial fatty acid ...

Since very long-chain fatty acids with a chain length of 24 carbons or more are known to accumulate in tissues and body fluids from patients with the cerebrohepato-renal (Zellweger) syndrome, infantile Refsum disease, neonatal adrenoleukodystrophy and X-linked adrenoleukodystrophy, we studied very long-chain fatty acid oxidation in cultured skin fibroblasts from these patients. In this paper, we ...

The retinal pigment epithelium (RPE) and the choriocapillaris are affected early in the retinopathy associated with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency. RPE in culture possesses the machinery needed for mitochondrial fatty acid beta-oxidation in vitro. To further elucidate pathogenesis of LCHAD retinopathy, we performed ...

Human acid ..beta..-glucosidase (D-glucosyl-N-acylsphingosine glucohydrolase, EC 3.2.1.45) cleaves the glucosidic bonds of glucosylceramide and synthetic ..beta..-glucosides. The deficient activity of this hydrolase is the enzymatic defect in the subtypes and variants of Gaucher disease, the most prevalent lysosomal storage disease. To isolate and ...

Ralstonia eutropha has been considered as a bacterium, incorporating hydroxyalkanoates of less than six carbons only into polyhydroxyalkanoates (PHAs). Cells of the wild type cultivated with sodium octanoate as the carbon source in the presence of the fatty acid beta-oxidation inhibitor sodium acrylate synthesized PHAs composed of the medium chain length ...

Band 3, the anion transport protein of erythrocyte membranes, is a major methyl-accepting substrate of the intracellular erythrocyte protein carboxyl methyltransferase (S-adenosyl-L-methionine: protein-D-aspartate O-methyltransferase; EC 2.1.1.77). The localization of methylation sites in intact cells by analysis of proteolytic fragments indicated that sites were present in the cytoplasmic ...

Mitochondrial fatty acids beta-oxidation disorder (FAOD) has become popular with development of tandem mass spectrometry (MS/MS) and enzymatic evaluation techniques. FAOD occasionally causes acute encephalopathy or even sudden death in children. On the other hand, hyperpyrexia may also trigger severe seizures or encephalopathy, which might be caused by the ...

Gaucher disease is the most frequent lysosomal storage disease and the most prevalent Jewish genetic disease. About 30 identified missense mutations are causal to the defective activity of acid [beta]-glucosidase in this disease. cDNAs were characterized from a moderately affected 9-year-old Ashkenazi Jewish Gaucher disease type 1 patient whose ...

It has been established that cordialin inhibits selectively succinic acid oxidation and intensifies the exudation of a NADH2 substrate which is energetically more advantageous, and in this way has a favorable effect on energy metabolism, evidence of which is increase of the ATP content in the myocardium. Cordialin correction of the biologic energy of cardiomyocytes in hypoxia and ischemia prevents ...

Gaucher disease is a genetic disorder in which the enzyme activity of lysosomal acid beta-glucocerebrosidase is impaired, causing excessive lysosomal storage of glucocerebrosidase in monocytes and macrophages. Coagulation and multiorgan disorders can develop during pregnancy in women with this disorder, affecting anesthetic management. We report the case ...

The fadBA operon in the fatty acid beta-oxidation pathway of P. putida KCTC1639 was blocked to induce a metabolic flux of the intermediates to the biosynthesis of medium chain-length PHA (mcl-PHA). Succinate at 150 mg l(-1) stimulated cell growth and also the biosynthesis of medium chain-length-polyhydroxyalkanoate. pH-stat fed-batch cultivation of the ...

Two new monodesmosidic triterpene saponins were isolated from the roots of Gypsophila oldhamiana (Caryophyllaceae). Their structures were elucidated on the basis of spectral data to be quillaic acid, alpha-L-arabinopyranosyl-(1-->4)-alpha-L-arabinopyranosyl-(1-->3)-beta-D-xylopyranosyl-(1-->4)-alpha-L-rhamnopyranosyl-(1-->2)-beta-D-fucopyranosyl ester (1), and vaccaric ...

The porphyrin precursors beta-aminolevulinic acid (beta-ALA) and porphobilinogen (PBG) which accumulate, and are excreted in the urine in increased amounts during acute attacks of porphyria, were tested for their effects on reflex activity in the isolated hemisected spinal cords of Xenopus laevis. The two compounds were found to exert an inhibitory effect ...

Mutants of Escherichia coli defective in the regulation of the fatty acids beta oxidation pathway show an ultrastructural deficiency in septum formation at high growth rate. Several independent pairs of parent and mutant strains have been analyzed biochemically. Each parent strain displays a well-defined pattern of cellular phospholipids, which varies with ...

Acetoacetyl coenzyme A (acetoacetyl-CoA) thiolase, an enzyme required for short-chain fatty acid degradation, has been purified to near homogeneity from Caulobacter crescentus. The relative heat stability of this enzyme allowed it to be separated from beta-ketoacyl-CoA thiolase. The purification scheme minus the heating step also permitted the copurification of crotonase and 3-hydroxyacyl-CoA ...

Corynebacterium lepus was grown in 20-liter batch fermentations with kerosene as the sole carbon source. Critical micelle concentration measurements indicated the production of appreciable quantities of biosurfactants. This surface activity of the culture medium was due to lipids, which were extracted and identified. Samples of C. lepus whole broth were taken during a fermentation and monitored ...

The photochemistry of copper(II)-amino acid complexes (amino acid = glutamic acid, ..beta..-alanine, or glycine) has been investigated by continuous and flash photolysis. The charge-transfer irradiations induce the oxidation of the ligand and the reduction of copper(II) to copper(I). Transients observed in flash photolysis have been assigned as ...

Fanihi -- flying foxes (Pteropus mariannus mariannus, Pteropodidae) -- are a highly salient component of the traditional Chamorro diet. A neurotoxic, non-protein amino acid, beta-methylamino-l-alanine (BMAA) accumulates in flying foxes, which forage on the seeds of Cycas micronesica (Cycadaceae) in Guam's forests. BMAA occurs throughout flying fox tissues ...

We describe an unusually severe case of medium chain acyl-CoA dehydrogenase (MCAD) deficiency in a term female neonate, who presented at 12 h of age with lethargy, poor feeding, hypoglycemia and ventricular tachyarrhythmias. While arrhythmias are common in other disorders of fatty acid beta-oxidation, ventricular tachyarrhythmias have rarely been reported ...

Catalytic oxidation of various chelating agents in aqueous solutions, namely: diethylenetriaminepentakis(methylphosphonic acid) (DTPMP); diethylenediaminepentaacetic acid (DTPA); ethylenediamine tetraacetic acid (EDTA); nitrilotriacetic acid (NTA); and beta-alaninediacetic acid (beta-ADA) was studied. Hydrogen peroxide was used as oxidising agent in ...

Several canine cDNA libraries were screened with human {beta}-galactosidase cDNA as probe. Seven positive clones were isolated and sequenced yielding a partial (2060 bp) canine {beta}-galactosidase cDNA with 86% identity to the human {beta}-galactosidase cDNA. Preliminary analysis of a canine genomic library indicated conservation of exon number and size. Analysis by Northern blotting disclosed a ...

Recently, two studies were published that examined the structure of the acid-?-glucosidase N370S mutant, the most common mutant that causes Gaucher disease. One study used the experimental tool of X-ray crystallography, and the other utilized molecular dynamics (MD). The two studies reinforced each other through the similarities in their findings, but each approach also added some unique ...

Condensation of N6-benzoyl-2',3'-O-isopropylideneadenosine-5'-aldehyde with nitromethane followed by acid catalyzed acetylation and borohydride reduction leads to N6-benzoyl-9-(5,6-dideoxy-2,3-O-isopropylidene-6-nitro-beta-D-ribo-hexofuranosyl)adenine (4). A second nitroaldol condensation between 4 and N-benzyloxycarbonly-L-aspartic acid-beta-semialdehyde alpha-benzyl ester ...

The CH2Cl2/MeOH extract of the stem bark of Oriciopsis glaberrima ENGL. afforded four new acridone alkaloids namely oriciacridone C, D, E and F along with six known compounds: atalaphyllidine, oleanolic acid, butulinic acid, beta-sitosterol, stigmasterol, glucoside of stigmasterol and one synthetically known acridone: 1,3,5-trihydroxy-4-prenylacridone. The ...

Carnitine plays an essential role in fatty acid metabolism, as well as modulation of intracellular concentrations of free coenzyme A by esterification of acyl residues. Acylcarnitine analysis of various biological fluids is a sensitive method to detect >20 inborn errors of metabolism that result in abnormal accumulation of acylcarnitine species due to several organic acidemias and most fatty ...

Medium-chain acyl coenzyme A dehydrogenase (MCAD) deficiency is the most common of the inborn errors of mitochondrial fatty acid beta-oxidation. A male infant was born at 39 weeks of gestation following an uneventful pregnancy. He was discharged at age 28 h after a normal first-day check, but was subsequently re-admitted and died aged 44 h. Post-mortem ...

Mitochondrial fatty acid beta-oxidation plays a major role in providing the ATP required for reabsorptive processes in the adult rat kidney. However, the molecular mechanisms and signals involved in induction of the enzymes of fatty acid oxidation during development in this and other organs are unknown. We therefore studied the changes in the steady-state ...

Expression of recombinant human lysosomal acid beta-glucosidase (hGCase) by a transgenic animal bioreactor, using somatic cell nuclear transfer (SCNT), would decrease the cost of producing this product. The objective was to establish an effective procedure to prepare hGCase transgenic donor cells and nuclear transfer (NT) embryos to produce hGCase protein ...

In this study, we investigated whether the anti-inflammatory drug PP56 (alpha-trinositol) may improve cancer-induced metabolic disorders. We implanted human MiaPaCa2 pancreatic cancer cells in the pancreas of 14 athymic mice for 12 weeks, using six intact littermates as normal controls. During the 12 weeks, seven tumor-cell recipients were treated with PP56 by daily injection (PPT mice). The ...

Two purified isozymes of protein carboxyl methyltransferase from bovine brain catalyze the substoichiometric transfer of methyl groups in vitro from S-adenosyl-L-[methyl-3H]methionine to several erythrocyte membrane proteins, which include bands 2.1, 3, and 4.1, as well as several integral membrane polypeptides. D-Aspartic acid beta-[3H]methyl ester has ...

In response to cellular hypoxia, cardiomyocytes adapt to consume less oxygen by shifting ATP production from mitochondrial fatty acid {beta}-oxidation to glycolysis. The transcriptional activation of glucose transporters and glycolytic enzymes by hypoxia is mediated by hypoxia-inducible factor 1 (HIF-1). In this study, we examined whether HIF-1 was ...

Administration of hypolipidemic compounds leads to marked proliferation of peroxisomes and peroxisome-associated enzymes (PAE) in the livers of rodents and non-rodent species. The increase peroxisome-associated enzymes such as fatty acid ..beta..-oxidation system and catalase is shown to be due to an increase in the levels of mRNA. In this experiment they ...

Numerous mutations ({approximately}45) of the acid {beta}-glucosidase gene have been identified in patients with Gaucher disease. Many of these have been characterized by partial sequencing of cDNAs derived by RT-PCR or PCR of genomic DNA. In addition, genotype/phenotype correlations have been based on screening for known mutations. Thus, only a part of ...

Kinetic and immunologic techniques were developed to investigate the nature of the acid {beta}-glucosidase ({beta}-Glc) defects which results in human and canine Gaucher disease (GD). Two new affinity columns, using the potent inhibitors of {beta}-Glc (N-alkyl-deoxynojirimycins) as affinity ligands, were synthesized and methods were developed to obtain ...

Peroxisomal malate dehydrogenase (PMDH) oxidises NADH produced by fatty acid beta-oxidation during seed germination and seedling growth. Arabidopsis thaliana beta-oxidation mutants exhibit seed dormancy or impaired seed germination and failure of seedlings to degrade triacylglycerol (TAG), but the pmdh1 pmdh2 null mutant germinates readily and degrades TAG ...

Sediment trap moorings deployed in three distinct oceanographic zones along the equator in the western Pacific Ocean during 1991-93, 1994-95 and 1999 provided time series data on total mass and amino acid fluxes and composition of settling particulate organic matter (POM). The traps were deployed at shallow (970-1769 m) and deep (2060-4574 m) water depths, where seafloor depth ranged from 3181 to ...

Two overlapping cDNA clones (1,991 bp and 736 bp, respectively) encoding the precursor of human mitochondrial very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) were cloned and sequenced. The cDNA inserts of these clones together encompass a region of 2,177 bases, encoding the entire protein of 655 amino acids, including a 40-amino acid leader peptide and a 615-amino acid mature polypeptide. ...

Gaucher disease, the most common lysosomal storage disease, can be treated with enzyme replacement therapy (ERT), in which defective acid-beta-glucosidase (GlcCerase) is supplemented by a recombinant, active enzyme. The X-ray structures of recombinant GlcCerase produced in Chinese hamster ovary cells (imiglucerase, Cerezyme) and in transgenic carrot cells (prGCD) have been ...

The acyl-CoA dehydrogenases are a family of multimeric flavoenzymes that catalyze the alpha,beta -dehydrogenation of acyl-CoA esters in fatty acid beta -oxidation and amino acid catabolism. Genetic defects have been identified in most of the acyl-CoA dehydrogenases in humans. Acyl-CoA dehydrogenase 9 (ACAD9) is a recently identified acyl-CoA dehydrogenase ...

Trichosporon cutaneum, when grown with p-cresol, catalyzed intradiol fission of the benzene nucleus of 4-methylcatechol before the complete catabolism of these two substrates. Steps in their conversion to pyruvate and acetyl coenzyme A were investigated by using cell extracts, and some properties of various new microbial catabolites are also described. These included ...

This paper studied the effects of Lantana camera fresh leaves aqueous extract and its contained phenolic compounds on the growth and physiologic-biochemical indexes of Eichhornia crassipes. The results showed that this extract had obvious inhibitory effects on the growth and development of E. crassipes. When the concentration was higher than 30 g FW x L(-1), it could kill E. crassipes after 6 days ...

1. The structural requirements for activation of the glycine receptor were studied in isolated ventromedial hypothalamic neurones of rats by use of a 'concentration-clamp' technique under single-electrode voltage-clamp conditions. 2. alpha-Amino acids (L-alpha-alanine, and D-alpha-alanine, and L-serine), and glycine-methylester, glycine-ethylester and beta-amino acids ...

This work shows that the neurotoxic excitatory amino acids beta-N-methylamino alanine, BMAA, and kainate, modulate neurite outgrowth; this was assessed by measuring the levels of two separate neurofilament proteins (68 kD and 160 kD), in a mouse neuroblastoma cell line, (NB41A3). BMAA has been proposed to be the exogenous excitotoxin in Guam disease or ...

Although some important biomarkers for myocardial injury have been identified, there still lacks a systematic view of the development and progression of myocardial infarction, including enzymatic regulation, metabolite levels, fluxes, etc., which are pivotal to elucidate the physiological mechanism of disease. Here we present an untargeted analytical approach based on gas chromatography coupled ...

The first total synthesis of the cyclodepsipeptide chondramide A (2 b) is described. This depsipeptide is composed of four subunits, namely L-alanine, N-Me-D-tryptophan, 3-amino-2-methoxy-propionic acid (beta-tyrosine derivative), and a 7-hydroxy-alkenoic acid. While the configuration of the stereogenic centers in the 7-hydroxy-alkenoic acid were known, ...

A study was conducted on the effects of 12-O-tetradecanoyl-phorbol-13-acetate (TPA) on peroxisomal enzyme activities in mouse embryo fibroblasts C3H/10T1/2 C18 cells and chemically transformed C3H/10T1/2 MCA16 cells. TPA is a potent tumour promoter and treatment with this compound of the two cell lines induced peroxisomal fatty acid beta-oxidation, ...

Mitochondrial superoxide production is an important source of reactive oxygen species in cells, and may cause or contribute to ageing and the diseases of ageing. Seven major sites of superoxide production in mammalian mitochondria are known and widely accepted. In descending order of maximum capacity they are the ubiquinone binding sites in complex I (site IQ) and complex III (site IIIQo), ...

The safe use of grasspea (Lathyrus sativus) and allied species (L. cicera, L. clymenum and L. ochrus) requires a better understanding of the factors that are involved in the development of neurolathyrism. A suitable animal model is needed. The nutritional quality, seed chemical composition, the role of malnutrition, synergistic action of antinutritional factors, the toxicity of both seed and ...

The underground caudex of the cycad Stangeria eriopus is used extensively by several ethnic groups in South Africa, mainly as an ingredient in magical potions but also as an emetic. An assessment of two main outlets showed that 3410 plants were sold in the month of July 1992; continued usage of this material now threatens the remaining plant populations. A proximate analysis of the caudex material ...

Oxidation of fatty acids in mitochondria is a key physiological process in higher eukaryotes including humans. The importance of the mitochondrial beta-oxidation system in humans is exemplified by the existence of a group of genetic diseases in man caused by an impairment in the mitochondrial oxidation of fatty acids. Identification of patients with a defect in mitochondrial beta-oxidation has ...

Plants have developed numerous mechanisms to store hormones in inactive but readily available states, enabling rapid responses to environmental changes. The phytohormone auxin has a number of storage precursors, including indole-3-butyric acid (IBA), which is apparently shortened to active indole-3-acetic acid (IAA) in peroxisomes by a process similar to fatty acid ...

The mechanism of the enzyme asparaginase (L-asparagine amidohydrolase, EC 3.5.1.1) from Escherichia coli was examined using 13C NMR spectroscopy. The pH-dependent oxygen exchange reactions between water and aspartic acid were followed by use of the 18O isotope-induced shift of the resonance positions of directly bonded 13C nuclei. Both L-1- and L-1,4-[13C]aspartic acid were used in experiments ...

The opioid agonists endomorphins (Tyr-Pro-Trp-Phe-NH(2); EM1 and Tyr-Pro-Phe-Phe-NH(2); EM2) and morphiceptin (Tyr-Pro-Phe-Pro-NH(2)) exhibit an extremely high selectivity for mu-opioid receptor. Here a series of novel EM2 and morphiceptin analogues containing in place of the proline at position 2 the S and R residues of beta-homologues of proline (HPro), of 2-pyrrolidinemethanesulphonic acid ...

The amino acid beta-alanine is an intermediate in pantothenic acid (vitamin B(5)) and coenzyme A (CoA) biosynthesis. In contrast to bacteria, yeast derive the beta-alanine required for pantothenic acid production via polyamine metabolism, mediated by the four SPE genes and by the FAD-dependent amine oxidase encoded by FMS1. Because amine oxidases generally ...

A new HPLC method with coulometric detection for the quantification of xanthohumol, alpha-acids and beta-acids in hops was developed. The separation of compounds was accomplished with a C18 column and isocratic elution with methanol: 50 mM potassium phosphate: ortho-phosphoric acid=80:20:0.25 (v/v/v). The method was validated and UV and electrochemical detectors (ECD) were compared. The HPLC ...

The non-protein amino acid, beta-N-methyl-amino-L-alanine (L-BMAA) from Cycas circinalis seeds, has been implicated as a causative agent of amyotrophic lateral sclerosis-Parkinsonism dementia complex (ALS-PDC), a disease known from Guam and other areas in the western Pacific. We analyzed C. circinalis seeds for additional free non-protein amino acids by a ...

Trichoderma sp. K9301 secreting endoxylanases with an activity of 2836 U/g (dry weight) was screened for XOs production. Two acidic beta-endoxylanases EX1 (30.1 kDa) and EX2 (20.1 kDa) were purified from crude extract of the strain K9301 in solid fermentation. Action modes of EX1 and EX2 towards XOs showed similar hydrolysis characters to endoxylanases ...

A new version of the single-reference-extended kinetic method is presented in which direct entropy correction is incorporated. Results of calibration experiments with the monodentate base pyridine and the bidentate base ethylenediamine are presented for which the method provides proton affinities in excellent agreement with published values and reasonable predictions for the protonation entropies. ...

Polyhydroxyalkanoates (PHAs) are bacterial polyesters having the properties of biodegradable thermoplastics and elastomers. Synthesis of PHAs has been demonstrated in transgenic plants. Both polyhydroxybutyrate and the co-polymer poly(hydroxybutyrate-co-hydroxyvalerate) have been synthesized in the plastids of Arabidopsis thaliana and Brassica napus. Furthermore, a range of medium-chain-length ...

The Gobi Desert in Mongolia, home to the critically endangered Gobi bear (Ursus arctos isabellinus), has few water resources for the animals that inhabit this environment. The majority of these water resources are shallow, small bodies of water, from approximately 30 cm to 3 m in diameter. Due to the harsh nature of the Gobi Desert environment, such pools of water are crucial resources for ...

Polyaniline (PANI) nanotubes (130-250 nm in average diameter) doped with {alpha}-naphthalene sulfonic acid ({alpha}-NSA), {beta}-naphthalene sulfonic acid ({beta}-NSA) and 1,5-naphthalene disulfonic acid were synthesized via a self-assembly process. It was found that the formation yield, morphology (hollow or solid), size, crystalline and electrical ...

Self-assembly of molecules is an attractive materials construction strategy due to its simplicity in application. By considering peptidic, charged synthetic molecules in the bottom-up materials self-assembly design process, one can take advantage of inherently biomolecular attributes; intramolecular folding events, secondary structure, and electrostatic interactions; in addition to more ...

Normally, the brain's fuel is glucose, but during fasting it increasingly relies on ketones (beta-hydroxybutyrate, acetoacetate, and acetone) produced in liver mitochondria from fatty acid beta-oxidation. Although moderately raised blood ketones produced on a very high fat ketogenic diet have important clinical effects on the brain, including reducing ...

Oxygen radical formation in mitochondria is a highly important, but incompletely understood, attribute of eukaryotic cells. I propose a kinetic model in which the ratio between electrons entering the respiratory chain via FADH? or NADH is a major determinant in radical formation. During the breakdown of glucose, this ratio is low; during fatty acid breakdown, this ratio is much higher. The longer ...

There is growing evidence that mitochondrial dysfunction, and more specifically fatty acid ?-oxidationimpairment, is involved in the pathophysiology of non-alcoholic steatohepatitis (NASH). The goal of the present study was to achieve more understanding on the modification/s of carnitinepalmitoyltransferase-I (CPT-I), the rate-limiting enzyme of the mitochondrial fatty acid ?-oxidation, ...

Phytochemical screening of the roots of Gomphrena macrocephala, with particular attention to its triterpene glycoside constituents, has resulted in the isolation of two new oleanane glycosides (1 and 2) and a new taraxerane glycoside (3). The structures of 1-3 were determined as 11alpha,12alpha-epoxy-3beta-[(O-beta-D-glucuronopyranosyl)oxy]olean-28,13-olide (1), ...

beta-N-Oxalyl amino-L-alanine (L-BOAA); synonym beta-N-oxalyl-alpha,beta-diaminopropionic acid (beta-ODAP) is a naturally occurring non-protein amino acid present in the chickling pea from the plant Lathyrus sativus grown in drought prone areas. Ingestion of L-BOAA as a staple diet results in a progressive neurodegenerative condition, neurolathyrism, a ...

Obesity and its associated complications, including diabetes, dyslipidemia, atherosclerosis, and some cancers, have been a global health problem with a rapid increase of the obese population. In this study, we selected 31 obesity candidate genes in the liver of high-fat-induced obese C57BL/6J mice through investigation of literature search and analyzed functional protein-protein interaction of the ...

Recurrent episodes of life-threatening myoglobinuria in childhood are caused by inborn errors of glycogenolysis, mitochondrial fatty acid beta-oxidation, and oxidative phosphorylation. Nonetheless, approximately half of the patients do not suffer from a defect in any of these pathways. Using homozygosity mapping, we identified six deleterious mutations in ...

As essential cofactor in many proteins and redox enzymes, copper and iron are involved in a wide range of biological processes. Mild dietary deficiency of metals represents an underestimated problem for human health, because it does not cause clear signs and clinical symptoms, but it is associated to long-term deleterious effects in cardiovascular system and alterations in lipid metabolism. The ...

Four new iridoids viz., plumeridoids A, B, and C and epiplumeridoid C were isolated from the stem bark of Plumeria rubra Linn. together with twenty-four known compounds viz., 1-( P-hydroxyphenyl)propan-1-one, isoplumericin, plumericin, dihydroplumericin, allamcin, fulvoplumerin, allamandin, plumieride, P- E-coumaric acid, 2,6-dimethoxy- P-benzoquinone, scopoletin, cycloart-25-en-3 beta,24-diol, ...

Metabolism of abscisic acid was compared in stressed and in rehydrated leaf blades of Xanthium strumarium L. Chicago strain that were either detached or left intact on the plant. Under all conditions, phaseic acid was the major metabolite. The high level of phaseic acid that was observed in intact plants 1 day after recovery from stress declined slowly and had not yet reached the prestress level 1 ...

Although mitochondrial fatty acid {beta}-oxidation (FAO) is considered to be well understood, further elucidation of the pathway continues through evaluation of patients with FAO defects. The FAO pathway can be examined by measuring the 3-hydroxy-fatty acid (3-OHFA) intermediates. We present a unique finding in the study of this pathway: the addition of ...

Since the initial discovery that the amino acid beta-N-methylamino-L-alanine (BMAA) was a neurotoxin, a great deal has been learned about its mechanism of action. However, exactly how it causes death of motor neurons, and how its actions may interact with other neurotoxins or pathological conditions, is not well understood. The focus of study on the ...

Swim training for 6 weeks significantly decreased body weight gain, adipose tissue mass, and adipocyte size in both sexes of genetically obese db/db mice compared with their respective sedentary controls. Swim training also caused significant decreases in serum levels of free fatty acids, triglycerides, and total cholesterol in both sexes of obese mice. Concomitantly, hepatic mRNA levels of ...

3-Hydroxy fatty acids (3-OH FAs) of 10-18-carbon chain lengths are constituents of the lipopolysaccharide of Gram-negative bacteria. These acids are used as chemical markers for determining endotoxin in environmental samples. The present communication addresses the question whether this type of analysis also would be applicable to mammalian samples. Low levels (6.1+/-1.6-94.0+/-23.2 pmol/ml) of ...

Four lignanamides, a tyramine derivative, and 10 other nonalkaloidal components were isolated from the seeds of Hyoscyamus niger. Among them, hyoscyamide (1), 1,24-tetracosanediol diferulate (6), and 1-O-(9Z,12Z-octadecadienoyl)-3-O-nonadecanoyl glycerol (7) are new structures. The other compounds were identified as grossamide, cannabisin D, cannabisin G, N-trans-feruloyl tyramine, ...

The kinetics of D- and L-methionine uptake by rings of everted intestine in vitro are consistent with a saturable Michaelis-Menten component plus a linear, diffusional one. All the data could be fit with a diffusion constant, which was essentially the same, independent of whether it was estimated by iteration or by using the extracellular marker, (/sup 3/H), inulin. Similar results were obtained ...

The naturally occurring, non-essential amino acid beta-N-methylamino-L-alanine (BMAA) has been recently found in high concentrations in brain tissues of patients with tauopathies such as the Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex (ALS/PDC) in the South Pacific island of Guam and in a small number of Caucasian, North American patients ...

A phytochemical study of two plant species, Viscum cruciatum Sieber and Crataegus monogyna Jacq., was completed to investigate the influence of the parasite Viscum cruciatum on the host Crataegus monogyna. The study was carried out with two samples and consisted of hexane extracts of the Viscum cruciatum parasitizing on Crataegus monogyna and C monogyna. In these samples ursolic ...

Following the study of the mutagenic action of UV and ..gamma..-radiation on plasmid DNA in vitro, they investigated the induction of mutations under the influence of chemical mutagens on the same DNA of plasmid RSF2124, determining the synthesis of colicine E1 and resistance to ampicillin. The inactivating action of the mutagen was assessed from the yield of transformants resistant to the ...

Peroxisome proliferator activated receptor alpha (PPAR alpha) regulates fatty acid beta-oxidation (FAO) and plays a central role in the metabolic and energetic homeostasis of striated muscles. The thermodynamic consequences of the absence of PPAR alpha were investigated in diaphragm muscle of PPAR alpha knockout mice (KO). Statistical mechanics provides a ...

Two different assays have been developed and used in order to investigate the optimal conditions for derivatization and detection of acid beta-N-methyl-amino-L-alanine (BMAA) in a cyanobacterial sample. BMAA was extracted from cyanobacterial cultures both from the cytosolic ("free") fraction and in the precipitated ("protein") fraction using a newly ...

Various pharmacologic vitreolysis agents, including hyaluronidase, urea, plasmin, dispase, tissue plasminogen activator and chondroitinase have been tested. Pharmacologic vitreolysis can avoid the complications of surgery such as cataract, endophthalmitis, retinal hemorrhage, tear or detachment, and anesthesia related complications. Hyaluronan is a major macromolecule of vitreous. It is a long, ...

Hyaluronan (HA) is a large but simple glycosaminoglycan composed of repeating D-glucuronic acid, beta1-3 linked to N-acetyl-D-glucosamine beta1-4, found in body fluids and tissues, in both intra- and extracellular compartments. Despite its structural simplicity, HA has diverse functions in skeletal biology. In development, HA-rich matrices facilitate ...

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is a severe neurodegenerative disease associated with an impairment of very long chain fatty acids {beta}-oxidation. The authors have recently identified by positional cloning the gene responsible for ALD, located in Xq28. It encodes a new member of the {open_quotes}ABC{close_quotes} ...

The relationship between environmental cadmium pollution and prevalence of signs of renal disturbance was investigated. Women over 60 years of age who had spent the major part of their life in a cadmium-polluted area in Belgium (Liege, n = 60) and who had never been occupationally exposed to cadmium constituted the exposed group. Women living in two areas less polluted by cadmium (Charleroi, n = ...

The deletion of the intracellular polyhydroxyalkanoate (PHA) depolymerase gene (phaZ) in Pseudomonas fluorescens BM07 was found to increase more efficiently the levels of longer medium-chain-length (MCL) omega-aromatic monomer-units than in the wild-type strain when the cells were grown with a mixture of fructose and MCL omega-aromatic fatty acid in the presence of salicylic acid that is known as ...

Peroxisome proliferation is inducible in hepatocytes of rodent and nonrodent species by structurally dissimilar hypolipidemic drugs and certain phthalate ester plasticizers. The induction of peroxisome proliferation appears to be a tissue specific response limited largely to the hepatocyte. Peroxisome proliferation is associated with increases in the activity of the H2O2-generating peroxisomal ...

Five agave plants typically used in Mexico for making mezcal in places included in the Denomination of Origin (Mexican federal law that establishes the territory within which mezcal can be produced) of this spirit were analyzed: Agave salmiana ssp. crassispina, A. salmiana var. salmiana, Agave angustifolia, Agave cupreata, and Agave karwinskii. Fatty acid and total simple lipid profiles of the ...

Acute passive Heymann glomerulonephritis in rats induced heavy proteinuria and highly increased urinary activity of N-acetyl-beta-D-glucosaminidase, acid beta-galactosidase and acid phosphatase. The cortical activity of these acid hydrolases was increased essentially in the large lysosomes as demonstrated by subfractionation of the lysosome-rich ...

This review describes current advances in understanding the biology of plant infection by the rice blast fungus Magnaporthe grisea. Development of the specialized infection structure, the appressorium, in M. grisea has recently been shown to be controlled by cell cycle progression and initiation of autophagic, programmed cell death in the fungal spore. Re-cycling of the contents of the fungal ...

The non-protein amino acid, beta-N-methylaminoalanine (BMAA), is neurotoxic and has been implicated in the amyotrophic lateral sclerosis-Parkinsonism-dementia (ALS-PD) complex of Guam. This concept remains controversial, in part because of the lack of a convincing animal model. The neuropharmacology of BMAA is well established, but little is known of its ...

The role of neurotoxic non-protein amino acid beta-N-methylamino-L-alanine (L-BMAA) as a putative causative agent of Western pacific amyotrophic lateral sclerosis/Parkinsonism dementia complex (ALS/PDC) has recently been reinvigorated. In view of this data we have investigated the strength and mechanism of effect of L-BMAA in presence of 20 mmol/L ...

The antioxidant activities of the methanol extract of Sarcodon imbricatum wildly grown in the Black Sea Region of Turkey were investigated in this study. Antioxidant activities were evaluated in terms of total antioxidant activity, reducing power, metal chelating ability, inhibition of linoleic acid peroxidation, superoxide, peroxide and hydrogen peroxide scavenging effects. Various antioxidant ...

The methanolic crude and methanol-aqueous extract of Alstonia macrophylla leaves and n-butanol part of the crude extract showed antimicrobial activity against various strains of Staphylococcus aureus, Staphylococcus saprophyticus, Streptococcus faecalis, Escherichia coli, Proteus mirabilis, Trichophyton rubrum, Trichophyton mentagrophytes var. mentagrophytes and Microsporum gypseum. The minimum ...

"Hierba santa," a Peruvian herbal medicine, is used to alleviate many symptoms, including headache, hemorrhoids, fever, and rheumatism. Several Cestrum species are said to be the origin of hierba santa. Three lots of hierba santa: Cestrum auriculatum (herb 1 and herb 2) and C. hediundinum (herb 3), which were purchased from Peruvian markets at Cuzco (Andes area) and Equitos (Amazon area), ...

There is evidence showing abnormalities in collagen from the skin of patients with sporadic Amyotrophic Lateral Sclerosis (sALS) both from Guam and elsewhere. The non-proteinogenic amino acid beta-N-methylamino-l-alanine (BMAA) was first associated with the high incidence of Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex (ALS/PDC) in Guam, and ...

Free amino play an important role in regulating cell volume in fishes. Four tissues/cells (skeletal muscle, RBC, brain, and myocardium) of the little skate, Raja erinacea, were selected for detailed study because of their special importance or unique advantage as experimental models. Three particular amino acids, beta-alanine, taurine, and sarcosine play a ...

The allelopathic potential of Citrus junos fruit waste after juice extraction was investigated. Aqueous methanol extracts of peel, inside and seeds separated from the fruit waste inhibited the growth of the roots and shoots of alfalfa (Medicago sativa L.), cress (Lepidium sativum L.), crabgrass (Digitaria sanguinalis L.), lettuce (Lactuca sativa L.), timothy (Pheleum pratense L.), and ryegrass ...

A survey of the literature to date on the enzyme histochemistry of intracellular organelles has not yielded any reference to the presence of acid phosphatase reaction products in the mammalian mitochondria of the central nervous system. A combination of Gomori's acid phosphatase method, however, with standard electron microscopy has disclosed the presence of enzyme reaction ...

The function of the Idaho Chemical Processing Plant is to process fuel elements containing uranium enriched in the isotope 235 for recovery of the unfissioned U. A major throughput of the plant is the solid Al-U type element. The plant process is based on acid dissolution, then separation of the U from the Al cladding and fission products by liquid-liquid extraction. It is the purpose ...

Abraham's H-bonding parameters alpha and beta have been described in terms of a minimal set of readily obtainable molecular descriptors. These parameters are basically equilibrium constants for complexation of acids with a reference base (alpha) or bases with a reference acid (beta) measured in a non-hydrogen bonding solvent such as tetrachloromethane. The ...

The biosynthesis of chloramphenicol requires a beta-hydroxylation tailoring reaction of the precursor L-p-aminophenylalanine (L-PAPA). Here, it is shown that this reaction is catalyzed by the enzyme CmlA from an operon containing the genes for biosynthesis of L-PAPA and the nonribosomal peptide synthetase CmlP. EPR, M�ssbauer, and optical spectroscopies reveal that CmlA contains an oxo-bridged ...

The biosynthesis of chloramphenicol requires a ?-hydroxylation tailoring reaction of the precursor L-p-aminophenylalanine (L-PAPA). Here, it is shown that this reaction is catalyzed by the enzyme CmlA from an operon containing the genes for biosynthesis of L-PAPA and the nonribosomal peptide synthetase CmlP. EPR, M�ssbauer, and optical spectroscopies reveal that CmlA contains an oxo-bridged ...

In order to investigate the role of the putative epimerase function of the beta-oxidation multienzyme complex (FadBA) in the provision of (R)-3-hydroxyacyl-CoA thioesters for medium-chain-length polyhydroxyalkanoate (PHA(MCL)) biosynthesis, the fadBA(Po) operon of Pseudomonas oleovorans was cloned and characterized. The fadBA(Po) operon and a class-II PHA synthase gene of Pseudomonas aeruginosa ...

The purpose of this study was to discover the effects of soybean beta-conglycinin (7S-globulin) and glycinin (11S-globulin) on serum lipid levels and metabolism in the livers of normal and genetically obese mice. Male normal (ICR) and obese (KK-Ay) mice were fed ad libitum high fat diets for two weeks, followed by a 2-week restriction of diet (2 g diet/mouse/day) containing 20% casein, soybean ...

Normal function of the peroxisome proliferator-activated receptor alpha (PPARalpha) is crucial for the regulation of hepatic fatty acid metabolism. Fatty acids serve as ligands for PPARalpha, and when fatty acid levels increase, activation of PPARalpha induces a battery of fatty acid-metabolizing enzymes to restore fatty acid levels to normal. Hepatic fatty acid levels are increased during ethanol ...

Little is known about the factors involved in the brown adipocyte gene regulatory program. In contrast to the white adipocyte, the brown adipocyte is characterized by abundant mitochondria and high level expression of mitochondrial fatty acid beta-oxidation enzymes. Previous studies in transgenic mice have shown that the brown adipose-enriched expression ...

A 10-year-old boy is referred to the pediatric ward for regression of school performance and behavior problems. The clinic has been evolving for two years in a context of parental separation. Given the clinical deterioration, the patient is sent to child psychiatrist. A complete somatic checkup is achieved, he reveals white matter lesions suggesting the diagnosis of X-linked adrenoleukodystrophy ...

A number of monosaccharides composing the glycocalix carbohydrates of some Mollicutes (Mycoplasma pneumoniae FH, M. hominis PG21, M. fermentans PG18, Acholeplasma laidlawii PG8, A. laidlawii var. granulum st. 118) and phylogenetically related bacteria from the genus Bacillus (Bacillus cereus 89, B. cereus DM423, B. subtilis 1/2, B. licheniformis 31, B. licheniformis 49) were detected using plant ...

Our previous studies suggest that peroxisome proliferator-activated receptor-alpha (PPARalpha) plays a critical role in regulating fatty acid beta-oxidation in kidney tissue and this directly correlated with preservation of kidney morphology and function during acute kidney injury. To further study this, we generated transgenic mice expressing PPARalpha in ...

Despite being extensively studied in various organisms due to scientific, industrial and medical interest, the galactose assimilation function and regulation, and especially its interaction with other parts of cellular physiology, have not been fully elucidated yet. The post-genomic era holistic techniques ("omics") could assist towards this goal. In this paper, we holistically analyzed ...

During development, gene expression of medium-chain acyl-CoA dehydrogenase (MCAD), a nuclear-encoded mitochondrial enzyme that catalyses the first step of medium-chain fatty acid beta-oxidation, is highly regulated in tissues in accordance with fatty acid utilization, but the factors involved in this regulation are largely unknown. To investigate a ...

The hypolipidemic fibric acid drugs are peroxisome proliferator-activated receptor a (PPAR alpha) ligands. PPAR alpha activated by fibric acids form heterodimers with the 9-cis retinoic acid receptor (RXR). The PPAR/RXR heterodimers bind to peroxisome proliferator response elements (PPRE), which are located in numerous gene promoters and increase the level of the expression of mRNAs encoded by ...

The promoter of the pepper pathogen-induced membrane protein gene CaPIMP1 was analyzed by an Agrobacterium-mediated transient expression assay in tobacco leaves. Several stress-related cis-acting elements (GT-1, W-box and ABRE) are located within the CaPIMP1 promoter. In tobacco leaf tissues transiently transformed with a CaPIMP1 promoter-beta-glucuronidase (GUS) gene fusion, serially 5'-deleted ...

The role of the Saccharomyces cerevisae peroxisomal acyl-coenzyme A (acyl-CoA) thioesterase (Pte1p) in fatty acid beta-oxidation was studied by analyzing the in vitro kinetic activity of the purified protein as well as by measuring the carbon flux through the beta-oxidation cycle in vivo using the synthesis of peroxisomal polyhydroxyalkanoate (PHA) from ...

Dothideaceous black yeast-like fungi (BYF) are known to synthesise DHN-melanin that is inhibited by the systemic fungicide tricyclazole. The final step of the DHN melanin pathway is the conjoining of 1,8-DHN molecules to form the melanin polymer. There are several candidate enzymes for this step, including phenoloxidases such as tyrosinase and laccases, peroxidases, and perhaps also catalases. We ...

Seeds of the Guam cycad Cycas micronesica K.D. Hill (Cycadaceae), which contain ss-methylamino-L-alanine (BMAA), have been implicated in the etiology of the devastating neurodisease ALS-PDC that is found among the native Chamorros on Guam. The disease also occurs in the native populations on Irian Jaya and the Kii Peninsula of Japan, and in all three areas the cycad seeds are used either dietarily ...

ABSTRACT: BACKGROUND: The nuclear receptors Peroxisome Proliferator-Activated Receptor (PPARgamma) and Peroxisome Proliferator-Activated Receptor (PPARdelta) play central roles in regulating metabolism in adipose tissue, as well as being targets for the treatment of insulin resistance. While the role of PPARgamma in regulating insulin sensitivity has been well defined, research into PPARdelta has ...

Beta-N-oxalyl-L-alpha-beta-diaminopropionic acid (beta-L-ODAP) is an unusual amino acid present in seeds of plants from the Lathyrus genus that is generally accepted as the causative agent underlying the motor neuron degeneration and spastic paraparesis in human neurolathyrism. Much of the neuropathology produced by beta-L-ODAP appears to be a direct ...

Methods for amino acid synthesis are described that involve the catalytic reduction of precursor aliphatic acids and aromatic amino acids, and the catalytic dehalogenation with tritium of brominated and iodinated precursors. The following amino acids were synthesized: /sub DL/-alanine- alpha , BETA T/sub 2/ (from alpha -acetaminoacrylic acid ethyl ester) with a specific activity of ...

Vitamin A and its derivaties (retinoids) are necessary for the maintenance of normal phenotypic expression. An attempt at understanding the biochemical role of vitamin A had led to the demonstration of a new pathway for retinol. In this pathway, vitamin A is phosphorylated to retinylphosphate (RP), which is then glycosylated to retinylphosphatemannose (MRP). These two derivatives have been found ...

Fatty acids are the preferred substrate for the normoxic heart. About sixty percent of the energy required by the myocardium is provided by fatty acid [beta]-oxidation. Many scientists have focused on the alterations in fatty acid metabolism in the ischemic heart for the development of radiolabelled fatty acids for functional imaging of the heart. Three ...

Acyl-CoA dehydrogenase [acyl-CoA:(acceptor) 2,3-oxidoreductase; EC 1.3.99.3] catalyzes the first reaction step in mitochondrial fatty-acid beta-oxidation. Here, the very-long-chain acyl-CoA dehydrogenase from Caenorhabditis elegans (cVLCAD) has been cloned and overexpressed in Escherichia coli strain BL21 (DE3). Interestingly, unlike other very-long-chain ...

An aminopeptidase was purified about 1,700-fold from seeds of Japanese apricot (Prunus mume Sieb.) by a seven-step procedure comprising extraction from seeds, ammonium sulfate fractionation, DEAE-cellulose chromatography, first and second DEAE-Sepharose chromatography, hydroxyapatite chromatography, and Sephadex G-200 gel filtration. The purified enzyme was shown to be homogeneous on ...

Diverse species of cyanobacteria have recently been discovered to produce the neurotoxic non-protein amino acid beta-methylamino-L-alanine (BMAA). In Guam, BMAA has been studied as a possible environmental toxin in the diets of indigenous Chamorro people known to have high levels of Amyotrophic Lateral Sclerosis/ Parkinsonism Dementia Complex (ALS/PDC). ...

BackgroundThe origin of eukaryotes remains a fundamental question in evolutionary biology. Although it is clear that eukaryotic genomes are a chimeric combination of genes of eubacterial and archaebacterial ancestry, the specific ancestry of most eubacterial genes is still unknown. The growing availability of microbial genomes offers the possibility of analyzing the ancestry of eukaryotic genomes ...

The peroxisomal oxidation of the long chain fatty acid palmitate (C16:0) and the very long chain fatty acids lignocerate (C24:0) and cerotate (C26:0) was studied in freshly prepared homogenates of cultured skin fibroblasts from control individuals and patients with peroxisomal disorders. The peroxisomal oxidation of the fatty acids is almost completely dependent on the addition of ATP, coenzyme A ...

Channel catfish (Ictalarus punctatus) were exposed to 0, 10, 20, and 40% bleached kraft pulp and paper mill effluent (BKME) for up to two weeks. Fish were sacrificed on days 1, 3, 7, and 14 of exposure. Blood, liver, and gill tissues were assayed for biochemical responses associated with oxidative stress (superoxide dismutase, catalase, and glutathione peroxidase activities and methemoglobin, ...

Metabolite profiling (metabolomics) elucidates changes in biochemical pathways under various conditions, e.g., different nutrition scenarios or compound administration. BASF and metanomics have obtained plasma metabolic profiles of approximately 500 compounds (agrochemicals, chemicals and pharmaceuticals) from 28-day rat studies. With these profiles the establishment of a database ...

Medium-chain acyl-CoA dehydrogenase is one of three similar enzymes that catalyze the initial step of fatty acid ..beta..-oxidation. Definition of the primary structure of MCAD and the tissue distribution of its mRNA is of biochemical and clinical importance because of the recent recognition of inherited MCAD deficiency in humans. The MCAD mRNA nucleotide ...

An integrated metabonomic approach based on high-resolution (1)H NMR spectroscopy has been applied to the investigation of the acute biochemical effects caused by Ce(NO(3))(3) in rats. Male Wistar rats were separated into 8 groups and each was treated with one of following compounds, mercury II chloride (HgCl(2)), 2-bromoethanamine hydrobromide (BEA), carbon tetrachloride (CCl(4)), ...

Sensory-guided decomposition of roasted cocoa nibs revealed that, besides theobromine and caffeine, a series of bitter-tasting 2,5-diketopiperazines and flavan-3-ols were the key inducers of the bitter taste as well as the astringent mouthfeel imparted upon consumption of roasted cocoa. In addition, a number of polyphenol glycopyranosides as well as a series of N-phenylpropenoyl-l-amino acids have ...

Although treatments with nucleoside reverse transcriptase inhibitors (NRTIs) can modify fat metabolism and fat distribution in humans, the mechanisms of these modifications and the roles of diverse NRTIs are unknown. We studied the mitochondrial and metabolic effects of stavudine (d4T), zidovudine (AZT), didanosine (ddI), lamivudine (3TC), zalcitabine (ddC), and three combinations (AZT-3TC, ...

Abstract The advent of Tandem Mass Spectrometry (MS/MS) around 10 years ago allowed to enlarge consistently the spectrum of metabolic diseases that might be easily and quickly detected. MS/MS was applied to newborn screening in many developed Countries, with a wide use, to detect as many as 55 abnormal biochemical conditions (USA), or a restricted one detecting only few diseases ( Germany, UK, ...

To examine the mechanism of nitrogen sparing in obese subjects on very low calorie diets (VLC-PS), effects of total fasting (TF) and VLC-PS (380 Kcal/day) supplying protein without carbohydrate on metabolites, hormones, urea nitrogen excretion, and plasma (3-/sup 3/H)glucose turnover, (U-/sup 14/C)lysine flux, and (1-/sup 14/C)leucine flux and oxidation were compared. Subjects with a wide range of ...

This review addresses recent advances in specific mechanisms of hepatotoxicity. Because of its unique metabolism and relationship to the gastrointestinal tract, the liver is an important target of the toxicity of drugs, xenobiotics, and oxidative stress. In cholestatic disease, endogenously generated bile acids produce hepatocellular apoptosis by stimulating Fas translocation from the cytoplasm to ...

The bitter acids of hops (Humulus lupulus L.) mainly consist of alpha-acids, beta-acids, and their oxidation products that contribute the unique aroma of the beer beverage. Hop bitter acids displayed a strong growth inhibitory effect against human leukemia HL-60 cells, with an estimated IC(50) value of 8.67 microg/mL, but were less effective against human ...

Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is one of the genetic defects of mitochondrial fatty acid beta-oxidation presenting in early infancy or childhood. If undiagnosed and untreated, VLCAD deficiency may be fatal, secondary to cardiac involvement. We assessed the effect of replacing part of the fat in the diet of a 2 �-month-old male ...

Lipopolysaccharides (LPS, O-antigens) of 12 strains of the photosynthetic bacterium Rhodopseudomonas gelatinosa were obtained by the phenol/chloroform/petroleum ether method, recommended for extracting lipophilic glycolipids of enterobacterial R-mutants. All R. gelatinosa LPS have essentially the same chemical composition. Similar to LPS of Salmonella R-mutants of chemotypes Rd1 and Rd2, the sole ...

The non-protein amino acid beta-aminobutyric acid (BABA) enhances Arabidopsis resistance to microbial pathogens and abiotic stresses through potentiation of the Arabidopsis defence responses. In this study, it is shown that BABA induces the stress-induced morphogenic response (SIMR). SIMR is observed in plants exposed to sub-lethal stress conditions. ...

The kinetics of Na-coupled solute transport by renal and jejunal brush-border vesicles in the rabbit were examined using the potential-sensitive fluorescent dye diS-C3-(5). All organic solutes known to be transported across these membranes by Na-coupled mechanisms increase the fluorescence of the dye in the presence of Na, but not K. An increase in fluorescence (delta F) corresponds to a ...

In this study, we investigated whether increased dietary fat influences established pancreatic cancer cells. MiaPaCa2 human pancreatic cancer cells were grown orthotopically in athymic mice fed normal diet (ND) or high-fat diet (HF). In the resulting tumors, medium-chain acyl-coenzyme A dehydrogenase (MCAD, a regulator of fatty acid beta-oxidation) and ...

Direct enzyme replacement therapy (ERT) has been introduced as a means to treat a number of rare, complex genetic conditions associated with lysosomal dysfunction. Gaucher disease was the first for which this therapy was applied and remains the prototypical example. Although ERT using recombinant lysosomal enzymes has been shown to be effective in altering the clinical course of Gaucher disease, ...

In the Hula Valley, Israel, four layers of peat of Middle Pleistocene to Holocene age, interbedded with limnic clays and marls, are found above 300-m depth. The main detrital minerals are kaolinite, montmorillonite, and illite. Average elemental values of organic matter of dry peat are: C, 15-29 percent; H, 1.8-3 percent; N, 0.8-1.2 percent; S, 2.3-5 percent. The ash content (on dry basis) is ...

Peroxisomes are ubiquitous cellular organelles that perform vital functions including fatty acid beta-oxidation, plasmalogen synthesis, and detoxification of harmful oxidative species. In rodents numerous compounds that increase peroxisome biogenesis also alleviate metabolic syndrome (MetS)/type 2 diabetes (T2D) symptoms. However, compounds that increase ...

Peroxisome proliferator-activated receptor alpha (PPARalpha) is a member of the nuclear/steroid receptor gene superfamily that plays an essential role in fatty acid metabolism. PPARalpha modulates the expression of genes encoding peroxisomal fatty acid beta-oxidation enzymes and microsomal fatty acid hydroxylases CYP4As. We have previously reported that ...

Inhibition of cannabinoid receptor 1 (CB1) has shown efficacy in reducing body weight and improving metabolic parameters, with the effects correlating with target engagement in the brain. The peripheral effects of inhibiting the CB1 receptor has been appreciated through studies in diet-induced obese and liver-specific CB1 knockout mice. In this article, we systematically investigated gene ...

Gold nanoparticles (Au NPs) were functionalized by cysteine (Cys), beta-amyloid peptides (Cys(0)Abeta(1-28), Cys(0)Abeta(1-40), Abeta(1-42)) and a pentapeptide fragment (Leu-Pro-Phe-Phe-Asp-OH (LPFFD-OH)). Optical absorption spectra of these systems were recorded and the plasmon resonance maximum values (lambda(max)) of the UV-vis spectra together with the transmission electron microscopy (TEM) ...

Betaine is an osmolyte accumulated in cells during osmotic cell shrinkage. The canine transporter mediating cellular accumulation of the osmolyte betaine and the neurotransmitter gamma-aminobutyric acid (BGT-1) was expressed in Xenopus oocytes and analyzed by two-electrode voltage clamp and tracer flux studies. Exposure of oocytes expressing BGT-1 to betaine or gamma-aminobutyric acid (GABA) ...

Xanthomonas campestris is a Gram-negative bacterium that produces an exopolysaccharide known as xanthan gum. Xanthan is involved in a variety of biological functions, including pathogenesis, and is widely used in the industry as thickener and viscosifier. Although the genetics and biosynthetic process of xanthan are well documented, the enzymatic components have not been examined and no data on ...

Various commercial lyophilized and immobilized preparations of lipase A from Candida antarctica (CAL-A) were studied for their ability to catalyze the hydrolysis of amide bonds in N-acylated alpha-amino acids, 3-butanamidobutanoic acid (beta-amino acid) and its ethyl ester. The activity toward amide bonds is highly untypical of lipases, despite the close ...

We tested the effect of leaching on the concentration of western red cedar (WRC; Thuja plicata Donn ex D. Don) heartwood extractives that are known to exhibit antimicrobial activity and correlated this with fungal growth and decay. We assessed the extractive tolerance of the following fungal species: Acanthophysium lividocaeruleum, Coniophora puteana, Heterobasidion annosum, Pachnocybe ferruginea, ...

The present study was undertaken to determine the effects of dietary folic acid (FOL) supplementation on ruminal fermentation, duodenal nutrient flow, serum and milk variables, and on B-vitamin concentration in serum. The study was divided into two experiments: in Exp. 1 the forage to concentrate (F:C) ratio of the diet (DM basis) was 34:66 (high concentrate, HC), while in Exp. 2 the F:C ratio was ...

Refinement of in vitro systems for predictive toxicology is important in order to develop high-throughput early toxicity screening assays and to minimize animal testing studies. This study assesses the ability of mouse Hepa-1c1c7 hepatoma cell model under differing culture conditions to predict in vivo estrogen-induced hepatic gene expression changes. Custom mouse cDNA microarrays were used to ...

Refinement of in vitro systems for predictive toxicology is important in order to develop high-throughput early toxicity screening assays and to minimize animal testing studies. This study assesses the ability of mouse Hepa-1c1c7 hepatoma cell model under differing culture conditions to predict in vivo estrogen-induced hepatic gene expression changes. Custom mouse cDNA microarrays were used to ...

X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disorder associated with reduced very long-chain fatty acid beta-oxidation, mainly affecting the nervous system, the adrenal cortex and the testes. The clinical manifestations of hypogonadism, alopecia and the impairment of the enzyme 5alpha-reductase, which converts testosterone into ...

A gap junction blocker, 18beta-glycyrrhetinic acid (beta-GA), increased the membrane resistance of Ia, Ib and II/III cells of frog taste disk by 50, 160, and 300 M Omega, respectively, by blocking the gap junction channels and hemichannels. The amplitudes of gustatory depolarizing potentials in the disk cells for 4 basic taste stimuli were reduced to ...

The polyphenol oxidase (LsPPO) from a wild edible mushroom Lactarius salmonicolor was purified using a Sepharose 4B-L-tyrosine-p-amino benzoic acid affinity column. At the optimum pH and temperature, the K(M) and V(Max) values of LsPPO towards catechol, 4-methylcatechol and pyrogallol were determined as 0.025 M & 0.748 EU/mL, 1.809 x 10(- 3) M & 0.723 EU/mL and 9.465 x 10(- 3) M & ...

This study examines the ability of dietary lipids to regulate gene expression of mitochondrial and peroxisomal fatty acid beta-oxidation enzymes in the kidney cortex and medulla of 3-wk-old rats and evaluates the role of glucagon or of the alpha-isoform of peroxisome proliferator-activated receptor (PPARalpha) in mediating beta-oxidation enzyme gene ...

We aimed to determine concentrations of the neurotoxic amino acids beta-N-methylamino-L-alanine (BMAA) and alpha-,gamma-diaminobutyric acid (DAB) in mixed species scum material from Dutch urban waters that suffer from cyanobacterial blooms. BMAA and DAB were analysed in scum material without derivatization by LC-MSMS (liquid chromatography coupled to ...

omega-3 fatty acids, alpha-tocopherol, ascorbic acid, beta-carotene and glutathione determined in leaves of purslane (Portulaca oleracea), grown in both a controlled growth chamber and in the wild, were compared in composition to spinach. Leaves from both samples of purslane contained higher amounts of alpha-linolenic acid (18:3w3) than did leaves of ...

The neurotoxic amino acid, beta-N-methylamino-L-alanine, was found to be present in all of 12 analysed samples of cyanobacterial blooms, scums and mats, which had been collected in seven years between 1990 and 2004 inclusive and stored at -20 degrees C. BMAA identification was by high performance liquid chromatography with fluorescence detection and by ...

Nuclear transfer using transgenic donor cells is an efficient way of generating transgenic goats, wherein the preparation of competent transgenic donor cells is the pivotal upstream step. We have measured the efficiency of transfection with a plasmid containing hGCase (human lysosomal acid beta-glucosidase) gene into goat FFC (fetal-derived fibroblast ...

Ionising radiation is known one of the most effective tools in the therapy of cancer but in many thoracic cancers, the total prescribed dose of radiation that can be safely administered to the target volume is limited by the risk of complications arising in the normal lung tissue. One of the major reasons for cellular injury after radiation is the formation of reactive oxygen species (ROS). ...

The organic solid (tholin) produced by spark discharge in a CH4 + NH3 + H2O atmosphere is investigated, along with the separable components of its water-soluble fraction. The chemistry of this material serves as a provisional model for the interaction of Jovian organic heteropolymers with the deep aqueous clouds of Jupiter. Intact (unhydrolyzed) tholin is resolved into four chemically distinct ...

We here report biomagnification (the increasing accumulation of bioactive, often deleterious molecules through higher trophic levels of a food chain) of the neurotoxic nonprotein amino acid beta-methylamino-l-alanine (BMAA) in the Guam ecosystem. Free-living cyanobacteria produce 0.3 microg/g BMAA, but produce 2-37 microg/g as symbionts in the coralloid ...

Although they are less abundant than their alpha-analogues, beta-amino acids occur in nature both in free form and bound to peptides. Oligomers composed exclusively of beta-amino acids (so-called beta-peptides) might be the most thoroughly investigated peptidomimetics. Beside the facts that they are stable to metabolism, exhibit slow microbial degradation, and are inherently stable to proteases ...

The positive effects of berberine (30 mg/kg/day, i.g. for 6 weeks) on cardiac dysfunction were evaluated in the rat model of hyperglycemia and hypercholesterolemia. Hyperglycemia and hypercholesterolemia were induced by feeding high-sucrose/fat diet (HSFD) consisting of 20% sucrose, 10% lard, 2.5% cholesterol, 1% bile salt for 12 weeks and streptozotocin (30 mg/kg, i.p.). The plasma sugar, total ...

This study examines the relationships between metabolic status and behavior in spontaneously fasting birds in the context of long-term regulation of body mass and feeding. Locomotor activity, escape behavior, display songs, body mass, and metabolic and endocrine status of captive male emperor penguins were recorded during a breeding fast. We also examined whether body mass at the end of the fast ...

The relative balance between the quantity of white and brown adipose tissue can profoundly affect lipid storage and whole-body energy homeostasis. However, the mechanisms regulating the formation, expansion, and interconversion of these 2 distinct types of fat remain unknown. Recently, the lysosomal degradative pathway of macroautophagy has been identified as a regulator of cellular ...

Substrate cycles are ubiquitous structures of the cellular metabolism (e.g. Krebs cycle, fatty acids beta-oxydation cycles, etc...). Moiety-conserved cycles (e.g. adenine nucleotides and NADH/NAD, etc...) are also important. The role played by such cycles in the metabolism and its regulation is not clearly understood so far. However, it was shown that ...

Seed dormancy is essential for most plants to control the timing of germination. In Arabidopsis thaliana, PED3 is a single-copy gene encoding an ATP-binding cassette transporter that is required for peroxisomal fatty acid beta-oxidation. PED3 is involved in the import of several biologically important molecules into the peroxisome, including ...