Botanical smuts and hermaphrodites: Lydia Becker, Darwin's botany, and education reform.

PubMed

Gianquitto, Tina

2013-06-01

In 1868, Lydia Becker (1827-1890), the renowned Manchester suffragist, announced in a talk before the British Association for the Advancement of Science that the mind had no sex. A year later, she presented original botanical research at the BAAS, contending that a parasitic fungus forced normally single-sex female flowers of Lychnis diurna to develop stamens and become hermaphroditic. This essay uncovers the complex relationship between Lydia Becker's botanical research and her stance on women's rights by investigating how her interest in evolutionary theory, as well as her correspondence with Charles Darwin, critically informed her reform agendas by providing her with a new vocabulary for advocating for equality. One of the facts that Becker took away from her work on Lychnis was that even supposedly fixed, dichotomous categories such as biological sex became unfocused under the evolutionary lens. The details of evolutionary theory, from specific arguments on structural adaptations to more encompassing theories on heredity (i.e., pangenesis), informed Becker's understanding of human physiology. At the same time, Becker's belief in the fundamental equality of the sexes enabled her to perceive the distinction between inherent, biological differences and culturally contingent ones. She applied biological principles to social constructs as she asked: Do analogous evolutionary forces act on humans?

[Late Becker's nevus. One hundred cases (author's transl)].

PubMed

Tymen, R; Forestier, J F; Boutet, B; Colomb, D

1981-01-01

The authors make a survey of a series of a hundred Becker's nevi diagnosed in a recruiting center of the French National Service. It is a statistic study bearing on the male population, aged 17 to 26, in a French region which is supposed to be representative of the French male population of the same age. First of all the authors focus on the various clinical descriptions since this nevus was first described by Becker in 1949, then discuss the problems raised by pathogenic interpretation and the results of microscopic studies. About this lesion, usually said to be pigmentary and hairy, and generally appearing before 20 years of age, is still very little known. Then after a short explanation of their method of study the authors give original results. A hundred Becker's nevi were diagnosed among 19,302 young men, that is to say in a proportion of 0.52 p. 100. A study of the racial, genetic and pigmentary background doesn't enable to retain general causes but rather leads to think of local causes. Half of these Becker's nevi appeared before ten years of age, the other half appearing between 10 and 20. In only one quarter of the cases can the role of the sun be pointed out. The location of the nevi is variable; they are mainly situated on the trunk and only 32 p. 100 of them are located above nipples, which was believed to be the usual place of the lesion. Pigmentation in three quarter of the cases is light brown. The average surface is 125 cm2. Hairs can be seen in only a little more than half of the cases of no lesion associated to the nevus could be found. The study points out that Becker's nevi seem to be more various in their clinical expression and more pigment than could be thought through more classical description.

Disparities in the diagnostic process of Duchenne and Becker muscular dystrophy.

PubMed

Holtzer, Caleb; Meaney, F John; Andrews, Jennifer; Ciafaloni, Emma; Fox, Deborah J; James, Katherine A; Lu, Zhenqiang; Miller, Lisa; Pandya, Shree; Ouyang, Lijing; Cunniff, Christopher

2011-11-01

To determine whether sociodemographic factors are associated with delays at specific steps in the diagnostic process of Duchenne and Becker muscular dystrophy. We examined abstracted medical records for 540 males from population-based surveillance sites in Arizona, Colorado, Georgia, Iowa, and western New York. We used linear regressions to model the association of three sociodemographic characteristics with age at initial medical evaluation, first creatine kinase measurement, and earliest DNA analysis while controlling for changes in the diagnostic process over time. The analytical dataset included 375 males with information on family history of Duchenne and Becker muscular dystrophy, neighborhood poverty levels, and race/ethnicity. Black and Hispanic race/ethnicity predicted older ages at initial evaluation, creatine kinase measurement, and DNA testing (P < 0.05). A positive family history of Duchenne and Becker muscular dystrophy predicted younger ages at initial evaluation, creatine kinase measurement and DNA testing (P < 0.001). Higher neighborhood poverty was associated with earlier ages of evaluation (P < 0.05). Racial and ethnic disparities in the diagnostic process for Duchenne and Becker muscular dystrophy are evident even after adjustment for family history of Duchenne and Becker muscular dystrophy and changes in the diagnostic process over time. Black and Hispanic children are initially evaluated at older ages than white children, and the gap widens at later steps in the diagnostic process.

Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

PubMed

Momma, Kazunari; Noguchi, Satoru; Malicdan, May Christine V; Hayashi, Yukiko K; Minami, Narihiro; Kamakura, Keiko; Nonaka, Ikuya; Nishino, Ichizo

2012-01-01

Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

Decline in growth of foraminifer Marginopora rossi under eutrophication and ocean acidification scenarios.

PubMed

Reymond, Claire E; Lloyd, Alicia; Kline, David I; Dove, Sophie G; Pandolfi, John M

2013-01-01

The combination of global and local stressors is leading to a decline in coral reef health globally. In the case of eutrophication, increased concentrations of dissolved inorganic nitrogen (DIN) and phosphorus (DIP) are largely attributed to local land use changes. From the global perspective, increased atmospheric CO2 levels are not only contributing to global warming but also ocean acidification (OA). Both eutrophication and OA have serious implications for calcium carbonate production and dissolution among calcifying organisms. In particular, benthic foraminifera precipitate the most soluble form of mineral calcium carbonate (high-Mg calcite), potentially making them more sensitive to dissolution. In this study, a manipulative orthogonal two-factor experiment was conducted to test the effects of dissolved inorganic nutrients and OA on the growth, respiration and photophysiology of the large photosymbiont-bearing benthic foraminifer, Marginopora rossi. This study found the growth rate of M. rossi was inhibited by the interaction of eutrophication and acidification. The relationship between M. rossi and its photosymbionts became destabilized due to the photosymbiont's release from nutrient limitation in the nitrate-enriched treatment, as shown by an increase in zooxanthellae cells per host surface area. Foraminifers from the OA treatments had an increased amount of Chl a per cell, suggesting a greater potential to harvest light energy, however, there was no net benefit to the foraminifer growth. Overall, this study demonstrates that the impacts of OA and eutrophication are dose dependent and interactive. This research indicates an OA threshold at pH 7.6, alone or in combination with eutrophication, will lead to a decline in M. rossi calcification. The decline in foraminifera calcification associated with pollution and OA will have broad ecological implications across their ubiquitous range and suggests that without mitigation it could have serious implications

Hypertrichotic Becker's nevi treated with combination 1,550 nm non-ablative fractional photothermolysis and laser hair removal.

PubMed

Balaraman, Brundha; Friedman, Paul M

2016-04-01

The removal of Becker's nevi poses a significant challenge due to limited available therapeutic options and increased risk of adverse effects, including scarring and dyspigmentation. Herein, we present the use of the non-ablative fractional photothermolysis in combination with laser hair removal for the treatment of hypertrichotic Becker's nevi. Retrospective analysis of three patients with Becker's nevi revealed that two patients with hypertrichotic Becker's nevi had greater than 75% clearance with combination therapy, and one patient with atrichotic Becker's nevus had a similar result with monotherapy non-ablative fractional photothermolysis. This report demonstrates the utility and safety of combination non-ablative fractional resurfacing and laser hair removal for the treatment of hypertrichotic Becker's nevi, and monotherapy non-ablative fractional photothermolysis for atrichotic Becker's nevi. Further comparative studies are necessary to determine optimal laser parameters, treatment schedules, and response duration. © 2016 Wiley Periodicals, Inc.

An Intimate Portrait of Evaluation Mentorship under Peter H. Rossi

ERIC Educational Resources Information Center

Chen, Huey T.

2007-01-01

The author grew up in Taiwan and in 1995 enrolled in Kent State University's PhD program in sociology. Early in his doctoral career at Kent State, he took a program evaluation course offered by Irwin Deutscher. The textbook he used in the class was Peter Rossi and Walter Williams's 1972 book, "Evaluating Social Programs: Theory, Practice, and…

[Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy].

PubMed

Silva, Helga Cristina Almeida da; Hiray, Marcia; Vainzof, Mariz; Schmidt, Beny; Oliveira, Acary Souza Bulle; Amaral, José Luiz Gomes do

2017-05-31

Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU.L -1 (normal 174IU.L -1 ). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. Copyright © 2017 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Cardiomyopathy in becker muscular dystrophy: Overview.

PubMed

Ho, Rady; Nguyen, My-Le; Mather, Paul

2016-06-26

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

Cardiomyopathy in becker muscular dystrophy: Overview

PubMed Central

Ho, Rady; Nguyen, My-Le; Mather, Paul

2016-01-01

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed. PMID:27354892

The natural history of Becker expandable breast implants: a single-center 10-year experience.

PubMed

Sindali, Katia; Davis, Marcus; Mughal, Maleeha; Orkar, Kusu S

2013-09-01

Use of Becker expandable breast implants in single-stage breast surgery is a well-established technique; however, replacement with fixed-volume implants is common. The authors sought to analyze the long-term natural history of these implants over a wide range of surgical indications. A retrospective review of 330 consecutive patients who underwent 384 Becker expander breast reconstructions over a 10-year period in a dedicated plastic surgery unit was undertaken. Implant indication, Becker type, volume and site, complications, expander lifespan, and explant reasons were assessed. Two hundred twenty-eight patients (267 implants) and 102 patients (117 implants) underwent implantation for congenital deformities and breast cancer reconstruction, respectively. One hundred eighty-seven (48 percent) were explanted at a median period of 13.0 months (range, 9.0 to 26.0 months), 149 (39 percent) for aesthetic reasons and 38 (10 percent) for complications. Complication rates were higher in breast cancer reconstruction compared with congenital patients (19.6 percent versus 7.9 percent; p = 0.002), driven by an increased rate of wound complications (13.7 percent versus 4.4 percent; p = 0.003). Cancer-related surgery and advancing age were the only predictors of complication risk. The overall Becker expander retention rate was 24.9 percent and 46.8 percent at 150 months in the cancer reconstruction and congenital groups, respectively. Forty-seven percent of Becker implants were retained long term after congenital corrective surgery; only 25 percent were retained after postmastectomy reconstruction. Poor aesthetics was driving the exchange for fixed-volume implants, indicating that after breast cancer reconstruction, Becker expanders were being used as part of a two-stage reconstructive strategy.

Genetics and emerging treatments for Duchenne and Becker muscular dystrophy.

PubMed

Wein, Nicolas; Alfano, Lindsay; Flanigan, Kevin M

2015-06-01

Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades. Copyright © 2015 Elsevier Inc. All rights reserved.

A generalization of the Becker model in linear viscoelasticity: creep, relaxation and internal friction

NASA Astrophysics Data System (ADS)

Mainardi, Francesco; Masina, Enrico; Spada, Giorgio

2018-02-01

We present a new rheological model depending on a real parameter ν \\in [0,1], which reduces to the Maxwell body for ν =0 and to the Becker body for ν =1. The corresponding creep law is expressed in an integral form in which the exponential function of the Becker model is replaced and generalized by a Mittag-Leffler function of order ν . Then the corresponding non-dimensional creep function and its rate are studied as functions of time for different values of ν in order to visualize the transition from the classical Maxwell body to the Becker body. Based on the hereditary theory of linear viscoelasticity, we also approximate the relaxation function by solving numerically a Volterra integral equation of the second kind. In turn, the relaxation function is shown versus time for different values of ν to visualize again the transition from the classical Maxwell body to the Becker body. Furthermore, we provide a full characterization of the new model by computing, in addition to the creep and relaxation functions, the so-called specific dissipation Q^{-1} as a function of frequency, which is of particular relevance for geophysical applications.

Genetic diversity among Babesia rossi detected in naturally infected dogs in Abeokuta, Nigeria, based on 18S rRNA gene sequences.

PubMed

Takeet, Michael I; Oyewusi, Adeoye J; Abakpa, Simon A V; Daramola, Olukayode O; Peters, Sunday O

2017-03-01

Adequate knowledge of the genetic diversity among Babesia species infecting dogs is necessary for a better understanding of the epidemiology and control of canine babesiosis. Hence, this study determined the genetic diversity among the Babesia rossi detected in dogs presented for routine examination in Veterinary Hospitals in Abeokuta, Nigeria. Blood were randomly collected from 209 dogs. Field-stained thin smears were made and DNA extracted from the blood. Partial region of the 18S small subunit ribosomal RNA (rRNA) gene was amplified, sequenced and analysed. Babesia species was detected in 16 (7.7%) of the dogs by microscopy. Electrophoresed PCR products from 39 (18.66%) dogs revealed band size of 450 bp and 2 (0.95%) dogs had band size of 430 bp. The sequences obtained from 450 bp amplicon displayed homology of 99.74% (387/388) with partial sequences of 18S rRNA gene of Babesia rossi in the GeneBank. Of the two sequences that had 430 bp amplicon, one was identified as T. annulata and second as T. ovis. A significantly (p<0.05) higher prevalence of B. rossi was detected by PCR compared to microscopy. The mean PCV of Babesia infected dogs was significantly (p<0.05) lower than non-infected dogs. Phylogenetic analysis revealed minimal diversity among B. rossi with the exception of one sequence that was greatly divergent from the others. This study suggests that more than one genotype of B. rossi may be in circulation among the dog population in the study area and this may have potential implication on clinical outcome of canine babesiosis.

Genetics Home Reference: Duchenne and Becker muscular dystrophy

MedlinePlus

... Citation on PubMed Mah JK, Korngut L, Dykeman J, Day L, Pringsheim T, Jette N. A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy. Neuromuscul Disord. 2014 Jun;24(6):482-91. doi: 10.1016/j.nmd.2014.03.008. Epub 2014 Mar 22. ...

Advances in the computation of the Sjöstrand, Rossi, and Feynman distributions

DOE PAGES

Talamo, A.; Gohar, Y.; Gabrielli, F.; ...

2017-02-01

This study illustrates recent computational advances in the application of the Sjöstrand (area), Rossi, and Feynman methods to estimate the effective multiplication factor of a subcritical system driven by an external neutron source. The methodologies introduced in this study have been validated with the experimental results from the KUKA facility of Japan by Monte Carlo (MCNP6 and MCNPX) and deterministic (ERANOS, VARIANT, and PARTISN) codes. When the assembly is driven by a pulsed neutron source generated by a particle accelerator and delayed neutrons are at equilibrium, the Sjöstrand method becomes extremely fast if the integral of the reaction rate frommore » a single pulse is split into two parts. These two integrals distinguish between the neutron counts during and after the pulse period. To conclude, when the facility is driven by a spontaneous fission neutron source, the timestamps of the detector neutron counts can be obtained up to the nanosecond precision using MCNP6, which allows obtaining the Rossi and Feynman distributions.« less

Marital dissolution: an economic analysis.

PubMed

Hunter, K A

1984-01-01

A longitudinal analysis of factors affecting marital dissolution in the United States is presented using data from the Coleman-Rossi Retrospective Life History. Factors considered include labor force participation of both spouses, wage growth, size of family unit, age at marriage, and educational status. The study is based on the economic analysis approach developed by Gary S. Becker and others.

Source of lead and mineralizing brines for rossie-type Pb-Zn veins in the Frontenac axis area, New York ( USA).

USGS Publications Warehouse

Ayuso, Robert; Foley, Nora K.; Brown, C. Erwin

1987-01-01

The present study of fluid inclusions and lead isotopes was done to understand better the nature and possible source of the mineralizing solutions for both vertical and gash veins and the possible origin of the lead and zinc mineralization. Large deposits of essentially syngenetic sphalerite with minor to trace galena occur in the Proterozoic rocks of this region (e.g., Balmat-Edwards, New York). Although none of these deposits are known in the immediate vicinity of Rossie veins, the possibility that Rossie veins contain remobilized metals from these older deposits was considered.

Studying the role of dystrophin-associated proteins in influencing Becker muscular dystrophy disease severity.

PubMed

van den Bergen, J C; Wokke, B H A; Hulsker, M A; Verschuuren, J J G M; Aartsma-Rus, A M

2015-03-01

Becker muscular dystrophy is characterized by a variable disease course. Many factors have been implicated to contribute to this diversity, among which the expression of several components of the dystrophin associated glycoprotein complex. Together with dystrophin, most of these proteins anchor the muscle fiber cytoskeleton to the extracellular matrix, thus protecting the muscle from contraction induced injury, while nNOS is primarily involved in inducing vasodilation during muscle contraction, enabling adequate muscle oxygenation. In the current study, we investigated the role of three components of the dystrophin associated glycoprotein complex (beta-dystroglycan, gamma-sarcoglycan and nNOS) and the dystrophin homologue utrophin on disease severity in Becker patients. Strength measurements, data about disease course and fresh muscle biopsies of the anterior tibial muscle were obtained from 24 Becker patients aged 19 to 66. The designation of Becker muscular dystrophy in this study was based on the mutation and not on the clinical severity. Contrary to previous studies, we were unable to find a relationship between expression of nNOS, beta-dystroglycan and gamma-sarcoglycan at the sarcolemma and disease severity, as measured by muscle strength in five muscle groups and age at reaching several disease milestones. Unexpectedly, we found an inverse correlation between utrophin expression at the sarcolemma and age at reaching disease milestones. Copyright © 2015 Elsevier B.V. All rights reserved.

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.

PubMed

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S; Verschuuren, Jan J; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E; Muntoni, Francesco

2011-12-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative study on both

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials

PubMed Central

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S.; Verschuuren, Jan J.; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E.

2011-01-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative

Usefulness of sugammadex in a patient with Becker muscular dystrophy and dilated cardiomyopathy.

PubMed

Shimauchi, Tsukasa; Yamaura, Ken; Sugibe, Sayaka; Hoka, Sumio

2014-09-01

A 54-year-old patient with Becker muscular dystrophy and dilated cardiomyopathy underwent laparoscopic cholecystectomy under total intravenous anesthesia. Muscle relaxation was induced by rocuronium (0.4 mg/kg body weight) under train-of-four (TOF) ratio monitoring. The TOF ratio was 0 at intubation, and 0.2 at the end of surgery. Residual muscle relaxant activity was successfully reversed by sugammadex (2 mg/kg body weight) without any hemodynamic adverse effects (TOF ratio 1.0 at extubation). The clinical and hemodynamic findings suggest that sugammadex can be safely used in patients with Becker muscular dystrophy and dilated cardiomyopathy. Copyright © 2014. Published by Elsevier B.V.

Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment.

PubMed

Assereto, Stefania; Stringara, Silvia; Sotgia, Federica; Bonuccelli, Gloria; Broccolini, Aldobrando; Pedemonte, Marina; Traverso, Monica; Biancheri, Roberta; Zara, Federico; Bruno, Claudio; Lisanti, Michael P; Minetti, Carlo

2006-02-01

In this report, we have developed a novel method to identify compounds that rescue the dystrophin-glycoprotein complex (DGC) in patients with Duchenne or Becker muscular dystrophy. Briefly, freshly isolated skeletal muscle biopsies (termed skeletal muscle explants) from patients with Duchenne or Becker muscular dystrophy were maintained under defined cell culture conditions for a 24-h period in the absence or presence of a specific candidate compound. Using this approach, we have demonstrated that treatment with a well-characterized proteasome inhibitor, MG-132, is sufficient to rescue the expression of dystrophin, beta-dystroglycan, and alpha-sarcoglycan in skeletal muscle explants from patients with Duchenne or Becker muscular dystrophy. These data are consistent with our previous findings regarding systemic treatment with MG-132 in a dystrophin-deficient mdx mouse model (Bonuccelli G, Sotgia F, Schubert W, Park D, Frank PG, Woodman SE, Insabato L, Cammer M, Minetti C, and Lisanti MP. Am J Pathol 163: 1663-1675, 2003). Our present results may have important new implications for the possible pharmacological treatment of Duchenne or Becker muscular dystrophy in humans.

Secondary Conditions Among Males With Duchenne or Becker Muscular Dystrophy.

PubMed

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-06-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred nine caregivers of affected males (aged 3-31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The 5 most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones were found among nonambulatory males compared to ambulatory males. Attention-deficit hyperactivity disorder (ADHD) was more common in ambulatory than nonambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis.

Secondary conditions among males with Duchenne or Becker muscular dystrophy

PubMed Central

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-01-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred and nine caregivers of affected males (aged 3–31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The five most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones, were found among non-ambulatory males compared to ambulatory males. Attention deficit hyperactivity disorder was more common in ambulatory than non-ambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis. PMID:28393671

Late-onset Becker-type muscular dystrophy in a Border terrier dog.

PubMed

Jeandel, A; Garosi, L S; Davies, L; Guo, L T; Salgüero, R; Shelton, G D

2018-01-29

A 9-year-old Border terrier was presented to a referral hospital after a 1-year history of progressive stiffness and exercise intolerance. Neurological examination was consistent with a neuromuscular disorder. Serum creatine kinase activity was mildly elevated. A myopathy was suspected based on MRI findings and electrophysiological examination. Muscle histopathology was consistent with a severe non-inflammatory myopathy of a dystrophic type. Immunofluorescence and western blotting confirmed a dystrophinopathy with an 80-kDa truncated dystrophin fragment similar to Becker muscular dystrophy in people. To our knowledge, this is the first description of a late-onset Becker-type muscular dystrophy in a dog, and the first description of a dystrophinopathy in a Border terrier. Muscular dystrophy in dogs should not be ruled out based on late onset clinical signs and only mildly elevated creatine kinase. © 2018 British Small Animal Veterinary Association.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy.

PubMed

Mathews, Katherine D; Cunniff, Chris; Kantamneni, Jiji R; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F John; Sladky, John; Romitti, Paul A

2010-09-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ''definite'' or ''probable'' Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): Case Definition in Surveillance for Childhood-Onset Duchenne/Becker Muscular Dystrophy

PubMed Central

Mathews, Katherine D.; Cunniff, Chris; Kantamneni, Jiji R.; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F. John; Sladky, John; Romitti, Paul A.

2013-01-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in one of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by ≥4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either “definite” or “probable” Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982–1987) to 94% in the cohort born 2004–2009. PMID:20817884

SIBLING CONCORDANCE FOR CLINICAL FEATURES OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES

PubMed Central

PETTYGROVE, SYDNEY; LU, ZHENQIANG; ANDREWS, JENNIFER G.; MEANEY, F. JOHN; SHEEHAN, DANIEL W.; PRICE, ELINORA T.; FOX, DEBORAH J.; PANDYA, SHREE; OUYANG, LIJING; APKON, SUSAN D.; POWIS, ZOE; CUNNIFF, CHRISTOPHER

2015-01-01

Introduction The correlation of markers of disease severity among brothers with Duchenne or Becker muscular dystrophy has implications for clinical guidance and clinical trials. Methods Sibling pairs with Duchenne or Becker muscular dystrophy (n = 60) were compared for ages when they reached clinical milestones of disease progression, including ceased ambulation, scoliosis of ≥ 20°, and development of cardiomyopathy. Results The median age at which younger brothers reached each milestone, compared with their older brothers ranged from 25 months younger for development of cardiomyopathy to 2 months older for ceased ambulation. For each additional month of ambulation by the older brother, the hazard of ceased ambulation by the younger brother decreased by 4%. Conclusions The ages when siblings reach clinical milestones of disease vary widely between siblings. However, the time to ceased ambulation for older brothers predicts the time to ceased ambulation for their younger brothers. PMID:24030636

From cosmic ray physics to cosmic ray astronomy: Bruno Rossi and the opening of new windows on the universe

NASA Astrophysics Data System (ADS)

Bonolis, Luisa

2014-01-01

Bruno Rossi is considered one of the fathers of modern physics, being also a pioneer in virtually every aspect of what is today called high-energy astrophysics. At the beginning of 1930s he was the pioneer of cosmic ray research in Italy, and, as one of the leading actors in the study of the nature and behavior of the cosmic radiation, he witnessed the birth of particle physics and was one of the main investigators in this fields for many years. While cosmic ray physics moved more and more towards astrophysics, Rossi continued to be one of the inspirers of this line of research. When outer space became a reality, he did not hesitate to leap into this new scientific dimension. Rossi's intuition on the importance of exploiting new technological windows to look at the universe with new eyes, is a fundamental key to understand the profound unity which guided his scientific research path up to its culminating moments at the beginning of 1960s, when his group at MIT performed the first in situ measurements of the density, speed and direction of the solar wind at the boundary of Earth's magnetosphere, and when he promoted the search for extra-solar sources of X rays. A visionary idea which eventually led to the breakthrough experiment which discovered Scorpius X-1 in 1962, and inaugurated X-ray astronomy.

Dystrophin insufficiency causes a Becker muscular dystrophy-like phenotype in swine

USDA-ARS?s Scientific Manuscript database

Duchenne muscular dystrophy (DMD) is caused by a dystrophin deficiency while Becker MD is caused by a dystrophin insufficiency or expression of a partially functional dystrophin protein. Deficiencies in existing mouse and dog models necessitate the development of a novel large animal model. Our pu...

Genetic diagnosis of Duchenne and Becker muscular dystrophy using multiplex ligation-dependent probe amplification in Rwandan patients.

PubMed

Uwineza, Annette; Hitayezu, Janvier; Murorunkwere, Seraphine; Ndinkabandi, Janvier; Kalala Malu, Celestin Kaputu; Caberg, Jean Hubert; Dideberg, Vinciane; Bours, Vincent; Mutesa, Leon

2014-04-01

Duchenne and Becker muscular dystrophies are the most common clinical forms of muscular dystrophies. They are genetically X-linked diseases caused by a mutation in the dystrophin (DMD) gene. A genetic diagnosis was carried out in six Rwandan patients presenting a phenotype of Duchenne and Becker muscular dystrophies and six asymptomatic female carrier relatives using multiplex ligation-dependent probe amplification (MLPA). Our results revealed deletion of the exons 48-51 in one patient, an inherited deletion of the exons 8-21 in two brothers and a de novo deletion of the exons 46-50 in the fourth patient. No copy number variation was found in two patients. Only one female carrier presented exon deletion in the DMD gene. This is the first cohort of genetic analysis in Rwandan patients affected by Duchenne and Becker muscular dystrophies. This report confirmed that MLPA assay can be easily implemented in low-income countries.

Brain natriuretic peptide is not predictive of dilated cardiomyopathy in Becker and Duchenne muscular dystrophy patients and carriers.

PubMed

Schade van Westrum, Steven; Dekker, Lukas; de Haan, Rob; Endert, Erik; Ginjaar, Ieke; de Visser, Marianne; van der Kooi, Anneke

2013-07-16

Cardiomyopathy is reported in Duchenne and Becker muscle dystrophy patients and female carriers. Brain Natriuretic peptide (BNP) is a hormone produced mainly by ventricular cardiomyocytes and its production is up regulated in reaction to increased wall stretching. N-terminal-proBNP (NT-proBNP) has been shown to be a robust laboratory parameter to diagnose and monitor cardiac failure, and it may be helpful to screen for asymptomatic left ventricular dysfunction. Therefore we tested whether NT-proBNP can distinguish patients with Duchenne or Becker muscular dystrophy patients and carriers of a dystrophin mutation with a dilated cardiomyopathy from those without. In a cohort of Duchenne and Becker muscle dystrophy patients (n = 143) and carriers (n = 219) NT-proBNP was measured, and echocardiography was performed to diagnose dilated cardiomyopathy (DCM). In total sixty-one patients (17%) fulfilled the criteria for DCM, whereas 283 patients (78%) had an elevated NT-pro BNP. The sensitivity of NT-proBNP for DCM in patients or carriers was 85%, the specificity 23%, area under the ROC-curve = 0.56. In the specified subgroups there was also no association. Measurement of NT-pro BNP in patients suffering from Duchenne or Becker muscular dystrophy and carriers does not distinguish between those with and without dilated cardiomyopathy.

Bruno Rossi and the Racial Laws of Fascist Italy

NASA Astrophysics Data System (ADS)

Bonolis, Luisa

2011-03-01

Bruno Rossi (1905-1993), one of the giants of 20th-century physics, was a pioneer in cosmic-ray physics and virtually every other aspect of high-energy astrophysics. His scientific career began at the University of Florence in 1928 and continued at the University of Padua until 1938, when the Fascist anti-Semitic racial laws were passed in Italy. He was dismissed from his professorship and was forced to emigrate, as described in unpublished letters and documents that display the international character of physics and physicists. His young bride Nora Lombroso, his love of physics, and the solidarity of the physics community gave him the courage to begin a new life in Copenhagen, Manchester, and in the New World at the University of Chicago, Cornell University, Los Alamos, and after the Second World War at the Massachusetts Institute of Technology where he became the center of a worldwide research network.

Breast reconstruction using permanent Becker expander implants: an 18 year experience.

PubMed

Goh, S C J; Thorne, A L; Williams, G; Laws, S A M; Rainsbury, R M

2012-12-01

Single-stage reconstruction using permanent expander implants is an established technique following mastectomy. Short and long-term outcome data following breast reconstruction using Becker tissue expanders is limited. A retrospective case note review of patients undergoing expander-based procedures between 1989 and 2007 was undertaken. Data recorded included postoperative symptoms and complications, the use of radiotherapy, revisional surgery, and device failure. Three hundred and thirteen expanders were used in 276 patients with a mean age of 48.3 (17-78) years, over the 18 year study period. The mean follow up period was 64.6 (1-199) months. 256 Becker expanders were used during 175 latissimus dorsi (LD) and 52 subpectoral (SP) reconstructions, 13 contralateral augmentations and 16 implant replacements. The postoperative infection rate was 5.8%, leading to an expander loss rate of 3.8%. The use of prophylactic antibiotics was associated with an increased postoperative infection rate (p = 0.046). Six haematomas (2.5%) and 12 cases of skin envelope necrosis (5.0%) required unscheduled intervention. Symptoms of pain, distortion and hardness were experienced by 21.3% of patients, and radiotherapy was associated with a significantly higher risk of adverse symptoms (p < 0.0001). No patient developed symptomatic implant rupture or silicone granuloma but 17.9% of reconstructions underwent revisional surgery, the rate being highest following SP reconstruction (p = 0.029). Nine patients developed injection port complications (3.8%), and the overall device failure rate was 1.3%. The original expander has been retained by 74.2% of women. The Becker permanent expander is a reliable implant associated with a low complication rate and a high retention rate when used during breast reconstruction. Copyright © 2012 Elsevier Ltd. All rights reserved.

Complementary and alternative medicine for Duchenne and Becker muscular dystrophies: characteristics of users and caregivers.

PubMed

Zhu, Yong; Romitti, Paul A; Conway, Kristin M; Andrews, Jennifer; Liu, Ke; Meaney, F John; Street, Natalie; Puzhankara, Soman; Druschel, Charlotte M; Matthews, Dennis J

2014-07-01

Complementary and alternative medicine is frequently used in the management of chronic pediatric diseases, but little is known about its use by those with Duchenne or Becker muscular dystrophy. Complementary and alternative medicine use by male patients with Duchenne or Becker muscular dystrophy and associations with characteristics of male patients and their caregivers were examined through interviews with 362 primary caregivers identified from the Muscular Dystrophy Surveillance, Tracking, and Research Network. Overall, 272 of the 362 (75.1%) primary caregivers reported that they had used any complementary and alternative medicine for the oldest Muscular Dystrophy Surveillance, Tracking, and Research Network male in their family. The most commonly reported therapies were from the mind-body medicine domain (61.0%) followed by those from the biologically based practice (39.2%), manipulative and body-based practice (29.3%), and whole medical system (6.9%) domains. Aquatherapy, prayer and/or blessing, special diet, and massage were the most frequently used therapies. Compared with nonusers, male patients who used any therapy were more likely to have an early onset of symptoms and use a wheel chair; their caregivers were more likely to be non-Hispanic white. Among domains, associations were observed with caregiver education and family income (mind-body medicines [excluding prayer and/or blessing only] and whole medical systems) and Muscular Dystrophy Surveillance, Tracking, and Research Network site (biologically based practices and mind-body medicines [excluding prayer and/or blessing only]). Complementary and alternative medicine use was common in the management of Duchenne and Becker muscular dystrophies among Muscular Dystrophy Surveillance, Tracking, and Research Network males. This widespread use suggests further study to evaluate the efficacy of integrating complementary and alternative medicine into treatment regimens for Duchenne and Becker muscular

Becker muscular dystrophy with widespread muscle hypertrophy and a non-sense mutation of exon 2.

PubMed

Witting, N; Duno, M; Vissing, J

2013-01-01

Becker muscular dystrophy features progressive proximal weakness, wasting and often focal hypertrophy. We present a patient with pain and cramps from adolescence. Widespread muscle hypertrophy, preserved muscle strength and a 10-20-fold raised CPK were noted. Muscle biopsy was dystrophic, and Western blot showed a 95% reduction of dystrophin levels. Genetic analyses revealed a non-sense mutation in exon 2 of the dystrophin gene. This mutation is predicted to result in a Duchenne phenotype, but resulted in a mild Becker muscular dystrophy with widespread muscle hypertrophy. We suggest that this unusual phenotype is caused by translation re-initiation downstream from the mutation site. Copyright © 2012 Elsevier B.V. All rights reserved.

Neutron coincidence counting based on time interval analysis with one- and two-dimensional Rossi-alpha distributions: an application for passive neutron waste assay

NASA Astrophysics Data System (ADS)

Bruggeman, M.; Baeten, P.; De Boeck, W.; Carchon, R.

1996-02-01

Neutron coincidence counting is commonly used for the non-destructive assay of plutonium bearing waste or for safeguards verification measurements. A major drawback of conventional coincidence counting is related to the fact that a valid calibration is needed to convert a neutron coincidence count rate to a 240Pu equivalent mass ( 240Pu eq). In waste assay, calibrations are made for representative waste matrices and source distributions. The actual waste however may have quite different matrices and source distributions compared to the calibration samples. This often results in a bias of the assay result. This paper presents a new neutron multiplicity sensitive coincidence counting technique including an auto-calibration of the neutron detection efficiency. The coincidence counting principle is based on the recording of one- and two-dimensional Rossi-alpha distributions triggered respectively by pulse pairs and by pulse triplets. Rossi-alpha distributions allow an easy discrimination between real and accidental coincidences and are aimed at being measured by a PC-based fast time interval analyser. The Rossi-alpha distributions can be easily expressed in terms of a limited number of factorial moments of the neutron multiplicity distributions. The presented technique allows an unbiased measurement of the 240Pu eq mass. The presented theory—which will be indicated as Time Interval Analysis (TIA)—is complementary to Time Correlation Analysis (TCA) theories which were developed in the past, but is from the theoretical point of view much simpler and allows a straightforward calculation of deadtime corrections and error propagation. Analytical expressions are derived for the Rossi-alpha distributions as a function of the factorial moments of the efficiency dependent multiplicity distributions. The validity of the proposed theory is demonstrated and verified via Monte Carlo simulations of pulse trains and the subsequent analysis of the simulated data.

Adult patient with Becker dystrophy undergoing orthopedic surgery: an anesthesia challenge.

PubMed

Parish, Masoud; Farzin, Haleh

2018-01-01

Muscular dystrophies are considered to be a series of neuromuscular diseases with genetic causes and are characterized by progressive muscle weakness and degeneration of the skeletal muscle. The case of an adult man with Becker dystrophy referred for repair of the patella tendon tearing and patella fracture is described. He underwent successful surgery using total intravenous anesthesia without any complications.

Life-threatening Arrhythmias in a Becker Muscular Dystrophy Family due to the Duplication of Exons 3-4 of the Dystrophin Gene.

PubMed

Ishizaki, Masatoshi; Fujimoto, Akiko; Ueyama, Hidetsugu; Nishida, Yasuto; Imamura, Shigehiro; Uchino, Makoto; Ando, Yukio

2015-01-01

We herein present a report of three patients with Becker muscular dystrophy in the same family who developed complete atrioventricular block or ventricular tachycardia with severe cardiomyopathy. Our cases became unable to walk in their teens, and were introduced to mechanical ventilation due to respiratory muscle weakness in their twenties and thirties. In all three cases, a medical device such as a permanent cardiac pacemaker or an implantable cardiac defibrillator was considered to be necessary. The duplication of exons 3-4 in the dystrophin gene was detected in two of the patients. In patients with Becker muscular dystrophy, complete atrioventricular block or ventricular tachycardia within a family has rarely been reported. Thus attention should be paid to the possibility of severe arrhythmias in the severe phenotype of Becker muscular dystrophy.

AAMD-Becker Reading-Free Vocational Interest Inventory Manual [and Male and Female Inventories].

ERIC Educational Resources Information Center

Becker, Ralph L.

The AAMD-Becker Reading-Free Vocational Interest Inventory is a non-reading vocational preference test for use with mentally retarded persons, particularly the educable mentally retarded at the high school level. Illustrations having occupational significance are presented in forced-choice format for selections. The instrument helps to identify…

Risk Factors for First Fractures Among Males With Duchenne or Becker Muscular Dystrophy.

PubMed

James, Katherine A; Cunniff, Christopher; Apkon, Susan D; Mathews, Katherine; Lu, Zhenqiang; Holtzer, Caleb; Pandya, Shree; Ciafaloni, Emma; Miller, Lisa

2015-09-01

Fractures are a significant concern for individuals with Duchenne/Becker muscular dystrophy with 21% to 44% of males experiencing a fracture. Factors that increase or decrease the risk for fracture have been suggested in past research, although statistical risk has not been determined. In this retrospective cohort study, we used the Muscular Dystrophy Surveillance, Tracking and Research Network cohort, a large, population-based sample to identify risk factors associated with first fractures in patients with Duchenne or Becker muscular dystrophy. Our study cohort included males with Duchenne or Becker muscular dystrophy born between 1982 and 2006 who resided in Arizona, Colorado, Georgia, Iowa, and Western New York, retrospectively identified and followed through 2010. We utilized a multivariate Cox proportional hazard model to determine hazard ratios for relevant factors associated with first fracture risk including race/ethnicity, surveillance site, ambulation status, calcium/vitamin D use and duration, bisphosphonate use and duration, and corticosteroid use and duration. Of 747 cases, 249 had at least 1 fracture (33.3%). Full-time wheelchair use increased the risk of first fracture by 75% for every 3 months of use (hazard ratio=1.75, 95% confidence interval, 1.14, 2.68), but corticosteroid use, bisphosphonate use, and calcium/vitamin D use did not significantly affect risk in the final adjusted model. In this cohort, first fractures were common and full-time wheelchair use, but not corticosteroid use, was identified as a risk factor. The impact of prevention measures should be more thoroughly assessed. Fractures are a significant concern for individuals with dystrophinopathies, but the contribution of various risk factors has not been consistently demonstrated.

Summary of Symposium on Cloud Systems, Hurricanes and TRMM: Celebration of Dr. Joanne Simpson's Career, The First Fifty Years

NASA Technical Reports Server (NTRS)

Tao, W.-K.; Adler, R.; Braun, S.; Einaudi, F.; Ferrier, B.; Halverson, J.; Heymsfield, G.; Kummerow, C.; Negri, A.; Kakar, R.;

TNF-α-Induced microRNAs Control Dystrophin Expression in Becker Muscular Dystrophy.

PubMed

Fiorillo, Alyson A; Heier, Christopher R; Novak, James S; Tully, Christopher B; Brown, Kristy J; Uaesoontrachoon, Kitipong; Vila, Maria C; Ngheim, Peter P; Bello, Luca; Kornegay, Joe N; Angelini, Corrado; Partridge, Terence A; Nagaraju, Kanneboyina; Hoffman, Eric P

2015-09-08

The amount and distribution of dystrophin protein in myofibers and muscle is highly variable in Becker muscular dystrophy and in exon-skipping trials for Duchenne muscular dystrophy. Here, we investigate a molecular basis for this variability. In muscle from Becker patients sharing the same exon 45-47 in-frame deletion, dystrophin levels negatively correlate with microRNAs predicted to target dystrophin. Seven microRNAs inhibit dystrophin expression in vitro, and three are validated in vivo (miR-146b/miR-374a/miR-31). microRNAs are expressed in dystrophic myofibers and increase with age and disease severity. In exon-skipping-treated mdx mice, microRNAs are significantly higher in muscles with low dystrophin rescue. TNF-α increases microRNA levels in vitro whereas NFκB inhibition blocks this in vitro and in vivo. Collectively, these data show that microRNAs contribute to variable dystrophin levels in muscular dystrophy. Our findings suggest a model where chronic inflammation in distinct microenvironments induces pathological microRNAs, initiating a self-sustaining feedback loop that exacerbates disease progression. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

Rossi X-Ray Timing Explorer All-Sky Monitor Localization of SGR 1627-41

NASA Astrophysics Data System (ADS)

Smith, Donald A.; Bradt, Hale V.; Levine, Alan M.

1999-07-01

The fourth unambiguously identified soft gamma repeater (SGR), SGR 1627-41, was discovered with the BATSE instrument on 1998 June 15. Interplanetary Network (IPN) measurements and BATSE data constrained the location of this new SGR to a 6° segment of a narrow (19") annulus. We present two bursts from this source observed by the All-Sky Monitor (ASM) on the Rossi X-Ray Timing Explorer. We use the ASM data to further constrain the source location to a 5' long segment of the BATSE/IPN error box. The ASM/IPN error box lies within 0.3 arcmin of the supernova remnant G337.0-0.1. The probability that a supernova remnant would fall so close to the error box purely by chance is ~5%.

Kinetic parameters of the GUINEVERE reference configuration in VENUS-F reactor obtained from a pile noise experiment using Rossi and Feynman methods

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

Geslot, Benoit; Pepino, Alexandra; Blaise, Patrick

A pile noise measurement campaign has been conducted by the CEA in the VENUS-F reactor (SCK-CEN, Mol Belgium) in April 2011 in the reference critical configuration of the GUINEVERE experimental program. The experimental setup made it possible to estimate the core kinetic parameters: the prompt neutron decay constant, the delayed neutron fraction and the generation time. A precise assessment of these constants is of prime importance. In particular, the effective delayed neutron fraction is used to normalize and compare calculated reactivities of different subcritical configurations, obtained by modifying either the core layout or the control rods position, with experimental onesmore » deduced from the analysis of measurements. This paper presents results obtained with a CEA-developed time stamping acquisition system. Data were analyzed using Rossi-α and Feynman-α methods. Results were normalized to reactor power using a calibrated fission chamber with a deposit of Np-237. Calculated factors were necessary to the analysis: the Diven factor was computed by the ENEA (Italy) and the power calibration factor by the CNRS/IN2P3/LPC Caen. Results deduced with both methods are consistent with respect to calculated quantities. Recommended values are given by the Rossi-α estimator, that was found to be the most robust. The neutron generation time was found equal to 0.438 ± 0.009 μs and the effective delayed neutron fraction is 765 ± 8 pcm. Discrepancies with the calculated value (722 pcm, calculation from ENEA) are satisfactory: -5.6% for the Rossi-α estimate and -2.7% for the Feynman-α estimate. (authors)« less

Muscle MRS detects elevated PDE/ATP ratios prior to fatty infiltration in Becker muscular dystrophy.

PubMed

Wokke, B H; Hooijmans, M T; van den Bergen, J C; Webb, A G; Verschuuren, J J; Kan, H E

2014-11-01

Becker muscular dystrophy (BMD) is characterized by progressive muscle weakness. Muscles show structural changes (fatty infiltration, fibrosis) and metabolic changes, both of which can be assessed using MRI and MRS. It is unknown at what stage of the disease process metabolic changes arise and how this might vary for different metabolites. In this study we assessed metabolic changes in skeletal muscles of Becker patients, both with and without fatty infiltration, quantified via Dixon MRI and (31) P MRS. MRI and (31) P MRS scans were obtained from 25 Becker patients and 14 healthy controls using a 7 T MR scanner. Five lower-leg muscles were individually assessed for fat and muscle metabolite levels. In the peroneus, soleus and anterior tibialis muscles with non-increased fat levels, PDE/ATP ratios were higher (P < 0.02) compared with controls, whereas in all muscles with increased fat levels PDE/ATP ratios were higher compared with healthy controls (P ≤ 0.05). The Pi /ATP ratio in the peroneus muscles was higher in muscles with increased fat fractions (P = 0.005), and the PCr/ATP ratio was lower in the anterior tibialis muscles with increased fat fractions (P = 0.005). There were no other significant changes in metabolites, but an increase in tissue pH was found in all muscles of the total group of BMD patients in comparison with healthy controls (P < 0.05). These findings suggest that (31) P MRS can be used to detect early changes in individual muscles of BMD patients, which are present before the onset of fatty infiltration. Copyright © 2014 John Wiley & Sons, Ltd.

A Cross-Sectional Study of School Experiences of Boys with Duchenne and Becker Muscular Dystrophy

ERIC Educational Resources Information Center

Soim, Aida; Lamb, Molly; Campbell, Kimberly; Pandya, Shree; Peay, Holly; Howard, James F., Jr.; Fox, Deborah

2016-01-01

The objectives of this study were to investigate types of supportive school services received and factors related to provision of these services. We conducted a cross-sectional study to describe the school experience of males with Duchenne and Becker muscular dystrophies. Study subjects were identified through the Muscular Dystrophy Surveillance,…

Lydia Becker's "School for Science": a challenge to domesticity.

PubMed

Parker, J E

2001-01-01

Lydia Becker (1827-1890) is known as a leader of the Women's Suffrage Movement but little is known about her work to include women and girls in science. Before her energy was channelled into politics, she aimed to have a scientific career. Mid-Victorian Britain was a period in which women's intellect and potential were widely debated, and in which the dominant ideology was that their primary role in life was that of wife and mother. Science was widely regarded as a "masculine" subject which women were deliberately discouraged from studying. The author concentrates on the two main areas in which important contributions were made, the British Association for the Advancement of Science, and the Manchester School Board.

Propofol-induced violent coughing in a patient with Becker's muscular dystrophy

PubMed Central

Jain, Amit

2011-01-01

Propofol anesthesia is often associated with decreased incidence of gagging, coughing or laryngospasm, and provides intense suppression on airway reflex during tracheal intubation and laryngeal mask airway insertion. Propofol pretreatment is also effective in reducing the occurrence of opioid-induced coughing. These benefits are often attributed to bronchodilator and sedative effects of propofol. However, severe coughing following sedative doses of 1% propofol has not been reported so far. We report a rare case of violent coughing following low-dose propofol infusion in a patient with Becker's muscular dystrophy. PMID:21845012

Repair of an inguinoscrotal hernia in a patient with Becker muscular dystrophy.

PubMed

Tatulli, F; Caraglia, A; Delcuratolo, A; Cassano, S; Chetta, G S

2017-01-01

Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier. He had a left-sided sizable inguinoscrotal hernia with repeat episodes of incarceration. An elective mesh repair with suction drainage was accomplished under selective spinal anesthesia. The post-operative course was uneventful. A few inguinal hernia repairs require admission due to peculiarities such as extensive scrotal hernias requiring suction drainage. Muscular dystrophies are inherited disorders with no cure and no two dystrophy patients are exactly alike, therefore the health issues will be different for each individual. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards the successful elective mesh repair with suction drainage of a large left-sided inguino-scrotal hernia in a 48 y. o. male patient affected by Becker muscular dystrophy by selective spinal anesthesia obtained by 10 milligrams of hyperbaric bupivacaine. Effective mesh repair with suction drainage of large inguinal hernias under spinal anesthesia can be achieved in patients affected by muscular dystrophy.

Molecular detection of Babesia rossi and Hepatozoon sp. in African wild dogs (Lycaon pictus) in South Africa.

PubMed

Matjila, Paul Tshepo; Leisewitz, Andrew L; Jongejan, Frans; Bertschinger, Henk J; Penzhorn, Barend L

2008-10-20

Blood specimens from wild dogs (n=301) were obtained from De Wildt Cheetah and Wildlife Centre (Pretoria) and five game reserves (4 in the North-West Province and 1 in Limpopo Province), South Africa. Specimens were screened for Babesia, Theileria, Hepatozoon and Ehrlichia/Anaplasma species using PCR and Reverse Line Blot (RLB) assays. Positive results were obtained in 18 (6%) wild dogs. Sixteen specimens were found positive for Babesia rossi and two dogs were Hepatozoon sp. positive. It appears that these tick-borne pathogens are not widely distributed in wild dog populations.

Magnetic resonance imaging phenotyping of Becker muscular dystrophy.

PubMed

Faridian-Aragh, Neda; Wagner, Kathryn R; Leung, Doris G; Carrino, John A

2014-12-01

There is little information on magnetic resonance imaging (MRI) phenotypes of Becker muscular dystrophy (BMD). This study presents the MRI phenotyping of the upper and lower extremities of a large cohort of BMD patients. In this retrospective study, MRI images of 33 BMD subjects were evaluated for severity, distribution, and symmetry of involvement. Teres major, triceps long head, biceps brachii long head, gluteus maximus, gluteus medius, vasti, adductor longus, adductor magnus, semitendinosus, semimembranosus, and biceps femoris muscles showed the highest severity and frequency of involvement. All analyzed muscles had a high frequency of symmetric involvement. There was significant variability of involvement between muscles within some muscle groups, most notably the arm abductors, posterior arm muscles, medial thigh muscles, and lateral hip rotators. This study showed a distinctive pattern of involvement of extremity muscles in BMD subjects. © 2014 Wiley Periodicals, Inc.

Risk Factors Associated With Complication Rates of Becker-Type Expander Implants in Relation to Implant Survival: Review of 314 Implants in 237 Patients.

PubMed

Taboada-Suarez, Antonio; Brea-García, Beatriz; Magán-Muñoz, Fernando; Couto-González, Iván; González-Álvarez, Eduardo

2015-12-01

Although autologous tissue reconstruction is the best option for breast reconstruction, using implants is still a reliable and simple method, offering acceptable aesthetic results. Becker-type implants are permanent implants that offer a 1-stage reconstructive option. A retrospective study was carried out in our center reviewing the clinical reports of 237 patients, in whom a total of 314 Becker-type prostheses were implanted. Overall survival was calculated using a Kaplan-Meier estimate. Cox proportional hazard models were used to calculate adjusted hazard ratios. At the end of the study, 214 expanders (68.15%) presented no complications, 40 (12.47%) developed significant capsular contracture, in 27 (8.60%) infection occurred, 24 (7.64%) suffered minor complications, and 9 (2.87%) ruptured. The mean survival time of the expanders was 120.41 months (95% CI: 109.62, 131.19). Radiotherapy, chemotherapy, high Molecular Immunology Borstel, age, mastectomy performed previously to the implant, ductal carcinoma, advanced tumoral stage, experience of the surgeon, and Becker 35-type implants were significantly related to a high number of complications in relation to the survival of the implants. Cox regression analysis revealed that the main risk factors for the survival of expander implants included radiotherapy and surgeon experience. The complication hazard ratio or relative risk caused by these 2 factors was 1.976 and 1.680, respectively. One-stage reconstruction using Becker-type expanders is an appropriate, simple, and reliable option in delayed breast reconstruction in patients who have not received radiotherapy and as long as the procedure is carried out by surgeons skilled in the technique.

The response of the pituitary-adrenal and pituitary-thyroidal axes to the plasma glucose perturbations in Babesia canis rossi babesiosis.

PubMed

Schoeman, J P; Herrtage, M E

2007-12-01

This prospective, cross-sectional, interventional study was designed to determine the association between the hormones of the pituitary-adrenal and pituitary-thyroid axes and other clinical parameters with the blood glucose perturbations in dogs with naturally occurring Babesia canis rossi babesiosis. Thirty-six dogs with canine babesiosis were studied. Blood samples were obtained from the jugular vein in each dog prior to treatment at admission to hospital and serum endogenous adrenocorticotrophic hormone (ACTH), pre-ACTH cortisol, thyroxine, free thyroxine and TSH concentrations were measured. Immediately thereafter each dog was injected intravenously with 5 microg/kg of ACTH (tetracosactrin). A 2nd blood sample was taken 1 hour later for serum post-ACTH cortisol measurement. Three patient groups were recruited: hypoglycaemic dogs (glucose < 3.3 mmol/l, n = 12); normoglycaemic dogs (glucose 3.3-5.5 mmol/l, n = 12); hyperglycaemic dogs (glucose > 5.5 mmol/l, n = 12). Basal and post-ACTH serum cortisol concentrations were significantly higher in hypoglycaemic dogs, whereas body temperature, serum thyroxine and free thyroxine were significantly lower in hypoglycaemic dogs. Haematocrit was significantly lower in both hypo-and hyperglycaemic dogs compared with normoglycaemic dogs. Low blood glucose concentrations were significantly associated with high basal and post-ACTH cortisol concentrations and with low serum thyroxine and free thyroxine concentrations in dogs suffering from B. canis rossi babesiosis.

Muscular dystrophy in a dog resembling human becker muscular dystrophy.

PubMed

Baroncelli, A B; Abellonio, F; Pagano, T B; Esposito, I; Peirone, B; Papparella, S; Paciello, O

2014-05-01

A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. Copyright © 2014 Elsevier Ltd. All rights reserved.

Becker's rational addiction theory: An empirical test with price elasticities for distilled spirits in Denmark 1911-31.

PubMed

Skog, Ole-Jørgen; Melberg, Hans Olav

2006-10-01

To test an implication of Becker's rational addiction theory, namely that price changes will lead both to simultaneous consumption changes as well as lagged changes (and potentially also immediate changes if future changes in prices are anticipated). Time-series analysis, first of aggregate sales of distilled spirits and prices, controlled for gross national product (GNP), and secondly of deaths from delirium tremens. Denmark 1911-31. Price changes were very large in the period 1916-18 due to shortages during World War I, and the Danish case can be conceived as a natural experiment. No evidence for lagged price effects in the expected direction was found. On the contrary, the evidence pointed in the opposite direction. The immediate reduction in sales following rising prices are, to some degree, counteracted by an adjustment in the opposite direction the following year. The delirium tremens data confirm this pattern. Becker's theory is not confirmed. Several possible explanations are discussed. If the pattern observed in these data is representative of a more general mechanism, current price elasticity estimates may be too high, by ignoring lagged compensatory effects.

Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study.

PubMed

Nakamura, Yuko; Saito, Yoshiaki; Kubota, Norika; Matsumura, Wataru; Hosoda, Chika; Tamasaki-Kondo, Akiko; Nishimura, Yoko; Sunada, Yoshihide; Fukada, Masuyuki; Ohno, Takako; Maegaki, Yoshihiro; Matsuo, Masafumi; Tokita, Yasuko

2018-03-08

To report on sleep hypercapnia in Becker muscular dystrophy (BMD) at earlier stages than ever recognized. This retrospective study examined nocturnal hypercapnia in six young Becker muscular dystrophy (BMD) patients with deletions of one or more exons of DMD gene. Clinical information, consecutive data on forced vital capacity (FVC%), forced expiratory volume in one second (FEV1%), peak expiratory flow (PEF%), peak cough flow (PCF), average PCO 2 in all-night monitoring, and left ventricular ejection fraction (LVEF) were reviewed. In five BMD patients, including three who were still ambulant, nocturnal average PCO 2 was elevated to >45 mmHg at 12-31 years of age. Noninvasive positive pressure ventilation was initiated in four patients. Gradual declines in FVC% and PEF% were evident in one BMD patient with exon 3-7 deletion, whereas these functions did not change in the remaining BMD patients. PCF, FEV1%, and LVEF were less informative for the assessment of respiratory function in this patient series. Sleep hypercapnia was present in certain BMD patients, which was unexpected from the routine pulmonary function tests. Individualized assessment of nocturnal PCO 2 , partly based on the deletion types, should be further explored in the clinical practice of BMD patients. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Becker muscular dystrophy severity is linked to the structure of dystrophin.

PubMed

Nicolas, Aurélie; Raguénès-Nicol, Céline; Ben Yaou, Rabah; Ameziane-Le Hir, Sarah; Chéron, Angélique; Vié, Véronique; Claustres, Mireille; Leturcq, France; Delalande, Olivier; Hubert, Jean-François; Tuffery-Giraud, Sylvie; Giudice, Emmanuel; Le Rumeur, Elisabeth

2015-03-01

In-frame exon deletions of the Duchenne muscular dystrophy (DMD) gene produce internally truncated proteins that typically lead to Becker muscular dystrophy (BMD), a milder allelic disorder of DMD. We hypothesized that differences in the structure of mutant dystrophin may be responsible for the clinical heterogeneity observed in Becker patients and we studied four prevalent in-frame exon deletions, i.e. Δ45-47, Δ45-48, Δ45-49 and Δ45-51. Molecular homology modelling revealed that the proteins corresponding to deletions Δ45-48 and Δ45-51 displayed a similar structure (hybrid repeat) than the wild-type dystrophin, whereas deletions Δ45-47 and Δ45-49 lead to proteins with an unrelated structure (fractional repeat). All four proteins in vitro expressed in a fragment encoding repeats 16-21 were folded in α-helices and remained highly stable. Refolding dynamics were slowed and molecular surface hydrophobicity were higher in fractional repeat containing Δ45-47 and Δ45-49 deletions compared with hybrid repeat containing Δ45-48 and Δ45-51 deletions. By retrospectively collecting data for a series of French BMD patients, we showed that the age of dilated cardiomyopathy (DCM) onset was delayed by 11 and 14 years in Δ45-48 and Δ45-49 compared with Δ45-47 patients, respectively. A clear trend toward earlier wheelchair dependency (minimum of 11 years) was also observed in Δ45-47 and Δ45-49 patients compared with Δ45-48 patients. Muscle dystrophin levels were moderately reduced in most patients without clear correlation with the deletion type. Disease progression in BMD patients appears to be dependent on the deletion itself and associated with a specific structure of dystrophin at the deletion site. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Cost-Effectiveness Analysis of Diagnosis of Duchenne/Becker Muscular Dystrophy in Colombia.

PubMed

Atehortúa, Sara C; Lugo, Luz H; Ceballos, Mateo; Orozco, Esteban; Castro, Paula A; Arango, Juan C; Mateus, Heidi E

2018-03-09

To determine the cost-effectiveness ratio of different courses of action for the diagnosis of Duchenne or Becker muscular dystrophy in Colombia. The cost-effectiveness analysis was performed from the Colombian health system perspective. Decision trees were constructed, and different courses of action were compared considering the following tests: immunohistochemistry (IHC), Western blot (WB), multiplex polymerase chain reaction, multiplex ligation-dependent probe amplification (MLPA), and the complete sequencing of the dystrophin gene. The time horizon matched the duration of sample extraction and analysis. Transition probabilities were obtained from a systematic review. Costs were constructed with a type-case methodology using the consensus of experts and the valuation of resources from consulting laboratories and the 2001 Social Security Institute cost manual. Deterministic sensitivity and scenario analyses were performed with one or more unavailable alternatives. Costs were converted from Colombian pesos to US dollars using the 2014 exchange rate. In the base case, WB was the dominant strategy, with a cost of US $419.07 and a sensitivity of 100%. This approach remains the dominant strategy down to a 98.2% sensitivity and while costs do not exceed US $837.38. If WB was not available, IHC had the best cost-effectiveness ratio, followed by MLPA and sequencing. WB is a cost-effective alternative for the diagnosis of patients suspected of having Duchenne or Becker muscular dystrophy in the Colombian health system. The IHC test is rated as the second-best detection method. If these tests are not available, MLPA followed by sequencing would be the most cost-effective alternative. Copyright © 2018. Published by Elsevier Inc.

A Case of Refractory Heart Failure in Becker Muscular Dystrophy Improved With Corticosteroid Therapy.

PubMed

Nakamura, Makiko; Sunagawa, Osahiko; Hokama, Ryo; Tsuchiya, Hiroyuki; Miyara, Takafumi; Taba, Yoji; Touma, Takashi

2016-09-28

The patient was a 26 year-old man who was referred to our hospital in June 2011 because of severe heart failure. At age 24 years, he was found to have Becker muscular dystrophy. He received enalapril for cardiac dysfunction; however, he had worsening heart failure and was thus referred to our hospital. Echocardiography showed enlargement of the left ventricle, with a diastolic dimension of 77 mm and ejection fraction of 19%. His condition improved temporarily after an infusion of dobutamine and milrinone. He was then administered amiodarone for ventricular tachycardia; however, he subsequently developed hemoptysis. Amiodarone was discontinued and corticosteroid pulse therapy was administered followed by oral prednisolone (PSL). His creatinine phosphokinase (CPK) level and cardiomegaly improved after the corticosteroid therapy. The PSL dose was reduced gradually, bisoprolol was introduced, and the catecholamine infusion was tapered. A cardiac resynchronization device was implanted; however, the patient's condition gradually worsened, which necessitated dobutamine infusion for heart failure. We readministered 30 mg PSL, which decreased the CPK level and improved the cardiomegaly. The dobutamine infusion was discontinued, and the patient was discharged. He was given 7.5 mg PSL as an outpatient, and he returned to normal life without exacerbation of the heart failure. There are similar reports showing that corticosteroids are effective for skeletal muscle improvement in Duchenne muscular dystrophy; however, their effectiveness for heart failure has been rarely reported. We experienced a case of Becker muscular dystrophy in which corticosteroid therapy was effective for refractory heart failure.

Detection of Babesia canis rossi, B. canis vogeli, and Hepatozoon canis in dogs in a village of eastern Sudan by using a screening PCR and sequencing methodologies.

PubMed

Oyamada, Maremichi; Davoust, Bernard; Boni, Mickaël; Dereure, Jacques; Bucheton, Bruno; Hammad, Awad; Itamoto, Kazuhito; Okuda, Masaru; Inokuma, Hisashi

2005-11-01

Babesia and Hepatozoon infections of dogs in a village of eastern Sudan were analyzed by using a single PCR and sequencing. Among 78 dogs, 5 were infected with Babesia canis rossi and 2 others were infected with B. canis vogeli. Thirty-three dogs were positive for Hepatozoon. Hepatozoon canis was detected by sequence analysis.

The Research of Dr. Joanne Simpson: Fifty Years Investigating Hurricanes, Tropical Clouds and Cloud Systems

NASA Technical Reports Server (NTRS)

Tao, W. -K.; Halverson, J.; Adler, R.; Garstang, M.; Houze, R., Jr.; LeMone, M.; Pielke, R., Sr.; Woodley, W.; O'C.Starr, David (Technical Monitor)

2001-01-01

This AMS Meteorological Monographs is dedicated to Dr. Joanne Simpson for her many pioneering research efforts in tropical meteorology during her fifty-year career. Dr. Simpson's major areas of scientific research involved the "hot tower" hypothesis and its role in hurricanes, structure and maintenance of trade winds, air-sea interaction, and observations and the mechanism for hurricanes and waterspouts. She was also a pioneer in cloud modeling with the first one-dimensional model and had the first cumulus model on a computer. She also played a major role in planning and leading observational experiments on convective cloud systems. The launch of the Tropical Rainfall Measuring Mission (TRMM) satellite, a joint U.S.-Japan project, in November of 1997 made it possible for quantitative measurements of tropical rainfall to be obtained on a continuous basis over the entire global tropics. Dr. Simpson was the TRAM Project Scientist from 1986 until its launch in 1997. Her efforts during this crucial period ensured that the mission was both well planned scientifically and well engineered as well as within budget. In this paper, Dr. J. Simpson's nine specific accomplishments during her fifty-year career: (1) hot tower hypothesis, (2) hurricanes, (3) airflow and clouds over heated islands, (4) cloud models, (5) trade winds and their role in cumulus development, (6) air-sea interaction, (7) cloud-cloud interactions and mergers, (8) waterspouts, and (9) TRMM science, will be described and discussed.

A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

PubMed

Yamada, Yuka; Kawakami, Michiyuki; Wada, Ayako; Otsuka, Tomoyoshi; Muraoka, Kaori; Liu, Meigen

2018-06-01

Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p < 0.001). In the BMD group, total VDS score significantly correlated with Swinyard stage (r = 0.78, p < 0.001), but not with age or lung function. Patients with BMD have swallowing problems similar to those observed in patients with DMD when matched according to physical functional status. These patients should be evaluated and followed-up for the duration of their disease. Implications for rehabiliation Dysphagia is one of the most critical problems in patients with progressive neuromuscular disease but dysphagia in patients with Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

Detection of Babesia canis rossi, B. canis vogeli, and Hepatozoon canis in Dogs in a Village of Eastern Sudan by Using a Screening PCR and Sequencing Methodologies

PubMed Central

Oyamada, Maremichi; Davoust, Bernard; Boni, Mickaël; Dereure, Jacques; Bucheton, Bruno; Hammad, Awad; Itamoto, Kazuhito; Okuda, Masaru; Inokuma, Hisashi

2005-01-01

Babesia and Hepatozoon infections of dogs in a village of eastern Sudan were analyzed by using a single PCR and sequencing. Among 78 dogs, 5 were infected with Babesia canis rossi and 2 others were infected with B. canis vogeli. Thirty-three dogs were positive for Hepatozoon. Hepatozoon canis was detected by sequence analysis. PMID:16275954

Knowledge of Carrier Status and Barriers to Testing among Mothers of Sons with Duchenne or Becker Muscular Dystrophy

PubMed Central

Bogue, Lauren; Peay, Holly; Martin, Ann; Lucas, Ann; Ramchandren, Sindhu

2016-01-01

Our study objective was to survey female carriers for Duchenne and Becker muscular dystrophy to identify barriers to carrier testing and the impact of carrier risk knowledge on cardiac and reproductive health management. We surveyed women who have or had biological sons with Duchenne or Becker muscular dystrophy and were enrolled in the US DuchenneConnect patient registry, with questions assessing knowledge of carrier status and recurrence risk, knowledge of care standards for carriers, and barriers to testing. Of the 182 eligible respondents, 25% did not know their carrier status and 14% incorrectly classified themselves as not at risk. Cost of testing was the most commonly identified barrier to testing. Women reporting unknown carrier status were 13 times as likely to express uncertainty regarding their recurrence risk compared to women reporting positive carrier status. 37% of women at an increased risk for cardiomyopathy had never had an echocardiogram. Women who were certain of their positive carrier status were twice as likely to have had an echocardiogram in the last five years compared to women with unknown carrier status. Future research on reducing barriers to counseling and carrier testing, such as cost, may improve care standard adherence. PMID:27863875

A Functional Central Limit Theorem for the Becker-Döring Model

NASA Astrophysics Data System (ADS)

Sun, Wen

2018-04-01

We investigate the fluctuations of the stochastic Becker-Döring model of polymerization when the initial size of the system converges to infinity. A functional central limit problem is proved for the vector of the number of polymers of a given size. It is shown that the stochastic process associated to fluctuations is converging to the strong solution of an infinite dimensional stochastic differential equation (SDE) in a Hilbert space. We also prove that, at equilibrium, the solution of this SDE is a Gaussian process. The proofs are based on a specific representation of the evolution equations, the introduction of a convenient Hilbert space and several technical estimates to control the fluctuations, especially of the first coordinate which interacts with all components of the infinite dimensional vector representing the state of the process.

A world revision of the bee fly tribe Usiini (Diptera, Bombyliidae) Part 2: Usia sensu stricto.

PubMed

Gibbs, David

2014-05-22

This is the second part of a world revision of the genera Usia Latreille and Parageron Paramonov, of the tribe Usiini Becker, and covers the pale-haired species, the Usia sensu stricto group. Usia sensu stricto as defined here contains 24 species of which 16 species fall into two monophyletic groups, the U. lata group with 10 species and the U. florea group with six species. Eight species cannot be placed in either of these two groups, four of them form two pairs of sibling species while the remaining four species have no clear affinities. Of the 25 formerly available names that belong in Usia sensu stricto, U. putilla Becker stat. rev., previously synonymised under U. angustifrons, is reinstated as a full species. U. sicula Egger syn. nov., is synonymised under U. manca Loew, U. anus Becker syn. nov., is synonymised under U. vestita Macquart and U. claripennis Macquart syn. nov., is synonymised under U. atrata (Fabricius). Usia vicina Macquart, formerly placed as a synonym of U. atrata, is shown to be a junior synonym of U. aenea Rossi. Five new species are described, U. anatoliensis sp. nov., U. annetteae sp. nov., U. greatheadi sp. nov., U. maghrebensis sp. nov. and U. cornigera sp. nov. Both the male and female genitalia are illustrated in detail for 21 species, female only in the cases of U. calva Loew and U. notata Loew and male only for U. incognita Paramonov.

Is mammary reconstruction with the anatomical Becker expander a simple procedure? Complications and hidden problems leading to secondary surgical procedures: a follow-up study.

PubMed

Farace, Francesco; Faenza, Mario; Bulla, Antonio; Rubino, Corrado; Campus, Gian Vittorio

2013-06-01

Debate over the role of Becker expander implants (BEIs) in breast reconstruction is still ongoing. There are no clear indications for BEI use. The main indications for BEI use are one-stage breast reconstruction procedure and congenital breast deformities correction, due to the postoperative ability to vary BEI volume. Recent studies showed that BEIs were removed 5 years after mammary reconstruction in 68% of operated patients. This entails a further surgical procedure. BEIs should not, therefore, be regarded as one-stage prostheses. We performed a case-series study of breast reconstructions with anatomically shaped Becker-35™ implants, in order to highlight complications and to flag unseen problems, which might entail a second surgical procedure. A total of 229 patients, reconstructed from 2005 to 2010, were enrolled in this study. Data relating to implant type, volume, mean operative time and complications were recorded. All the patients underwent the same surgical procedure. The minimum follow-up period was 18 months. During a 5-year follow-up, 99 patients required secondary surgery to correct their complications or sequelae; 46 of them underwent BEI removal within 2 years of implantation, 56 within 3 years, 65 within 4 years and 74 within 5 years. Our findings show that two different sorts of complications can arise with these devices, leading to premature implant removal, one common to any breast implant and one peculiar to BEIs. The Becker implant is a permanent expander. Surgeons must, therefore, be aware that, once positioned, the Becker expander cannot be adjusted at a later date, as in two-stage expander/prosthesis reconstructions for instance. Surgeons must have a clear understanding of possible BEI complications in order to be able to discuss these with their patients. Therefore, only surgeons experienced in breast reconstruction should use BEIs. Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by

Three new species of Rectiostoma Becker, 1982 (Lepidoptera: Gelechioidea: Depressariidae) from Area de Conservación Guanacaste, northwestern Costa Rica

USDA-ARS?s Scientific Manuscript database

We describe three new species of Rectiostoma Becker, 1982 from Costa Rica: R. annemayae Heikkilä and Metz spec. nov., R. eowilsoni Heikkilä and Metz spec. nov. and R. philipmayi Heikkilä and Metz spec. nov. We used a data set of DNA COI-barcodes accumulated for Lepidoptera collected at Area de Conse...

Follistatin Gene Therapy Improves Ambulation in Becker Muscular Dystrophy

PubMed Central

Al-Zaidy, Samiah A.; Sahenk, Zarife; Rodino-Klapac, Louise R.; Kaspar, Brian; Mendell, Jerry R.

2015-01-01

Abstract Follistatin is a ubiquitous secretory propeptide that functions as a potent inhibitor of the myostatin pathway, resulting in an increase in skeletal muscle mass. Its ability to interact with the pituitary activin-inhibin axis and suppress the secretion of follicle-stimulating hormone (FSH) called for caution in its clinical applicability. This limitation was circumvented by the use of one of the alternatively spliced follistatin variants, FS344, undergoing post-translational modification to FS315. This follistatin isoform is serum-based, and has a 10-fold lower affinity to activin compared to FS288. Preclinical studies of intramuscular delivery of the follistatin gene demonstrated safety and efficacy in enhancing muscle mass. We herein review the evidence supporting the utility of follistatin as a genetic enhancer to improve cellular performance. In addition, we shed light on the results of the first clinical gene transfer trial using the FS344 isoform of follistatin in subjects with Becker muscular dystrophy as well as the future directions for clinical gene therapy trials using follistatin. PMID:27858738

Follistatin Gene Therapy Improves Ambulation in Becker Muscular Dystrophy.

PubMed

Al-Zaidy, Samiah A; Sahenk, Zarife; Rodino-Klapac, Louise R; Kaspar, Brian; Mendell, Jerry R

2015-09-02

Follistatin is a ubiquitous secretory propeptide that functions as a potent inhibitor of the myostatin pathway, resulting in an increase in skeletal muscle mass. Its ability to interact with the pituitary activin-inhibin axis and suppress the secretion of follicle-stimulating hormone (FSH) called for caution in its clinical applicability. This limitation was circumvented by the use of one of the alternatively spliced follistatin variants, FS344, undergoing post-translational modification to FS315. This follistatin isoform is serum-based, and has a 10-fold lower affinity to activin compared to FS288. Preclinical studies of intramuscular delivery of the follistatin gene demonstrated safety and efficacy in enhancing muscle mass. We herein review the evidence supporting the utility of follistatin as a genetic enhancer to improve cellular performance. In addition, we shed light on the results of the first clinical gene transfer trial using the FS344 isoform of follistatin in subjects with Becker muscular dystrophy as well as the future directions for clinical gene therapy trials using follistatin.

Health services received by individuals with duchenne/becker muscular dystrophy.

PubMed

Pandya, Shree K; Campbell, Kim A; Andrews, Jennifer G; Meaney, F John; Ciafaloni, Emma

2016-02-01

Anecdotal reports from families and care providers suggest a wide variation in services received by individuals with Duchenne/Becker muscular dystrophy (DBMD). We documented the type and frequency of health services received by individuals with DBMD using the Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) interview data released in June 2012. Interviews with eligible caregivers from 5 sites (Arizona, Colorado, Georgia, Iowa, and western New York) were conducted from April 2007 to March 2012. Two hundred ninety-six caregivers (66% of those contactable) participated in the interview. There were significant differences among sites in the specialists seen and services received. Concurrence with cardiac recommendations was higher than that with respiratory recommendations. The results of this survey support and quantify the anecdotal reports from families and care providers regarding the disparities in services received by individuals with DBMD. It remains to be determined whether these differences affect outcomes. © 2015 Wiley Periodicals, Inc.

Knowledge of carrier status and barriers to testing among mothers of sons with Duchenne or Becker muscular dystrophy.

PubMed

Bogue, Lauren; Peay, Holly; Martin, Ann; Lucas, Ann; Ramchandren, Sindhu

2016-12-01

Our study objective was to survey female carriers for Duchenne and Becker muscular dystrophy to identify barriers to carrier testing and the impact of carrier risk knowledge on cardiac and reproductive health management. We surveyed women who have or had biological sons with Duchenne or Becker muscular dystrophy and were enrolled in the US DuchenneConnect patient registry, with questions assessing knowledge of carrier status and recurrence risk, knowledge of care standards for carriers, and barriers to testing. Of the 182 eligible respondents, 25% did not know their carrier status and 14% incorrectly classified themselves as not at risk. Cost of testing was the most commonly identified barrier to testing. Women reporting unknown carrier status were 13 times as likely to express uncertainty regarding their recurrence risk compared to women reporting positive carrier status. 37% of women at an increased risk for cardiomyopathy had never had an echocardiogram. Women who were certain of their positive carrier status were twice as likely to have had an echocardiogram in the last five years compared to women with unknown carrier status. Future research on reducing barriers to counseling and carrier testing, such as cost, may improve care standard adherence. Copyright © 2016 Elsevier B.V. All rights reserved.

International scientific cooperation during the 1930s. Bruno Rossi and the development of the status of cosmic rays into a branch of physics.

PubMed

Bonolis, Luisa

2014-07-01

During the 1920s and 1930s, Italian physicists established strong relationships with scientists from other European countries and the United States. The career of Bruno Rossi, a leading personality in the study of cosmic rays and an Italian pioneer of this field of research, provides a prominent example of this kind of international cooperation. Physics underwent major changes during these turbulent years, and the traditional internationalism of physics assumed a more institutionalized character. Against this backdrop, Rossi's early work was crucial in transforming the study of cosmic rays into a branch of modern physics. His friendly relationships with eminent scientists--notably Enrico Fermi, Walther Bothe, Werner Heisenberg, Hans Bethe, and Homi Bhabha--were instrumental both for the exchange of knowledge about experimental practices and theoretical discussions, and for attracting the attention of physicists such as Arthur Compton, Louis Leprince-Ringuet, Pierre Auger and Patrick Blackett to the problem of cosmic rays. Relying on material from different archives in Europe and the United States, this case study aims to provide a glimpse of the intersection between national and international dimensions during the 1930s, at a time when the study of cosmic rays was still very much in its infancy, strongly interlaced with nuclear physics, and full of uncertain, contradictory, and puzzling results. Nevertheless, as a source of high-energy particles it became a proving ground for testing the validity of the laws of quantum electrodynamics, and made a fundamental contribution to the origins of particle physics.

Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies.

PubMed

Janghra, Narinder; Morgan, Jennifer E; Sewry, Caroline A; Wilson, Francis X; Davies, Kay E; Muntoni, Francesco; Tinsley, Jonathon

2016-01-01

Duchenne muscular dystrophy is a severe and currently incurable progressive neuromuscular condition, caused by mutations in the DMD gene that result in the inability to produce dystrophin. Lack of dystrophin leads to loss of muscle fibres and a reduction in muscle mass and function. There is evidence from dystrophin-deficient mouse models that increasing levels of utrophin at the muscle fibre sarcolemma by genetic or pharmacological means significantly reduces the muscular dystrophy pathology. In order to determine the efficacy of utrophin modulators in clinical trials, it is necessary to accurately measure utrophin levels and other biomarkers on a fibre by fibre basis within a biopsy section. Our aim was to develop robust and reproducible staining and imaging protocols to quantify sarcolemmal utrophin levels, sarcolemmal dystrophin complex members and numbers of regenerating fibres within a biopsy section. We quantified sarcolemmal utrophin in mature and regenerating fibres and the percentage of regenerating muscle fibres, in muscle biopsies from Duchenne, the milder Becker muscular dystrophy and controls. Fluorescent immunostaining followed by image analysis was performed to quantify utrophin intensity and β-dystrogylcan and ɣ -sarcoglycan intensity at the sarcolemma. Antibodies to fetal and developmental myosins were used to identify regenerating muscle fibres allowing the accurate calculation of percentage regeneration fibres in the biopsy. Our results indicate that muscle biopsies from Becker muscular dystrophy patients have fewer numbers of regenerating fibres and reduced utrophin intensity compared to muscle biopsies from Duchenne muscular dystrophy patients. Of particular interest, we show for the first time that the percentage of regenerating muscle fibres within the muscle biopsy correlate with the clinical severity of Becker and Duchenne muscular dystrophy patients from whom the biopsy was taken. The ongoing development of these tools to quantify

Pseudoexon activation increases phenotype severity in a Becker muscular dystrophy patient.

PubMed

Greer, Kane; Mizzi, Kayla; Rice, Emily; Kuster, Lukas; Barrero, Roberto A; Bellgard, Matthew I; Lynch, Bryan J; Foley, Aileen Reghan; O Rathallaigh, Eoin; Wilton, Steve D; Fletcher, Sue

2015-07-01

We report a dystrophinopathy patient with an in-frame deletion of DMD exons 45-47, and therefore a genetic diagnosis of Becker muscular dystrophy, who presented with a more severe than expected phenotype. Analysis of the patient DMD mRNA revealed an 82 bp pseudoexon, derived from intron 44, that disrupts the reading frame and is expected to yield a nonfunctional dystrophin. Since the sequence of the pseudoexon and canonical splice sites does not differ from the reference sequence, we concluded that the genomic rearrangement promoted recognition of the pseudoexon, causing a severe dystrophic phenotype. We characterized the deletion breakpoints and identified motifs that might influence selection of the pseudoexon. We concluded that the donor splice site was strengthened by juxtaposition of intron 47, and loss of intron 44 silencer elements, normally located downstream of the pseudoexon donor splice site, further enhanced pseudoexon selection and inclusion in the DMD transcript in this patient.

Cerebrovascular diseases at the C. Mondino National Institute of Neurology: from Ottorino Rossi to the present day

PubMed Central

Micieli, Giuseppe; Martignoni, Emilia; Sandrini, Giorgio; Bono, Giorgio; Nappi, Giuseppe

Summary This paper traces the development of research and healthcare models in the field of cerebrovascular disorders at the C. Mondino National Institute of Neurology in Pavia, Italy. It starts with a description of the original experiences of Ottorino Rossi and his thesis on atherosclerosis which date back to the beginning of the last century; it then illustrates the connections between his seminal essay and the future directions followed by research in this institute, through to the development of one of the first stroke units in Italy. In this context, we examine a large range of scientific approaches, many related to cerebrovascular diseases (such as headaches) and autonomic disorders, and some of their biological and physiological markers. The originality of an approach also based on tools of advanced technology, including information technology, is emphasised, as is the importance of passion and perseverance in the pursuit of extraordinary results in what is an extremely complex and difficult field. PMID:21729590

Correlates of care for young men with Duchenne and Becker muscular dystrophy.

PubMed

Andrews, Jennifer G; Davis, Melinda F; Meaney, F John

2014-01-01

In progressive conditions, such as Duchenne and Becker muscular dystrophy (DBMD), the need for care may outpace care use. We examined correlates that contribute to utilization of needed care. Structured interviews were conducted on use of care among 34 young men with DBMD who were born before 1982. Disease severity, per capita income, and presence of other relatives with DBMD predicted greater use of services. Race/ethnicity, acculturation, and level of caregiver education did not significantly predict service utilization. We identified disparities in receipt of healthcare and related services in adult men with DBMD that can affect quality of life. Despite the high disease severity identified in this population, these men utilized only half of the services available to individuals with significant progressive conditions. Providers should be aware of low service utilization and focus on awareness and assistance to ensure access to available care. Copyright © 2013 Wiley Periodicals, Inc.

Guidelines for the Perianesthesia Care of the Duchenne Muscular Dystrophy/Becker Muscular Dystrophy Patient.

PubMed

Alliod, Barbara A; Ash, Rebecca A

2016-12-01

More patients suffering with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are presenting to perianesthesia settings for emergent and nonemergent treatment and care. A group of collaborative health care providers at Rush University Medical Center in Chicago developed a multidisciplinary DMD/BMD Task Force to study this disorder and create a set of guidelines to aid those engaging in the planning, execution of care, and recovery of this unique population in the perianesthesia setting. Attention to detail, well-executed preplanning, meticulous awareness of the patient, and prearranged implementation and intervention has proven to offset potential problems and complications and is the key to a successful perianesthesia period. Copyright © 2016 American Society of PeriAnesthesia Nurses. Published by Elsevier Inc. All rights reserved.

Duchenne and Becker muscular dystrophy in adolescents: current perspectives

PubMed Central

Andrews, Jennifer G; Wahl, Richard A

2018-01-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives. PMID:29588625

Duchenne and Becker muscular dystrophy in adolescents: current perspectives.

PubMed

Andrews, Jennifer G; Wahl, Richard A

2018-01-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives.

More deletions in the 5{prime} region than in the central region of the dystrophin gene were identified among Filipino Duchenne and Becker muscular dystrophy patients

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

NONE

1995-11-06

This report describes mutations in the dystrophin gene and the frequency of these mutations in Filipino pedigrees with Duchenne and Becker muscular dystrophy (DMD/BMD). The findings suggest the presence of genetic variability among DMD/BMD patients in different populations. 13 refs., 1 tab.

Longitudinal 2-point dixon muscle magnetic resonance imaging in becker muscular dystrophy.

PubMed

Bonati, Ulrike; Schmid, Maurice; Hafner, Patricia; Haas, Tanja; Bieri, Oliver; Gloor, Monika; Fischmann, Arne; Fischer, Dirk

2015-06-01

Quantitative MRI techniques detect disease progression in myopathies more sensitively than muscle function measures or conventional MRI. To date, only conventional MRI data using visual rating scales are available for measurement of disease progression in Becker muscular dystrophy (BMD). In 3 patients with BMD (mean age 36.8 years), the mean fat fraction (MFF) of the thigh muscles was assessed by MRI at baseline and at 1-year follow-up using a 2-point Dixon approach (2PD). The motor function measurement scale (MFM) was used for clinical assessment. The mean MFF of all muscles at baseline was 61.6% (SD 7.6). It increased by 3.7% to 65.3% (SD 4.7) at follow-up. The severity of muscle involvement varied between various muscle groups. As in other myopathies, 2PD can quantify fatty muscle degeneration in BMD and can detect disease progression in a small sample size and at relatively short imaging intervals. © 2015 Wiley Periodicals, Inc.

Late-onset Becker muscular dystrophy: Refining the clinical features and electrophysiological findings.

PubMed

Beltran Papsdorf, Tania; Howard, James F; Chahin, Nizar

2015-11-01

The aim of this study was to characterize a unique distribution of muscle involvement in sporadic Becker muscle dystrophy (BMD). Retrospective chart review, clinical examination, electrophysiological studies, cardiac testing, and genetic testing were performed in 5 patients. Predominant weakness and atrophy of biceps brachii, hip adduction, and quadriceps muscles was noted along with calf and extensor forearm hypertrophy. Finger flexor muscles were severely weak in 3 of 5 patients, a feature that could lead to a misdiagnosis of inclusion body myositis. Creatinine kinase was only mildly elevated in most patients. Electromyography was abnormal in all patients. Muscle biopsy in 1 patient demonstrated normal immunostaining for dystrophin. We found a unique and uniform distribution of muscle involvement in 5 sporadic cases of BMD. Recognizing these features is important for differentiating it from other myopathies that may have similar features and avoids unnecessary invasive procedures such as muscle biopsy. © 2015 Wiley Periodicals, Inc.

Calf muscle involvement in Becker muscular dystrophy: when size does not matter.

PubMed

Monforte, Mauro; Mercuri, Eugenio; Laschena, Francesco; Ricci, Enzo; Tasca, Giorgio

2014-12-15

Calf hypertrophy is a common feature in Becker muscular dystrophy (BMD), and it is still debated to which extent fatty degeneration or true muscle hypertrophy account for it. We wanted to investigate the relative contribution of these two components using a simple image analysis approach and their possible correlation with disease severity. Twenty-nine BMD patients' MRI scans were analyzed. A semiquantitative visual score assessing fatty replacement of calf muscles (calf MRI score, CMS) was calculated and correlated with the cross sectional area (CSA) of lower leg posterior compartment muscles, digitally measured on acquired images. The correlation between CSA and CMS was not significant. CMS in contrast correlated with disease severity (p<0.001) while CSA did not (p=0.969). In BMD, a major contribution to calf hypertrophy is provided by real muscle hypertrophy rather than by fatty degeneration. CMS appears to be a potential surrogate marker of disease severity. Copyright © 2014 Elsevier B.V. All rights reserved.

Diagnosis of becker muscular dystrophy: Results of Re-analysis of DNA samples.

PubMed

Straathof, Chiara S M; Van Heusden, Dave; Ippel, Pieternella F; Post, Jan G; Voermans, Nicol C; De Visser, Marianne; Brusse, Esther; Van Den Bergen, Janneke C; Van Der Kooi, Anneke J; Verschuuren, Jan J G M; Ginjaar, Hendrika B

2016-01-01

The phenotype of Becker muscular dystrophy (BMD) is highly variable, and the disease may be underdiagnosed. We searched for new mutations in the DMD gene in a cohort of previously undiagnosed patients who had been referred in the period 1985-1995. All requests for DNA analysis of the DMD gene in probands with suspected BMD were re-evaluated. If the phenotype was compatible with BMD, and no deletions or duplications were detected, DNA samples were screened for small mutations. In 79 of 185 referrals, no mutation was found. Analysis could be performed on 31 DNA samples. Seven different mutations, including 3 novel ones, were found. Long-term clinical follow-up is described. Refining DNA analysis in previously undiagnosed cases can identify mutations in the DMD gene and provide genetic diagnosis of BMD. A delayed diagnosis can still be valuable for the proband or the relatives of BMD patients. © 2015 Wiley Periodicals, Inc.

Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy

PubMed Central

Lo, Ivan F. M.; Cherk, Sharon W. W.; Cheng, Wai Wai; Fung, Eva L. W.; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S. F.; Wu, Shun Ping; Wong, Virginia C. N.

2015-01-01

The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower. PMID:28503591

Experience Gained From Launch and Early Orbit Support of the Rossi X-Ray Timing Explorer (RXTE)

NASA Technical Reports Server (NTRS)

Fink, D. R.; Chapman, K. B.; Davis, W. S.; Hashmall, J. A.; Shulman, S. E.; Underwood, S. C.; Zsoldos, J. M.; Harman, R. R.

1996-01-01

this paper reports the results to date of early mission support provided by the personnel of the Goddard Space Flight Center Flight Dynamics Division (FDD) for the Rossi X-Ray Timing Explorer (RXTE) spacecraft. For this mission, the FDD supports onboard attitude determination and ephemeris propagation by supplying ground-based orbit and attitude solutions and calibration results. The first phase of that support was to provide launch window analyses. As the launch window was determined, acquisition attitudes were calculated and calibration slews were planned. postlaunch, these slews provided the basis for ground determined calibration. Ground determined calibration results are used to improve the accuracy of onboard solutions. The FDD is applying new calibration tools designed to facilitate use of the simultaneous, high-accuracy star observations from the two RXTE star trackers for ground attitude determination and calibration. An evaluation of the performance of these tools is presented. The FDD provides updates to the onboard star catalog based on preflight analysis and analysis of flight data. The in-flight results of the mission support in each area are summarized and compared with pre-mission expectations.

Muscle MRI and functional outcome measures in Becker muscular dystrophy.

PubMed

Barp, Andrea; Bello, Luca; Caumo, Luca; Campadello, Paola; Semplicini, Claudio; Lazzarotto, Annalisa; Sorarù, Gianni; Calore, Chiara; Rampado, Alessandro; Motta, Raffaella; Stramare, Roberto; Pegoraro, Elena

2017-11-22

Becker muscular dystrophy (BMD) is a neuromuscular disorder allelic to Duchenne muscular dystrophy (DMD), caused by in-frame mutations in the dystrophin gene, and characterized by a clinical progression that is both milder and more heterogeneous than DMD. Muscle magnetic resonance imaging (MRI) has been proposed as biomarker of disease progression in dystrophinopathies. Correlation with clinically meaningful outcome measures such as North Star Ambulatory Assessment (NSAA) and 6 minute walk test (6MWT) is paramount for biomarker qualification. In this study, 51 molecularly confirmed BMD patients (aged 7-69 years) underwent muscle MRI and were evaluated with functional measures (NSAA and 6MWT) at the time of the MRI, and subsequently after one year. We confirmed a pattern of fatty substitution involving mainly the hip extensors and most thigh muscles. Severity of muscle fatty substitution was significantly correlated with specific DMD mutations: in particular, patients with an isolated deletion of exon 48, or deletions bordering exon 51, showed milder involvement. Fat infiltration scores correlated with baseline functional measures, and predicted changes after 1 year. We conclude that in BMD, skeletal muscle MRI not only strongly correlates with motor function, but also helps in predicting functional deterioration within a 12-month time frame.

Rehabilitative technology use among individuals with Duchenne/Becker muscular dystrophy.

PubMed

Pandya, Shree; Andrews, Jennifer; Campbell, Kim; Meaney, F John

2016-01-01

To document use of rehabilitative technology among individuals with Duchenne/Becker muscular dystrophy (DBMD) among sites of the Muscular Dystrophy Surveillance, Tracking, and Research network (MD STARnet). Data from 362 caregivers who participated in the MD STARnet caregiver interview between April 2006 and March 2012 (54.7% response rate) were analyzed to assess the type, frequency and duration of use of assistive technology. Caregiver reports of technology use by individuals with DBMD across five MD STARnet sites in the US demonstrated significant regional differences in the proportion of individuals who had ever used night splints (36.9%-73.0%), standers (3.1%-22.2%) and scooters (10.7%-54.5%). Among individuals who used night splints 59.7% stopped using them at a mean age of 10.3 years after a mean duration of 2.9 years in spite of the current recommendation to continue using them through the non-ambulatory phase. Results of this comprehensive survey document the frequency of assistive device use by individuals with DBMD in the USA and also provides data on differences across the sites. Further research is needed to understand the reasons for and the impact of these differences on clinical outcomes and health related quality of life of individuals with DBMD.

Revision of New World Species of the Shore-fly Subgenus Allotrichoma Becker of the Genus Allotrichoma with Description of the Subgenus Neotrichoma (Diptera, Ephydridae, Hecamedini)

PubMed Central

Mathis, Wayne N.; Zatwarnicki, Tadeusz

2012-01-01

Abstract The New World species of the subgenera Allotrichoma Becker and Neotrichoma (new subgenus) are revised, including a phylogenetic analysis of the species groups and subgenera within the genus Allotrichoma. For phylogenetic perspective and to document the monophyly of the genus Allotrichoma and its included subgenera and species groups, we also provide a cladistic analysis of genera within the tribe Hecamedini. The ingroup included seven exemplar congeners from within Allotrichoma. Outgroup sampling included exemplars of other genera within Hecamedini and from the putative sister group, Lipochaetini, and to root the analysis, we used an exemplar of the tribe Discocerinini. Analyses with successive weighting and implied weighting recovered a monophyletic Allotrichoma and indicated clades within the genus. Eight new species are described (type locality in parenthesis): Allotrichoma bifurcatum (Utah. Utah: Lake Shore (40°06.9'N, 111°41.8'W; 1370 m)), Allotrichoma dynatum (Oregon. Benton: Finley National Wildlife Refuge (44°24.6'N, 123°19.5'W)), Allotrichoma occidentale (Oregon. Lake: Lakeview (44 km E; Drake Creek; 42°11'N, 119°59.3'W)), Allotrichoma robustum (California. Kern: Kern River (35°16.1'N, 119°18.4'W)), Allotrichoma sabroskyi (New Mexico. Sandoval: La Cueva (Junction of Highways 126 and 4; 35°52'N, 106°38.4'W; 2342 m)), Allotrichoma wallowa (Oregon Baker: Goose Creek (35 km E Baker City; 44°49.2'N, 117°27.79'W; 825 m)), Allotrichoma baliops (Florida. Monroe: Key West (Willie Ward Park; 24°32.9'N, 81°47.9'W)), and Allotrichoma insulare (Dominica. Cabrits Swamp (15°35'N, 61°29'W)). Within Allotrichoma, we recognize three subgenera of which one, Neotrichoma (type species: Allotrichoma atrilabre), is newly described. All known species from the New World are described with an emphasis on structures of the male terminalia, which are fully illustrated. Detailed locality data and distribution maps for the New World species are provided. A

Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

PubMed

Wood, Molly F; Hughes, Sarah C; Hache, Lauren P; Naylor, Edwin W; Abdel-Hamid, Hoda Z; Barmada, M Michael; Dobrowolski, Steven F; Stickler, David E; Clemens, Paula R

2014-06-01

Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA). The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history. The level of support for NBS for DMD, BMD, and SMA was 95.9% among parents of children with DMD, BMD, or SMA and 92.6% among expectant parents. There was strong support for NBS for DMD, BMD, and SMA in both groups of parents. Given advances in diagnostics and promising therapeutic approaches, discussion of inclusion in NBS should continue. Copyright © 2013 Wiley Periodicals, Inc.

Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial.

PubMed

Silva, Marly Conceição; Magalhães, Tiago Augusto; Meira, Zilda Maria Alves; Rassi, Carlos Henrique Reis Esselin; Andrade, Amanda Cristina de Souza; Gutierrez, Paulo Sampaio; Azevedo, Clerio Francisco; Gurgel-Giannetti, Juliana; Vainzof, Mariz; Zatz, Mayana; Kalil-Filho, Roberto; Rochitte, Carlos Eduardo

2017-02-01

In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival. To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR). A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non-intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016. Randomization (1:1) to receive or not receive ACE inhibitor therapy. Primary outcome was MF progression from baseline to the 2-year CMR study. Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], -0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], -4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04

FULL SPECTRAL SURVEY OF ACTIVE GALACTIC NUCLEI IN THE ROSSI X-RAY TIMING EXPLORER ARCHIVE

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

Rivers, Elizabeth; Markowitz, Alex; Rothschild, Richard, E-mail: erivers@ucsd.edu

2013-08-01

We have analyzed spectra for all active galactic nuclei (AGNs) in the Rossi X-ray Timing Explorer archive. We present long-term average values of absorption, Fe line equivalent width (EW), Compton reflection, and photon index, and calculate fluxes and luminosities in the 2-10 keV band for 100 AGN with sufficient brightness and overall observation time to yield high-quality spectral results. We compare these parameters across the different classifications of Seyferts and blazars. Our distributions of photon indices for Seyfert 1s and 2s are consistent with the idea that Seyferts share a common central engine; however, our distributions of Compton reflection humpmore » strengths do not support the classical picture of absorption by a torus and reflection off a Compton-thick disk with type depending only on inclination angle. We conclude that a more complex reflecting geometry such as a combined disk and torus or clumpy torus is likely a more accurate picture of the Compton-thick material. We find that Compton reflection is present in {approx}85% of Seyferts and by comparing Fe line EW's to Compton reflection hump strengths we have found that on average 40% of the Fe line arises in Compton thick material; however, this ratio was not consistent from object to object and did not seem to be dependent on optical classification.« less

Mothers' psychological adaptation to Duchenne/Becker muscular dystrophy

PubMed Central

Peay, Holly L; Meiser, Bettina; Kinnett, Kathleen; Furlong, Pat; Porter, Kathryn; Tibben, Aad

2016-01-01

Duchenne and Becker muscular dystrophy (DBMD) cause significant emotional and care-related burden on caregivers, but no studies have evaluated predictors of positive caregiver outcomes, including disorder-specific psychological adaptation. Using a community-engaged approach focused on supporting mothers in positive aspects of caregiving, this prospective study aims to assess (i) the association between child's baseline functional status and mothers' illness perceptions, resilience, and coping self-efficacy; and (ii) predictors of mothers' psychological adaptation to caring for a child with DBMD. Biological mothers with at least one living child with DBMD completed a baseline survey (n=205) with 1-year (n=147) and 2-year (n=144) follow-up surveys. Worse child's baseline function was associated not only with increased caregiver burden and reduced maternal resilience, but also with perception of positive disease impact on the family. At two follow-ups, increased psychological adaptation to DBMD was predicted by resilience (β=0.264, P=0.001) and perceived positive impact (β=0.310, P<0.001), controlling for mother's age (β=−0.305, P<0.001) and income (β=−0.088, P=0.245). Child's functional status and caregiver burden of DBMD did not predict DBMD-specific adaptation. Though clinicians caring for families with DBMD should anticipate increased caregiver burden as the disorder progresses, interventions focused on caregiver burden are not expected to influence mothers' psychosocial adaptation. Efforts to improve mothers' well-being should focus on fostering mothers' resilience and enhancing perceptions of positive disease impact (benefit finding). Results suggest that psychosocial interventions can highlight strengths and well-being rather than burden and deficit. PMID:26306645

Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I.

PubMed

Løkken, Nicoline; Hedermann, Gitte; Thomsen, Carsten; Vissing, John

2016-09-01

We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471. © 2016 American Neurological Association.

Extensive Functional Evaluations to Monitor Aerobic Training in Becker Muscular Dystrophy: A Case Report.

PubMed

Tramonti, Caterina; Rossi, Bruno; Chisari, Carmelo

2016-06-13

Low-intensity aerobic training seems to have positive effects on muscle strength, endurance and fatigue in Becker Muscular Dystrophy (BMD) patients. We describe the case of a 33-year old BMD man, who performed a four-week aerobic training. Extensive functional evaluations were executed to monitor the efficacy of the rehabilitative treatment. Results evidenced an increased force exertion and an improvement in muscle contraction during sustained exercise. An improvement of walk velocity, together with agility, endurance capacity and oxygen consumption during exercise was observed. Moreover, an enhanced metabolic efficiency was evidenced, as shown by reduced lactate blood levels after training. Interestingly, CK showed higher levels after the training protocol, revealing possible muscle damage. In conclusion, aerobic training may represent an effective method improving exercise performance, functional status and metabolic efficiency. Anyway, a careful functional assessment should be taken into account as a useful approach in the management of the disease's rehabilitative treatment.

[Clinical features of patients with Becker muscular dystrophy and deletions of the rod domain of dystrophin gene].

PubMed

Wang, Yanyun; Zhu, Yuling; Yang, Juan; Li, Yaqin; Sun, Jiangwen; Zhan, Yixin; Zhang, Cheng

2018-02-10

OBJECTIVE To explore the clinical features of patients carrying deletions of the rod domain of the dystrophin gene. METHODS Clinical data of 12 Chinese patients with Becker muscular dystrophy (BMD) and such deletions was reviewed. RESULTS Most patients complained of muscle weakness of lower limbs. Two patients had muscle cramps, one had increased creatine kinase (CK) level, and one had dilated cardiomyopathy. CONCLUSION Compared with DMD, the clinical features of BMD are much more variable, particularly for those carrying deletions of the rod domain of the dystrophin gene. Muscular weakness may not be the sole complaint of BMD. The diagnosis of BMD cannot be excluded by moderately elevated CK. For male patients with dilated cardiomyopathy, the possibility of BMD should be considered.

Body weight-supported training in Becker and limb girdle 2I muscular dystrophy.

PubMed

Jensen, Bente R; Berthelsen, Martin P; Husu, Edith; Christensen, Sofie B; Prahm, Kira P; Vissing, John

2016-08-01

We studied the functional effects of combined strength and aerobic anti-gravity training in severely affected patients with Becker and Limb-Girdle muscular dystrophies. Eight patients performed 10-week progressive combined strength (squats, calf raises, lunges) and aerobic (walk/run, jogging in place or high knee-lift) training 3 times/week in a lower-body positive pressure environment. Closed-kinetic-chain leg muscle strength, isometric knee strength, rate of force development (RFD), and reaction time were evaluated. Baseline data indicated an intact neural activation pattern but showed compromised muscle contractile properties. Training (compliance 91%) improved functional leg muscle strength. Squat series performance increased 30%, calf raises 45%, and lunges 23%. Anti-gravity training improved closed-kinetic-chain leg muscle strength despite no changes in isometric knee extension strength and absolute RFD. The improved closed-kinetic-chain performance may relate to neural adaptation involving motor learning and/or improved muscle strength of other muscles than the weak knee extensors. Muscle Nerve 54: 239-243, 2016. © 2016 Wiley Periodicals, Inc.

Olive cultivar and maturation process on the oviposition preference of Bactrocera oleae (Rossi) (Diptera: Tephritidae).

PubMed

Malheiro, R; Casal, S; Pinheiro, L; Baptista, P; Pereira, J A

2018-02-21

The olive fly, Bactrocera oleae (Rossi) (Diptera: Tephritidae), is a key-pest in the main olives producing areas worldwide, and displays distinct preference to different olive cultivars. The present work intended to study oviposition preference towards three Portuguese cultivars (Cobrançosa, Madural, and Verdeal Transmontana) at different maturation indexes. Multiple oviposition bioassays (multiple-choice and no-choice) were conducted to assess cultivar preference. No-choice bioassays were conducted to assess the influence of different maturation indexes (MI 2; MI 3, and MI 4) in single cultivars. The longevity of olive fly adults according to the cultivar in which its larvae developed was also evaluated through survival assays. Cultivar and maturation are crucial aspects in olive fly preference. Field and laboratory assays revealed a preference towards cv. Verdeal Transmontana olives and a lower susceptibility to cv. Cobrançosa olives. A higher preference was observed for olives at MI 2 and MI 3. The slower maturation process in cv. Verdeal Transmontana (still green while the other cultivars are reddish or at black stage) seems to have an attractive effect on olive fly females, thus increasing its infestation levels. Olive fly adults from both sexes live longer if emerged from pupae developed from cv. Verdeal Transmontana fruits and live less if emerged from cv. Cobrançosa. Therefore, olive cultivar and maturation process are crucial aspects in olive fly preference, also influencing the longevity of adults.

SPECTRAL SURVEY OF X-RAY BRIGHT ACTIVE GALACTIC NUCLEI FROM THE ROSSI X-RAY TIMING EXPLORER

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

Rivers, Elizabeth; Markowitz, Alex; Rothschild, Richard, E-mail: erivers@ucsd.edu

2011-03-15

Using long-term monitoring data from the Rossi X-ray Timing Explorer (RXTE), we have selected 23 active galactic nuclei (AGNs) with sufficient brightness and overall observation time to derive broadband X-ray spectra from 3 to {approx}>100 keV. Our sample includes mainly radio-quiet Seyferts, as well as seven radio-loud sources. Given the longevity of the RXTE mission, the greater part of our data is spread out over more than a decade, providing truly long-term average spectra and eliminating inconsistencies arising from variability. We present long-term average values of absorption, Fe line parameters, Compton reflection strengths, and photon indices, as well as fluxesmore » and luminosities for the hard and very hard energy bands, 2-10 keV and 20-100 keV, respectively. We find tentative evidence for high-energy rollovers in three of our objects. We improve upon previous surveys of the very hard X-ray energy band in terms of accuracy and sensitivity, particularly with respect to confirming and quantifying the Compton reflection component. This survey is meant to provide a baseline for future analysis with respect to the long-term averages for these sources and to cement the legacy of RXTE, and especially its High Energy X-ray Timing Experiment, as a contributor to AGN spectral science.« less

Genetic diagnosis of Duchenne and Becker muscular dystrophy using next-generation sequencing technology: comprehensive mutational search in a single platform.

PubMed

Lim, Byung Chan; Lee, Seungbok; Shin, Jong-Yeon; Kim, Jong-Il; Hwang, Hee; Kim, Ki Joong; Hwang, Yong Seung; Seo, Jeong-Sun; Chae, Jong Hee

2011-11-01

Duchenne muscular dystrophy or Becker muscular dystrophy might be a suitable candidate disease for application of next-generation sequencing in the genetic diagnosis because the complex mutational spectrum and the large size of the dystrophin gene require two or more analytical methods and have a high cost. The authors tested whether large deletions/duplications or small mutations, such as point mutations or short insertions/deletions of the dystrophin gene, could be predicted accurately in a single platform using next-generation sequencing technology. A custom solution-based target enrichment kit was designed to capture whole genomic regions of the dystrophin gene and other muscular-dystrophy-related genes. A multiplexing strategy, wherein four differently bar-coded samples were captured and sequenced together in a single lane of the Illumina Genome Analyser, was applied. The study subjects were 25 16 with deficient dystrophin expression without a large deletion/duplication and 9 with a known large deletion/duplication. Nearly 100% of the exonic region of the dystrophin gene was covered by at least eight reads with a mean read depth of 107. Pathogenic small mutations were identified in 15 of the 16 patients without a large deletion/duplication. Using these 16 patients as the standard, the authors' method accurately predicted the deleted or duplicated exons in the 9 patients with known mutations. Inclusion of non-coding regions and paired-end sequence analysis enabled accurate identification by increasing the read depth and providing information about the breakpoint junction. The current method has an advantage for the genetic diagnosis of Duchenne muscular dystrophy and Becker muscular dystrophy wherein a comprehensive mutational search may be feasible using a single platform.

In the aftermath of State v. Becker: a review of state and federal jury instructions on insanity acquittal disposition.

PubMed

Piel, Jennifer

2012-01-01

An important topic related to the insanity defense is what jurors should be told about the disposition of a defendant found not guilty by reason of insanity (NGRI). In the federal court system, jurors are not instructed about the consequences of an NGRI verdict. State courts, however, are divided on the question. The federal precedent, Shannon v. United States, and the most recent state case to rule on NGRI juror instructions, State v. Becker, are reviewed in detail. What follows is the author's critique of the principal arguments for and against a jury instruction on NGRI disposition. The author argues in favor of a jury instruction on the consequences of an NGRI verdict.

Relatively low proportion of dystrophin gene deletions in Israeili Duchenne and Becker muscular dystrophy patients

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

Shomrat, R.; Gluck, E.; Legum, C.

1994-02-15

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are allelic disorders caused by mutations in the X-linked dystrophin gene. The most common mutations in western populations are deletions that are spread non-randomly throughout the gene. Molecular analysis of the dystrophin gene structure by hybridization of the full length cDNA to Southern blots and by PCR in 62 unrelated Israeli male DMD/BMD patients showed deletions in 23 (37%). This proportion is significantly lower than that found in European and North American populations (55-65%). Seventy-eight percent of the deletions were confined to exons 44-52, half of these exons 44-45, and themore » remaining 22% to exons 1 and 19. There was no correlation between the size of the deletion and the severity of the disease. All the deletions causing frameshift resulted in the DMD phenotypes. 43 refs., 1 fig., 1 tab.« less

Cardiac considerations in the operative management of the patient with Duchenne or Becker muscular dystrophy.

PubMed

Cripe, Linda H; Tobias, Joseph D

2013-09-01

Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) is a progressive multisystem neuromuscular disorder. In addition to the skeletal muscle, the myocardium in the DMD/BMD patient is dystrophin deficient which results in a progressive cardiomyopathy. The myopathic myocardium poses significant risk of increased morbidity and mortality at the time of major surgical procedures. Careful attention must be given to the DMD/BMD patient during the intraoperative and postoperative period. Anesthesia selection is critical and anesthetics should be avoided which have been shown to be harmful in this patient population. Preanesthesia assessment should include cardiac consultation and detailed preoperative evaluation. Intraoperative management needs to insure that the weakened myocardium is not compromised by physiologic changes such as hypotension or major fluid shifts. Finally, attention to the cardiac status of the patient must continue into the postoperative period. The surgical care of the DMD/BMD patient requires a multispecialty approach to insure operative success. © 2013 John Wiley & Sons Ltd.

Diagnostic and clinical characteristics of early-manifesting females with Duchenne or Becker muscular dystrophy.

PubMed

Imbornoni, Lauren; Price, Elinora T; Andrews, Jennifer; Meaney, F John; Ciafaloni, Emma; Cunniff, Christopher

2014-11-01

Manifestations of Duchenne and Becker muscular dystrophy (DBMD) are present in up to 40% of heterozygous females, but there are few reports of females who exhibit skeletal muscle symptoms in childhood. From the Muscular Dystrophy Surveillance Tracking and Research Network, a multi-site population-based surveillance network for dystrophinopathy, nine symptomatic female heterozygotes with onset of symptoms prior to age 9 years were identified. The median age at diagnosis was 8.3 years, and the median interval from first symptoms to diagnosis was 1.35 years. Of the nine female heterozygotes, four had a positive family history, seven had intellectual disability and five had at least one mental health disorder. Mental health concerns included attention deficit hyperactivity disorder (ADHD), autism spectrum features, bipolar disorder, and depression. The frequency of intellectual and mental health problems in this group is higher than previously reported for affected males and for symptomatic females. These findings may have implications for diagnosis of early manifesting heterozygotes and for their health supervision. © 2014 Wiley Periodicals, Inc.

Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures.

PubMed

Segura, Leal G; Lorenz, Jessica D; Weingarten, Toby N; Scavonetto, Federica; Bojanić, Katarina; Selcen, Duygu; Sprung, Juraj

2013-09-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. To review the perioperative outcomes of patients with DMD and BMD. We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children. © 2013 John Wiley & Sons Ltd.

Age at onset of first signs or symptoms predicts age at loss of ambulation in Duchenne and Becker Muscular Dystrophy: Data from the MD STARnet.

PubMed

Ciafaloni, Emma; Kumar, Anil; Liu, Ke; Pandya, Shree; Westfield, Christina; Fox, Deborah J; Caspers Conway, Kristin M; Cunniff, Christopher; Mathews, Katherine; West, Nancy; Romitti, Paul A; McDermott, Michael P

2016-01-01

We investigated the prognostic utility of onset age at first signs and symptoms (SS) to predict onset age at loss of ambulation (LOA) for childhood-onset Duchenne and Becker Muscular Dystrophies (DBMD). Our cohort comprised male cases with DBMD ascertained by the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet). Adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using Cox proportional hazards models for associations between onset ages of first SS and LOA. Covariates controlled for were corticosteroid use, family history of DBMD, birth year, race/ethnicity, and MD STARnet site. Onset age at first SS was considered as a continuous and as a categorical variable. A one-year increase in onset age at first SS was significantly associated with a 10% reduction in annual risk of LOA (HR = 0.90, CI = 0.87-0.94). Treating onset age at first SS as a categorical variable yielded a similar association (≥ 5 years: referent; ≥ 3 to < 5 years: HR = 1.36, CI = 1.02-1.81; 18 months to < 3 years: HR = 1.72, CI = 1.31-2.26; < 18 months: HR = 1.52, CI = 1.14-2.02). Earlier onset age at first SS is associated with earlier onset age at LOA and may have clinical utility in differentiating childhood-onset Duchenne and Becker muscular dystrophies.

Application of the International Classification of Functioning, Disability and Health system to symptoms of the Duchenne and Becker muscular dystrophies.

PubMed

Conway, Kristin M; Ciafaloni, Emma; Matthews, Dennis; Westfield, Chris; James, Kathy; Paramsothy, Pangaja; Romitti, Paul A

2018-07-01

Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are X-linked recessive diseases that affect dystrophin production resulting in compromised muscle function across multiple systems. The International Classification of Functioning, Disability and Health provides a systematic classification scheme from which body functions affected by a dystrophinopathy can be identified and used to examine functional health. The infrastructure of the Muscular Dystrophy Surveillance, Tracking, and Research Network was used to identify commonly affected body functions and link selected functions to clinical surveillance data collected through medical record abstraction. Seventy-one (24 second-, 41 third- and 7 fourth-level) body function categories were selected via clinician review and consensus. Of these, 15 of 24 retained second-level categories were linked to data elements from the Muscular Dystrophy Surveillance, Tracking, and Research Network surveillance database. Our findings support continued development of a core set of body functions from the International Classification of Functioning, Disability and Health system that are representative of disease progression in dystrophinopathies and the incorporation of these functions in standardized evaluations of functional health and implementation of individualized rehabilitation care plans. Implications for Rehabilitation Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are X-linked recessive disorders that affect the production of dystrophin resulting in compromised muscle function across multiple systems. The severity and progressive nature of dystrophinopathies can have considerable impact on a patient's participation in activities across multiple life domains. Our findings support continued development of an International Classification of Functioning, Disability and Health core set for childhood-onset dystrophinopathies. A standardized

Comment on "Carbon farming in hot, dry coastal areas: an option for climate change mitigation" by Becker et al. (2013)

NASA Astrophysics Data System (ADS)

Heimann, M.

2014-01-01

Becker et al. (2013) argue that an afforestation of 0.73 × 109 ha with Jatropha curcas plants would generate an additional terrestrial carbon sink of 4.3 PgC yr-1, enough to stabilise the atmospheric mixing ratio of carbon dioxide (CO2) at current levels. However, this is not consistent with the dynamics of the global carbon cycle. Using a well-established global carbon cycle model, the effect of adding such a hypothetical sink leads to a reduction of atmospheric CO2 levels in the year 2030 by 25 ppm compared to a reference scenario. However, the stabilisation of the atmospheric CO2 concentration requires a much larger additional sink or corresponding reduction of anthropogenic emissions.

Comment on "Carbon farming in hot, dry coastal areas: an option for climate change mitigation" by Becker et al. (2013)

NASA Astrophysics Data System (ADS)

Heimann, M.

2013-08-01

Becker et al. (2013) argue that an afforestation of 0.73 109 ha with Jatropha curcas plants would generate an additional terrestrial carbon sink of 4.3 PgC yr-1, enough to stabilise the atmospheric mixing ratio of carbon dioxide (CO2) at current levels. However, this is not consistent with the dynamics of the global carbon cycle. Using a well established global carbon cycle model, the effect of adding such a hypothetical sink leads to a reduction of atmospheric CO2 levels in the year 2030 by 25 ppm compared to a reference scenario. However, the stabilisation of the atmospheric CO2 concentration requires a much larger additional sink or corresponding reduction of anthropogenic emissions.

Identification of de novo mutations of Duchénnè/Becker muscular dystrophies in southern Spain.

PubMed

Garcia, Susana; de Haro, Tomás; Zafra-Ceres, Mercedes; Poyatos, Antonio; Gomez-Capilla, Jose A; Gomez-Llorente, Carolina

2014-01-01

Duchénnè/Becker muscular dystrophies (DMD/BMD) are X-linked diseases, which are caused by a de novo gene mutation in one-third of affected males. The study objectives were to determine the incidence of DMD/BMD in Andalusia (Spain) and to establish the percentage of affected males in whom a de novo gene mutation was responsible. Multiplex ligation-dependent probe amplification (MLPA) technology was applied to determine the incidence of DMD/BMD in 84 males with suspicion of the disease and 106 female relatives. Dystrophin gene exon deletion (89.5%) or duplication (10.5%) was detected in 38 of the 84 males by MLPA technology; de novo mutations account for 4 (16.7%) of the 24 mother-son pairs studied. MLPA technology is adequate for the molecular diagnosis of DMD/BMD and establishes whether the mother carries the molecular alteration responsible for the disease, a highly relevant issue for genetic counseling.

Estimating Attitude, Trajectory, and Gyro Biases in an Extended Kalman Filter using Earth Magnetic Field Data from the Rossi X-Ray Timing Explorer

NASA Technical Reports Server (NTRS)

Deutschmann, Julie; Bar-Itzhack, Itzhack

1997-01-01

Traditionally satellite attitude and trajectory have been estimated with completely separate systems, using different measurement data. The estimation of both trajectory and attitude for low earth orbit satellites has been successfully demonstrated in ground software using magnetometer and gyroscope data. Since the earth's magnetic field is a function of time and position, and since time is known quite precisely, the differences between the computed and measured magnetic field components, as measured by the magnetometers throughout the entire spacecraft orbit, are a function of both the spacecraft trajectory and attitude errors. Therefore, these errors can be used to estimate both trajectory and attitude. This work further tests the single augmented Extended Kalman Filter (EKF) which simultaneously and autonomously estimates spacecraft trajectory and attitude with data from the Rossi X-Ray Timing Explorer (RXTE) magnetometer and gyro-measured body rates. In addition, gyro biases are added to the state and the filter's ability to estimate them is presented.

Functional changes in Becker muscular dystrophy: implications for clinical trials in dystrophinopathies.

PubMed

Bello, Luca; Campadello, Paola; Barp, Andrea; Fanin, Marina; Semplicini, Claudio; Sorarù, Gianni; Caumo, Luca; Calore, Chiara; Angelini, Corrado; Pegoraro, Elena

2016-09-01

We performed a 1-year longitudinal study of Six Minute Walk Test (6MWT), North Star Ambulatory Assessment (NSAA), and timed function tests in Becker muscular dystrophy (BMD). Skeletal muscle dystrophin was quantified by immunoblot. We grouped deletions ending on exon 45 ("del 45-x", n = 28) or 51 ("del x-51", n = 10); isolated exon 48 deletion ("del 48", n = 10); and other mutations (n = 21). Only patients in the "del 45-x" or "other" groups became non-ambulatory (n = 5, log-rank p = n.s.) or unable to run (n = 22, p < 0.001). All measures correlated positively with dystrophin quantity and negatively with age, and were significantly more impaired in the "del 45-x" and "other" groups. After one year, NSAA score decreased significantly (-0.9 ± 1.6, p < 0.001); in the "del 45-x" group, both NSAA (-1.3 ± 1.7, p = 0.001) and 6MWT (-12 ± 31 m, p = 0.059) decreased. We conclude that patients with "del x-51" or "del 48" mutations have mild or asymptomatic BMD, while "del 45-x" mutations cause comparatively severe weakness, and functional deterioration in 1 year. Furthermore, exon 51 skipping could be more effective than exon 45 skipping in Duchenne muscular dystrophy.

Serum creatinine level: a supplemental index to distinguish Duchenne muscular dystrophy from Becker muscular dystrophy.

PubMed

Zhang, Huili; Zhu, Yuling; Sun, Yiming; Liang, Yingyin; Li, Yaqin; Zhang, Yu; Deng, Langhui; Wen, Xingxuan; Zhang, Cheng

2015-01-01

To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn) level reflects disease severity. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r = -0.793) and partial correlation analysis after adjustment for age, height, and weight (r = -0.791; both P < 0.01). Serum Crn level was significantly higher in patients with in-frame than out-of-frame mutations (Z = -4.716,  P < 0.01) and in Becker muscular dystrophy (BMD) patients than Duchenne muscular dystrophy (DMD) patients at ages 4, 5, 7, and 9 yr (all P < 0.0125). After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β = 7.140,  t = 6.277,  P < 0.01). Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

Prevalence and Genetic Profile of Duchene and Becker Muscular Dystrophy in Puerto Rico.

PubMed

Ramos, Edwardo; Conde, José G; Berrios, Rafael Arias; Pardo, Sherly; Gómez, Omar; Mas Rodríguez, Manuel F

2016-05-27

Duchenne and Becker Muscular Dystrophy (DMD and BMD, respectively), are common forms of inherited muscle disease. Information regarding the epidemiology of these conditions, including genotype, is still sparse. To establish the prevalence and genetic profile of DMD and BMD in Puerto Rico. We collected data from medical records in all Muscular Dystrophy Association (MDA) clinics in Puerto Rico in order to estimate the prevalence of DMD and BMD and to describe the genotypic profile of these patients. Patients selected for data analysis matched "definite", "probable" and "possible" case definitions as established by MD STARnet. A total of 141 patients matched the inclusion criteria, with 64.5% and 35.5% being categorized into DMD and BMD, respectively. DMD and BMD prevalence in Puerto Rico was estimated at 5.18 and 2.84 per 100,000 males, respectively. Deletion was the most common form of mutation (66.7%) in the dystrophin gene, with exons in segment 45 to 47 being the most frequently affected. This is the first report of the prevalence and genetic profile characteristics of DMD and BMD in Puerto Rico. Prevalence of DMD was similar to that reported worldwide, while prevalence of BMD was higher. Genetic profile was consistent with that reported in the literature.

Becker muscular dystrophy due to an intronic splicing mutation inducing a dual dystrophin transcript.

PubMed

Todeschini, Alice; Gualandi, Francesca; Trabanelli, Cecilia; Armaroli, Annarita; Ravani, Anna; Fanin, Marina; Rota, Silvia; Bello, Luca; Ferlini, Alessandra; Pegoraro, Elena; Padovani, Alessandro; Filosto, Massimiliano

2016-10-01

We describe a 29-year-old patient who complained of left thigh muscle weakness since he was 23 and of moderate proximal weakness of both lower limbs with difficulty in climbing stairs and running since he was 27. Mild weakness of iliopsoas and quadriceps muscles and muscle atrophy of both the distal forearm and thigh were observed upon clinical examination. He harboured a novel c.1150-3C>G substitution in the DMD gene, affecting the intron 10 acceptor splice site and causing exon 11 skipping and an out-of-frame transcript. However, protein of normal molecular weight but in reduced amounts was observed on Western Blot analysis. Reverse transcription analysis on muscle RNA showed production, via alternative splicing, of a transcript missing exon 11 as well as a low abundant full-length transcript which is enough to avoid the severe Duchenne phenotype. Our study showed that a reduced amount of full length dystrophin leads to a mild form of Becker muscular dystrophy. These results confirm earlier findings that low amounts of dystrophin can be associated with a milder phenotype, which is promising for therapies aiming at dystrophin restoration. Copyright © 2016 Elsevier B.V. All rights reserved.

A case report: Becker muscular dystrophy presenting with epilepsy and dysgnosia induced by duplication mutation of Dystrophin gene.

PubMed

Miao, Jing; Feng, Jia-Chun; Zhu, Dan; Yu, Xue-Fan

2016-12-12

Becker muscular dystrophy (BMD), a genetic disorder of X-linked recessive inheritance, typically presents with gradually progressive muscle weakness. The condition is caused by mutations of Dystrophin gene located at Xp21.2. Epilepsy is an infrequent manifestation of BMD, while cases of BMD with dysgnosia are extremely rare. We describe a 9-year-old boy with BMD, who presented with epilepsy and dysgnosia. Serum creatine kinase level was markedly elevated (3665 U/L). Wechsler intelligence tests showed a low intelligence quotient (IQ = 65). Electromyogram showed slight myogenic changes and skeletal muscle biopsy revealed muscular dystrophy. Immunohistochemical staining showed partial positivity of sarcolemma for dystrophin-N. Multiplex ligation-dependent probe amplification revealed a duplication mutation in exons 37-44 in the Dystrophin gene. The present case report helps to better understand the clinical and genetic features of BMD.

A Novel Mutation in DMD (c.10797+5G>A) Causes Becker Muscular Dystrophy Associated with Intellectual Disability.

PubMed

Banihani, Rudaina; Baskin, Berivan; Halliday, William; Kobayashi, Jeff; Kawamura, Anne; McAdam, Laura; Ray, Peter N; Yoon, Grace

2016-04-01

Severe intellectual disability has been reported in a subgroup of patients with Duchenne muscular dystrophy but is not typically associated with Becker muscular dystrophy. The authors report a 13-year-old boy, with severe intellectual disability (Wechsler Intelligence Scales for Children-IV, Full Scale IQ < 0.1 percentile), attention-deficit hyperactivity disorder, and mild muscle weakness. He had elevated serum creatine kinase and dystrophic changes on muscle biopsy. Dystrophin immunohistochemistry revealed decreased staining with the C-terminal and mid-rod antibodies and essentially absent staining of the N-terminal immunostain. Sequencing of muscle mRNA revealed aberrant splicing due to a c.10797+5G > A mutation in DMD. Dystrophinopathy may be associated with predominantly cognitive impairment and neurobehavioral disorder, and should be considered in the differential diagnosis of unexplained cognitive or psychiatric disturbance in males.

Towards understanding temporal and spatial dynamics of Bactrocera oleae (Rossi) infestations using decade-long agrometeorological time series

NASA Astrophysics Data System (ADS)

Marchi, Susanna; Guidotti, Diego; Ricciolini, Massimo; Petacchi, Ruggero

2016-11-01

Insect dynamics depend on temperature patterns, and therefore, global warming may lead to increasing frequencies and intensities of insect outbreaks. The aim of this work was to analyze the dynamics of the olive fruit fly, Bactrocera oleae (Rossi), in Tuscany (Italy). We profited from long-term records of insect infestation and weather data available from the regional database and agrometeorological network. We tested whether the analysis of 13 years of monitoring campaigns can be used as basis for prediction models of B. oleae infestation. We related the percentage of infestation observed in the first part of the host-pest interaction and throughout the whole year to agrometeorological indices formulated for different time periods. A two-step approach was adopted to inspect the effect of weather on infestation: generalized linear model with a binomial error distribution and principal component regression to reduce the number of the agrometeorological factors and remove their collinearity. We found a consistent relationship between the degree of infestation and the temperature-based indices calculated for the previous period. The relationship was stronger with the minimum temperature of winter season. Higher infestation was observed in years following warmer winters. The temperature of the previous winter and spring explained 66 % of variance of early-season infestation. The temperature of previous winter and spring, and current summer, explained 72 % of variance of total annual infestation. These results highlight the importance of multiannual monitoring activity to fully understand the dynamics of B. oleae populations at a regional scale.

Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy.

PubMed

Martin, Elizabeth A; Barresi, Rita; Byrne, Barry J; Tsimerinov, Evgeny I; Scott, Bryan L; Walker, Ashley E; Gurudevan, Swaminatha V; Anene, Francine; Elashoff, Robert M; Thomas, Gail D; Victor, Ronald G

2012-11-28

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. Like Duchenne muscular dystrophy (DMD), BMD is caused by mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the muscle sarcolemma. Among these is neuronal nitric oxide synthase (nNOSμ), which requires certain spectrin-like repeats in dystrophin's rod domain and the adaptor protein α-syntrophin to be targeted to the sarcolemma. When healthy skeletal muscle is subjected to exercise, sarcolemmal nNOSμ-derived NO attenuates local α-adrenergic vasoconstriction, thereby optimizing perfusion of muscle. We found previously that this protective mechanism is defective-causing functional muscle ischemia-in dystrophin-deficient muscles of the mdx mouse (a model of DMD) and of children with DMD, in whom nNOSμ is mislocalized to the cytosol instead of the sarcolemma. We report that this protective mechanism also is defective in men with BMD in whom the most common dystrophin mutations disrupt sarcolemmal targeting of nNOSμ. In these men, the vasoconstrictor response, measured as a decrease in muscle oxygenation, to reflex sympathetic activation is not appropriately attenuated during exercise of the dystrophic muscles. In a randomized placebo-controlled crossover trial, we show that functional muscle ischemia is alleviated and normal blood flow regulation is fully restored in the muscles of men with BMD by boosting NO-cGMP (guanosine 3',5'-monophosphate) signaling with a single dose of the drug tadalafil, a phosphodiesterase 5A inhibitor. These results further support an essential role for sarcolemmal nNOSμ in the normal modulation of sympathetic vasoconstriction in exercising human skeletal muscle and implicate the NO-cGMP pathway as a putative new target for treating BMD.

Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy

PubMed Central

Martin, Elizabeth A.; Barresi, Rita; Byrne, Barry J.; Tsimerinov, Evgeny I.; Scott, Bryan L.; Walker, Ashley E.; Gurudevan, Swaminatha V.; Anene, Francine; Elashoff, Robert M.; Thomas, Gail D.; Victor, Ronald G.

2013-01-01

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. Like Duchenne muscular dystrophy (DMD), BMD is caused by mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the muscle sarcolemma. Among these is neuronal nitric oxide synthase (nNOSμ), which requires certain spectrin-like repeats in dystrophin’s rod domain and the adaptor protein α-syntrophin to be targeted to the sarcolemma. When healthy skeletal muscle is subjected to exercise, sarcolemmal nNOSμ-derived nitric oxide (NO) attenuates local α-adrenergic vasoconstriction thereby optimizing perfusion of muscle. We found previously that this protective mechanism is defective—causing functional muscle ischemia—in dystrophin-deficient muscles of the mdx mouse (a model of DMD) and of children with DMD, in whom nNOSμ is mislocalized to the cytosol instead of the sarcolemma. Here, we report that this protective mechanism also is defective in men with BMD in whom the most common dystrophin mutations disrupt sarcolemmal targeting of nNOSμ. In these men, the vasoconstrictor response, measured as a decrease in muscle oxygenation, to reflex sympathetic activation is not appropriately attenuated during exercise of the dystrophic muscles. In a randomized placebo-controlled cross-over trial, we show that functional muscle ischemia is alleviated and normal blood flow regulation fully restored in the muscles of men with BMD by boosting NO-cGMP signaling with a single dose of the drug tadalafil, a phosphodiesterase (PDE5A) inhibitor. These results further support an essential role for sarcolemmal nNOSμ in the normal modulation of sympathetic vasoconstriction in exercising human skeletal muscle and implicate the NO-cGMP pathway as a putative new target for treating BMD. PMID:23197572

Evaluation of point mutations in dystrophin gene in Iranian Duchenne and Becker muscular dystrophy patients: introducing three novel variants.

PubMed

Haghshenas, Maryam; Akbari, Mohammad Taghi; Karizi, Shohreh Zare; Deilamani, Faravareh Khordadpoor; Nafissi, Shahriar; Salehi, Zivar

2016-06-01

Duchenne and Becker muscular dystrophies (DMD and BMD) are X-linked neuromuscular diseases characterized by progressive muscular weakness and degeneration of skeletal muscles. Approximately two-thirds of the patients have large deletions or duplications in the dystrophin gene and the remaining one-third have point mutations. This study was performed to evaluate point mutations in Iranian DMD/BMD male patients. A total of 29 DNA samples from patients who did not show any large deletion/duplication mutations following multiplex polymerase chain reaction (PCR) and multiplex ligation-dependent probe amplification (MLPA) screening were sequenced for detection of point mutations in exons 50-79. Also exon 44 was sequenced in one sample in which a false positive deletion was detected by MLPA method. Cycle sequencing revealed four nonsense, one frameshift and two splice site mutations as well as two missense variants.

THE LIGHT CURVE OF HERCULES X-1 AS OBSERVED BY THE ROSSI X-RAY TIMING EXPLORER

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

Leahy, D. A.; Igna, Ciprian, E-mail: leahy@ucalgary.ca

2011-07-20

Analysis of the light curve of Hercules X-1 using the full set of archival observations of Hercules X-1 by the Rossi X-Ray Timing Explorer/Proportional Counter Array (RXTE/PCA) is reported. The observations cover time periods that Her X-1 is in main high, short high, and low states, and an anomalous low state (ALS). They include over 1.4 Ms of net exposure time. We present 35 day and orbital phase folded light curves of the count rates and softness ratios, showing the range of behaviors of Her X-1 with the high sensitivity of the RXTE/PCA. New phenomena are uncovered and previous phenomenamore » are seen in greater detail. For both main high and short high states, the fraction of time in dips is found to be a function of orbital phase and of 35 day phase. It increases steadily with orbital phase past orbital phase 0.3 and is higher at the start and end of both main high and short high states. It is higher for short high state (62%) than for main high state (28%). The normal low state data and ALS data are compared: the low state count rate is {approx}twice as high as for ALS data. The 2-4 keV to 9-20 keV softness ratio changes smoothly with orbital phase for low states and ALSs, and is indistinguishable between the two, yet very different than for the high states. This supports models for which the cause of the ALS is changed disk geometry that prevents a direct line of sight from neutron star to observer at all 35 day phases.« less

A phase 1/2a follistatin gene therapy trial for becker muscular dystrophy.

PubMed

Mendell, Jerry R; Sahenk, Zarife; Malik, Vinod; Gomez, Ana M; Flanigan, Kevin M; Lowes, Linda P; Alfano, Lindsay N; Berry, Katherine; Meadows, Eric; Lewis, Sarah; Braun, Lyndsey; Shontz, Kim; Rouhana, Maria; Clark, Kelly Reed; Rosales, Xiomara Q; Al-Zaidy, Samiah; Govoni, Alessandra; Rodino-Klapac, Louise R; Hogan, Mark J; Kaspar, Brian K

2015-01-01

Becker muscular dystrophy (BMD) is a variant of dystrophin deficiency resulting from DMD gene mutations. Phenotype is variable with loss of ambulation in late teenage or late mid-life years. There is currently no treatment for this condition. In this BMD proof-of-principle clinical trial, a potent myostatin antagonist, follistatin (FS), was used to inhibit the myostatin pathway. Extensive preclinical studies, using adeno-associated virus (AAV) to deliver follistatin, demonstrated an increase in strength. For this trial, we used the alternatively spliced FS344 to avoid potential binding to off target sites. AAV1.CMV.FS344 was delivered to six BMD patients by direct bilateral intramuscular quadriceps injections. Cohort 1 included three subjects receiving 3 × 10(11) vg/kg/leg. The distance walked on the 6MWT was the primary outcome measure. Patients 01 and 02 improved 58 meters (m) and 125 m, respectively. Patient 03 showed no change. In Cohort 2, Patients 05 and 06 received 6 × 10(11) vg/kg/leg with improved 6MWT by 108 m and 29 m, whereas, Patient 04 showed no improvement. No adverse effects were encountered. Histological changes corroborated benefit showing reduced endomysial fibrosis, reduced central nucleation, more normal fiber size distribution with muscle hypertrophy, especially at high dose. The results are encouraging for treatment of dystrophin-deficient muscle diseases.

A Phase 1/2a Follistatin Gene Therapy Trial for Becker Muscular Dystrophy

PubMed Central

Mendell, Jerry R; Sahenk, Zarife; Malik, Vinod; Gomez, Ana M; Flanigan, Kevin M; Lowes, Linda P; Alfano, Lindsay N; Berry, Katherine; Meadows, Eric; Lewis, Sarah; Braun, Lyndsey; Shontz, Kim; Rouhana, Maria; Clark, Kelly Reed; Rosales, Xiomara Q; Al-Zaidy, Samiah; Govoni, Alessandra; Rodino-Klapac, Louise R; Hogan, Mark J; Kaspar, Brian K

2015-01-01

Becker muscular dystrophy (BMD) is a variant of dystrophin deficiency resulting from DMD gene mutations. Phenotype is variable with loss of ambulation in late teenage or late mid-life years. There is currently no treatment for this condition. In this BMD proof-of-principle clinical trial, a potent myostatin antagonist, follistatin (FS), was used to inhibit the myostatin pathway. Extensive preclinical studies, using adeno-associated virus (AAV) to deliver follistatin, demonstrated an increase in strength. For this trial, we used the alternatively spliced FS344 to avoid potential binding to off target sites. AAV1.CMV.FS344 was delivered to six BMD patients by direct bilateral intramuscular quadriceps injections. Cohort 1 included three subjects receiving 3 × 1011 vg/kg/leg. The distance walked on the 6MWT was the primary outcome measure. Patients 01 and 02 improved 58 meters (m) and 125 m, respectively. Patient 03 showed no change. In Cohort 2, Patients 05 and 06 received 6 × 1011 vg/kg/leg with improved 6MWT by 108 m and 29 m, whereas, Patient 04 showed no improvement. No adverse effects were encountered. Histological changes corroborated benefit showing reduced endomysial fibrosis, reduced central nucleation, more normal fiber size distribution with muscle hypertrophy, especially at high dose. The results are encouraging for treatment of dystrophin-deficient muscle diseases. PMID:25322757

Towards understanding temporal and spatial dynamics of Bactrocera oleae (Rossi) infestations using decade-long agrometeorological time series.

PubMed

Marchi, Susanna; Guidotti, Diego; Ricciolini, Massimo; Petacchi, Ruggero

2016-11-01

Insect dynamics depend on temperature patterns, and therefore, global warming may lead to increasing frequencies and intensities of insect outbreaks. The aim of this work was to analyze the dynamics of the olive fruit fly, Bactrocera oleae (Rossi), in Tuscany (Italy). We profited from long-term records of insect infestation and weather data available from the regional database and agrometeorological network. We tested whether the analysis of 13 years of monitoring campaigns can be used as basis for prediction models of B. oleae infestation. We related the percentage of infestation observed in the first part of the host-pest interaction and throughout the whole year to agrometeorological indices formulated for different time periods. A two-step approach was adopted to inspect the effect of weather on infestation: generalized linear model with a binomial error distribution and principal component regression to reduce the number of the agrometeorological factors and remove their collinearity. We found a consistent relationship between the degree of infestation and the temperature-based indices calculated for the previous period. The relationship was stronger with the minimum temperature of winter season. Higher infestation was observed in years following warmer winters. The temperature of the previous winter and spring explained 66 % of variance of early-season infestation. The temperature of previous winter and spring, and current summer, explained 72 % of variance of total annual infestation. These results highlight the importance of multiannual monitoring activity to fully understand the dynamics of B. oleae populations at a regional scale.

TWELVE AND A HALF YEARS OF OBSERVATIONS OF CENTAURUS A WITH THE ROSSI X-RAY TIMING EXPLORER

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

Rothschild, R. E.; Markowitz, A.; Rivers, E.

2011-05-20

The Rossi X-ray Timing Explorer (RXTE) has observed the nearest radio galaxy, Centaurus A (Cen A), in 13 intervals from 1996 August to 2009 February over the 3-200 keV band. Spectra accumulated over the 13 intervals were well described with an absorbed power law and an iron line. Cutoff power laws and Compton reflection from cold matter did not provide a better description. For the 2009 January observation, we set a lower limit on the cutoff energy at over 2 MeV. The power spectral density function was generated from RXTE/All Sky Monitor and Proportional Counter Array data as well asmore » an XMM-Newton long look, and clear evidence for a break at 18{sup +18}{sub -7} days (68% conf.) was seen. Given Cen A's high black hole mass and very low value of L{sub X}/L{sub Edd}, the break was a factor of 17{sup +36}{sub -13} times higher than the break frequency predicted by the McHardy et al. relation, which was empirically derived for a sample of objects, which are radio-quiet and accreting at relatively high values of L{sub bol}/L{sub Edd}. We have interpreted our observations in the context of a clumpy molecular torus. The variability characteristics and the broadband spectral energy distribution, when compared to Seyferts, imply that the bright hard X-ray continuum emission may originate at the base of the jet, yet from behind the absorbing line-of-sight material, in contrast to what is commonly observed from blazars.« less

Abnormal short-latency synaptic plasticity in the motor cortex of subjects with Becker muscular dystrophy: a rTMS study.

PubMed

Golaszewski, Stefan; Schwenker, Kerstin; Bergmann, Jürgen; Brigo, Francesco; Christova, Monica; Trinka, Eugen; Nardone, Raffaele

2016-01-01

We used repetitive transcranial magnetic stimulation (rTMS) to further investigate motor cortex excitability in 13 patients with Becker muscular dystrophy (BMD), six of them with slight mental retardation. RTMS delivered at 5Hz frequency and suprathreshold intensity progressively increases the size of motor evoked potentials (MEPs) in healthy subjects; the rTMS-induced facilitation of MEPs was significantly reduced in the BMD patients mentally retarded or classified as borderline when compared with age-matched control subjects and the BMD patients with normal intelligence. The increase in the duration of the cortical silent period was similar in both patient groups and controls. These findings suggest an altered cortical short-term synaptic plasticity in glutamate-dependent excitatory circuits within the motor cortex in BMD patients with intellectual disabilities. RTMS studies may shed new light on the physiological mechanisms of cortical involvement in dystrophinopathies. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

A novel point mutation (G[sup [minus]1] to T) in a 5[prime] splice donor site of intron 13 of the dystrophin gene results in exon skipping and is responsible for Becker Muscular Dystrophy

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

Hagiwara, Yoko; Nishio, Hisahide; Kitoh, Yoshihiko

1994-01-01

The mutations in one-third of Duchenne and Becker muscular dystrophy patients remain unknown, as they do not involve gross rearrangements of the dystrophin gene. The authors now report a defect in the splicing of precursor mRNA (pre-mRNA), resulting from a maternally inherited mutation of the dystrophin gene in a patient with Becker muscular dystrophy. This defect results from a G-to-T transversion at the terminal nucleotide of exon 13, within the 5[prime] splice site of intron 13, and causes complete skipping of exon 13 during processing of dystrophin pre-mRNA. The predicted polypeptide encoded by the aberrant mRNA is a truncated dystrophinmore » lacking 40 amino acids from the amino-proximal end of the rod domain. This is the first report of an intraexon point mutation that completely inactivates a 5[prime] splice donor site in dystrophin pre-mRNA. Analysis of the genomic context of the G[sup [minus]1]-to-T mutation at the 5[prime] splice site supports the exon-definition model of pre-mRNA splicing and contributes to the understanding of splice-site selection. 48 refs., 5 figs.« less

Psychosocial Needs and Facilitators of Mothers Caring for Children with Duchenne/Becker Muscular Dystrophy.

PubMed

Peay, Holly L; Meiser, Bettina; Kinnett, Kathleen; Tibben, Aad

2018-02-01

Care guidelines for Duchenne/Becker muscular dystrophy (DBMD) include recommendations for assessment of caregivers of patients with DBMD followed by proactive psychosocial interventions. To inform clinical assessment, this study described appraisals of psychosocial needs and caregiving facilitators of mothers of individuals with DBMD. Two hundred and five mothers completed an online survey. More than 50% endorsed unmet needs for managing uncertainty about the future and managing DBMD fears. Higher levels of unmet need were associated with less disease progression/earlier stage of DBMD (rho = -0.166 p = 0.02). Twenty-one percent regularly used respite care and 57% worried about allowing others to care for their child. Highly-endorsed care facilitators included partner relationships (63%), child's approach to life (59%), and family relationships (49%). Our findings highlight the importance of psychological and social support for caregivers. Starting when children are young, clinicians should assess caregivers' unmet psychological needs, particularly uncertainty and fear. Exploring needs and facilitators may allow clinics to target and customize interventions that build upon existing strengths and supports. Our findings have implications for efforts to promote early diagnosis and newborn screening, in that increased needs in mothers of younger children should be anticipated and built into counseling. Further research can assess whether and how unmet needs change as new therapies become available.

[Specific features of Becker Muscular Dystrophy patients and female carriers of Duchenne Muscular Dystrophy].

PubMed

Magot, A; Mercier, S; Péréon, Y

2015-12-01

Becker muscular dystrophy (BMD) was first described in 1955 and linked to the DMD gene in 1987. Compared to Duchenne muscular dystrophy (DMD), clinical onset of BMD usually occurs after the age of 12 and wheelchair is required after the age of 16. BMD is characterized by generalized weakness first affecting limb girdle muscles, hypertrophy of the calves and cardiomyopathy in males. Some patients have only mild symptoms such as cramps or elevated serum creatine kinases (SCK) throughout all their lives. SCK levels are usually elevated. Muscle biopsy (immunohistochemistry or immunoblotting) shows a dystrophic pattern with abnormal dystrophin staining. Diagnosis is confirmed by DMD gene sequencing. Deletions or duplications of one or several exons are identified in the majority of cases. A multidisciplinary approach is recommended for the care management of these patients with a particular attention to the cardiomyopathy, which is typically responsible for death but can be prevented by specific treatment. X-linked dilated cardiomyopathies linked to DMD gene are a phenotypic continuum of BMD. Some female carriers of DMD mutations exhibit clinical symptoms of variable severity, often milder and beginning later than in males. The cardiomyopathy is the most frequent feature that should be especially monitored in these patients. Genetic counselling should be systematically proposed. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Prenatal molecular diagnosis of inherited neuromuscular diseases: Duchenne/Becker muscular dystrophy, myotonic dystrophy type 1 and spinal muscular atrophy.

PubMed

Esposito, Gabriella; Ruggiero, Raffaella; Savarese, Maria; Savarese, Giovanni; Tremolaterra, Maria Roberta; Salvatore, Francesco; Carsana, Antonella

2013-12-01

Neuromuscular disease is a broad term that encompasses many diseases that either directly, via an intrinsic muscle disorder, or indirectly, via a nerve disorder, impairs muscle function. Here we report the experience of our group in the counselling and molecular prenatal diagnosis of three inherited neuromuscular diseases, i.e., Duchenne/Becker muscular dystrophy (DMD/BMD), myotonic dystrophy type 1 (DM1), spinal muscular atrophy (SMA). We performed a total of 83 DMD/BMD, 15 DM1 and 54 SMA prenatal diagnoses using a combination of technologies for either direct or linkage diagnosis. We identified 16, 5 and 10 affected foetuses, respectively. The improvement of analytical procedures in recent years has increased the mutation detection rate and reduced the analytical time. Due to the complexity of the experimental procedures and the high, specific professional expertise required for both laboratory activities and the related counselling, these types of analyses should be preferentially performed in reference molecular diagnostic centres.

Expectations and experiences of investigators and parents involved in a clinical trial for Duchenne/Becker muscular dystrophy.

PubMed

Peay, Holly L; Tibben, Aad; Fisher, Tyler; Brenna, Ethan; Biesecker, Barbara B

2014-02-01

The social context of rare disease research is changing, with increased community engagement around drug development and clinical trials. This engagement may benefit patients and families but may also lead to heightened trial expectations and therapeutic misconception. Clinical investigators are also susceptible to harboring high expectations. Little is known about parental motivations and expectations for clinical trials for rare pediatric disorders. We describe the experience of parents and clinical investigators involved in a phase II clinical trial for Duchenne and Becker muscular dystrophy: their expectations, hopes, motivations, and reactions to the termination of the trial. This qualitative study was based on interviews with clinical investigators and parents of sons with Duchenne and Becker muscular dystrophy (DBMD) who participated in the phase IIa or IIb ataluren clinical trial in the United States. Interviews were transcribed and coded for thematic analysis. Participants were 12 parents of affected boys receiving active drug and 9 clinical investigators. High trial expectations of direct benefit were reported by parents and many clinicians. Investigators described monitoring and managing parents' expectations; several worried about their own involvement in increasing parents' expectations. Most parents were able to differentiate their expectations from their optimistic hopes for a cure. Parents' expectations arose from other parents, advocacy organizations, and the sponsor. All parents reported some degree of clinical benefit to their children. Secondary benefits were hopefulness and powerful feelings associated with active efforts to affect the disease course. Parents and clinical investigators reported strong, close relationships that were mutually important. Parents and clinicians felt valued by the sponsor for the majority of the trial. When the trial abruptly stopped, they described loss of engagement, distress, and feeling unprepared for the

Serendipitous Detections of XTE J1906+09 with the Rossi X-Ray Timing Explorer

NASA Technical Reports Server (NTRS)

Wilson, Colleen A.; Finger, Mark H.; Gogus, Ersin; Woods, Peter M.; Kouveliotou, Chryssa

2002-01-01

The 89 s X-ray pulsar XTE J1906+09 was discovered during Rossi X-Ray Timing Explorer (RXTE) observations of SGR 1900+14 in 1996. Because of monitoring campaigns of SGR 1900+14, XTE J1906+09 was also monitored regularly in 1996 September, 1998 May-June, 1998 August-1999 July, and 2000 March-2001 January. A search for pulsations resulted in detections of only the two previously reported outbursts in 1996 September and 1998 August-September. Pulsed flux upper limits for the rest of the observations show that XTE J1906+09 is a transient X-ray pulsar and likely has a Be star companion. The RXTE all-sky monitor did not reveal XTE J1906+09. Pulse-timing analysis of the second outburst discovered a sinusoidal signature in the pulse frequencies that is likely produced by an orbital periastron passage. Fits to pulse phases using an orbital model and quadratic phase model have chi(exp 2) minima at orbital periods of 26-30 days for fixed mass functions of 5, 10, 15, and 20 solar masses. The pulse shape showed energy- and intensity-dependent variations. Pulse-phase spectroscopy quantified the energy-dependent variations. The phase-averaged spectrum used the pulse minimum spectrum as the background spectrum to eliminate effects from SGR 1900+14 and the Galactic ridge and was well fitted by an absorbed power law with a high-energy cutoff with column density N(sub H) = 6 +/- 1 x 10(exp 22)/sq cm, a photon index of 1.01 +/- 0.08, cutoff energy E(sub cut) = 11 +/- 1 keV, and e-folding energy E(sub fold) = 19 +/- 4 keV. Estimated 2-10 keV peak fluxes, corrected for contributions from the Galactic ridge and SGR 1900+14, are 6 x l0(exp -12) and 1.1 x 10(exp -10) ergs/sq cm/s for the 1996 and 1998 outbursts, respectively. XTE J1906+09 may be part of an unusual class of Be/X-ray binaries that do not lie on the general spin period versus orbital period correlation with the majority of Be/X-ray binaries.

Effects of Sildenafil on Cerebrovascular Reactivity in Patients with Becker Muscular Dystrophy.

PubMed

Lindberg, Ulrich; Witting, Nanna; Jørgensen, Stine Lundgaard; Vissing, John; Rostrup, Egill; Larsson, Henrik Bo Wiberg; Kruuse, Christina

2017-01-01

Patients suffering from Becker muscular dystrophy (BMD) have dysfunctional dystrophin proteins and are deficient in neuronal nitric oxide synthase (nNOS) in muscles. This causes functional ischemia and contributes to muscle wasting. Similar functional ischemia may be present in brains of patients with BMD, who often have mild cognitive impairment, and nNOS may be important for the regulation of the microvascular circulation in the brain. We hypothesized that treatment with sildenafil, a phosphodiesterase type 5 inhibitor that potentiates nitric oxide responses, would augment both the blood oxygen level-dependent (BOLD) response and cerebral blood flow (CBF) in patients with BMD. Seventeen patients (mean ± SD age 38.5 ± 10.8 years) with BMD were included in this randomized, double-blind, placebo-controlled, crossover trial. Twelve patients completed the entire study. Effects of sildenafil were assessed by 3 T magnetic resonance (MR) scanning, evoked potentials, somatosensory task-induced BOLD functional MR imaging, regional and global perfusion, and angiography before and after 4 weeks of sildenafil, 20 mg (Revatio in gelatine capsules, oral, 3 times daily), or placebo treatment. Sildenafil increased the event-related sensory and visual BOLD response compared with placebo (p < 0.01). However, sildenafil did not alter CBF, measured by MR phase contrast mapping, or the arterial diameter of the middle cerebral artery, measured by MR angiography. We conclude that nNOS may play a role in event-related neurovascular responses. Further studies in patients with BMD may help clarify the roles of dystrophin and nNOS in neurovascular coupling in general, and in patients with BMD in particular.

AN EXTENDED AND MORE SENSITIVE SEARCH FOR PERIODICITIES IN ROSSI X-RAY TIMING EXPLORER/ALL-SKY MONITOR X-RAY LIGHT CURVES

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

Levine, Alan M.; Bradt, Hale V.; Chakrabarty, Deepto

2011-09-01

We present the results of a systematic search in {approx}14 years of Rossi X-ray Timing Explorer All-Sky Monitor (ASM) data for evidence of periodicities. Two variations of the commonly used Fourier analysis search method have been employed to significantly improve upon the sensitivity achieved by Wen et al. in 2006, who also searched for periodicities in ASM data. In addition, the present search is comprehensive in terms of sources studied and frequency range covered, and has yielded the detection of the signatures of the orbital periods of eight low-mass X-ray binary systems and of ten high-mass X-ray binaries not listedmore » in the tables of Wen et al. Orbital periods, epochs, signal amplitudes, modulation fractions, and folded light curves are given for each of these systems. Seven of the orbital periods are the most precise reported to date. In the course of this work, the 18.545 day orbital period of IGR J18483-0311 was co-discovered, and the first detections in X-rays were made of the {approx}3.9 day orbital period of LMC X-1 and the {approx}3.79 hr orbital period of 4U 1636-536. The results inform future searches for orbital and other periodicities in X-ray binaries.« less

The fruit flies (Diptera: Tephritidae) described by Theodor Becker from Iran and Western China revisited in the collections of the Zoological Institute, Saint-Petersburg and Museum für Naturkunde, Berlin.

PubMed

Korneyev, Severyn V; Korneyev, Valery A

2017-01-31

The type specimens of fruit flies described by Dr. Theodor Becker based on material collected in China (Xinjiang and Xizang) and Iran by Russian expeditions directed by Petr Kozlov and Mykola Zarudny are listed and figured. They are deposited in the collection of the Zoological Institute of Russian Academy of Sciences, Saint Petersburg with some duplicates in the Museum für Naturkunde, Berlin. Current concepts of the species, their morphological characters (illustrated by photographs of type specimens), current condition, and nomenclature are discussed.

Willingness to pay for small solar powered bed net fans: results of a Becker-DeGroot-Marschak auction in Ghana.

PubMed

Yukich, Joshua O; Briët, Olivier J T; Ahorlu, Collins K; Nardini, Peter; Keating, Joseph

2017-08-07

Long-lasting insecticidal nets (LLINs) are one of the main interventions recommended by the World Health Organization for malaria vector control. LLINs are ineffective if they are not being used. Subsequent to the completion of a cluster randomized cross over trial conducted in rural Greater Accra where participants were provided with the 'Bɔkɔɔ System'-a set of solar powered net fan and light consoles with a solar panel and battery-or alternative household water filters, all trial participants were invited to participate in a Becker-DeGroot-Marschak auction to determine the mean willingness to pay (WTP) for the fan and light consoles and to estimate the demand curve for the units. Results demonstraed a mean WTP of approximately 55 Cedis (~13 USD). Demand results suggested that at a price which would support full manufacturing cost recovery, a majority of households in the area would be willing to purchase at least one such unit.

Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy: A Territory Wide Collaborative Study in Hong Kong.

PubMed

Chan, Sophelia H S; Lo, Ivan F M; Cherk, Sharon W W; Cheng, Wai Wai; Fung, Eva L W; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S F; Wu, Shun Ping; Wong, Virginia C N

2015-01-01

The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower.

A decade of Rossi X-ray Timing Explorer Seyfert observations: An RXTE Seyfert spectral database

NASA Astrophysics Data System (ADS)

Mattson, Barbara Jo

2008-10-01

With over forty years of X-ray observations, we should have a grasp on the X- ray nature of active galactic nuclei (AGN). The unification model of Antonucci and Miller (1985) offered a context for understanding observations by defining a "typical" AGN geometry, with observed spectral differences explained by line- of-sight effects. However, the emerging picture is that the central AGN is more complex than unification alone can describe. We explore the unified model with a systematic X-ray spectral study of bright Seyfert galaxies observed by the Rossi X-Ray Timing Explorer (RXTE) over its first 10 years. We develop a spectral-fit database of 821 time-resolved spectra from 39 Seyfert galaxies fitted to a model describing the effects of an X-ray power-law spectrum reprocessed and absorbed by material in the central AGN region. We observe a relationship between radio and X-ray properties for Seyfert 1s, with the spectral parameters differing between radio-loud and radio-quiet Seyfert 1s. We also find a complex relationship between the Fe K equivalent width ( EW ) and the power-law photon index (Gamma) for the Seyfert 1s, with a correlation for the radio-loud sources and an anti-correlation for the radio- quiet sources. These results can be explained if X-rays from the relativistic jet in radio-loud sources contribute significantly to the observed spectrum. We observe scatter in the EW-Gamma relationship for the Seyfert 2s, suggesting complex environments that unification alone cannot explain. We see a strong correlation between Gamma and the reflection fraction ( R ) in the Seyfert 1 and 2 samples, but modeling degeneracies are present, so this relationship cannot be trusted as instructive of the AGN physics. For the Seyfert 1 sample, we find an anticorrelation between EW and the 2 to 10 keV luminosity ( L x ), also known as the X-ray Baldwin effect. This may suggest that higher luminosity sources contain less material or may be due to a time-lag effect. We do not

Pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy.

PubMed

Lager, Christina; Kroksmark, Anna-Karin

2015-09-01

The purpose of this study was to explore the prevalence, nature and scope of pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy and whether the pain differs between diagnostic groups or between adolescents with different ambulation status. Furthermore to study the consequences of pain and to identify pain-exacerbating and pain-relieving factors. In a national survey, fifty-five adolescents with spinal muscular atrophy and dystrophinopathy completed a questionnaire assessing pain frequency, duration, location using a body map, intensity and discomfort using visual analogue scales, pain interference using a modified version of Brief Pain Inventory and factors exacerbating and relieving pain. Sixty-nine per cent of the adolescents reported pain during the past three months and 50% reported chronic pain. The pain prevalence did not differ significantly between diagnostic groups or between ambulators and non-ambulators. The average pain intensity was graded as mild and the worst pain as moderate. The pain typically occurred weekly, most frequently in the neck/back or legs. General activity and mood were the areas that were most affected by pain. Common pain-exacerbating factors were sitting, too much movement/activity and being lifted or transferred. Pain is a frequent problem in adolescents with spinal muscular atrophy and dystrophinopathy. The assessments used enable an understanding both of the nature and scope of pain and of the impact of pain in everyday life. The study highlights the importance of assessing pain in a systematic manner and offering an individual approach to interventions designed to reduce pain in this population. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Consecutive analysis of mutation spectrum in the dystrophin gene of 507 Korean boys with Duchenne/Becker muscular dystrophy in a single center.

PubMed

Cho, Anna; Seong, Moon-Woo; Lim, Byung Chan; Lee, Hwa Jeen; Byeon, Jung Hye; Kim, Seung Soo; Kim, Soo Yeon; Choi, Sun Ah; Wong, Ai-Lynn; Lee, Jeongho; Kim, Jon Soo; Ryu, Hye Won; Lee, Jin Sook; Kim, Hunmin; Hwang, Hee; Choi, Ji Eun; Kim, Ki Joong; Hwang, Young Seung; Hong, Ki Ho; Park, Seungman; Cho, Sung Im; Lee, Seung Jun; Park, Hyunwoong; Seo, Soo Hyun; Park, Sung Sup; Chae, Jong Hee

2017-05-01

Duchenne and Becker muscular dystrophies (DMD and BMD) are allelic X-linked recessive muscle diseases caused by mutations in the large and complex dystrophin gene. We analyzed the dystrophin gene in 507 Korean DMD/BMD patients by multiple ligation-dependent probe amplification and direct sequencing. Overall, 117 different deletions, 48 duplications, and 90 pathogenic sequence variations, including 30 novel variations, were identified. Deletions and duplications accounted for 65.4% and 13.3% of Korean dystrophinopathy, respectively, suggesting that the incidence of large rearrangements in dystrophin is similar among different ethnic groups. We also detected sequence variations in >100 probands. The small variations were dispersed across the whole gene, and 12.3% were nonsense mutations. Precise genetic characterization in patients with DMD/BMD is timely and important for implementing nationwide registration systems and future molecular therapeutic trials in Korea and globally. Muscle Nerve 55: 727-734, 2017. © 2016 Wiley Periodicals, Inc.

Identification of Neoceratitis asiatica (Becker) (Diptera: Tephritidae) based on morphological characteristics and DNA barcode.

PubMed

Guo, Shaokun; He, Jia; Zhao, Zihua; Liu, Lijun; Gao, Liyuan; Wei, Shuhua; Guo, Xiaoyu; Zhang, Rong; Li, Zhihong

2017-12-12

Neoceratitis asiatica (Becker), which especially infests wolfberry (Lycium barbarum L.), could cause serious economic losses every year in China, especially to organic wolfberry production. In some important wolfberry plantings, it is difficult and time-consuming to rear the larvae or pupae to adults for morphological identification. Molecular identification based on DNA barcode is a solution to the problem. In this study, 15 samples were collected from Ningxia, China. Among them, five adults were identified according to their morphological characteristics. The utility of mitochondrial DNA (mtDNA) cytochrome c oxidase I (COI) gene sequence as DNA barcode in distinguishing N. asiatica was evaluated by analysing Kimura 2-parameter distances and phylogenetic trees. There were significant differences between intra-specific and inter-specific genetic distances according to the barcoding gap analysis. The uncertain larval and pupal samples were within the same cluster as N. asiatica adults and formed sister cluster to N. cyanescens. A combination of morphological and molecular methods enabled accurate identification of N. asiatica. This is the first study using DNA barcode to identify N. asiatica and the obtained DNA sequences will be added to the DNA barcode database.

Evidence of Insulin Resistance and Other Metabolic Alterations in Boys with Duchenne or Becker Muscular Dystrophy.

PubMed

Rodríguez-Cruz, Maricela; Sanchez, Raúl; Escobar, Rosa E; Cruz-Guzmán, Oriana Del Rocío; López-Alarcón, Mardia; Bernabe García, Mariela; Coral-Vázquez, Ramón; Matute, Guadalupe; Velázquez Wong, Ana Claudia

2015-01-01

Aim. Our aim was (1) to determine the frequency of insulin resistance (IR) in patients with Duchenne/Becker muscular dystrophy (DMD/BMD), (2) to identify deleted exons of DMD gene associated with obesity and IR, and (3) to explore some likely molecular mechanisms leading to IR. Materials and Methods. In 66 patients with DMD/BMD without corticosteroids treatment, IR, obesity, and body fat mass were evaluated. Molecules involved in glucose metabolism were analyzed in muscle biopsies. Results show that 18.3%, 22.7%, and 68% were underweight, overweight, or obese, and with high adiposity, respectively; 48.5% and 36.4% presented hyperinsulinemia and IR, respectively. Underweight patients (27.3%) exhibited hyperinsulinemia and IR. Carriers of deletions in exons 45 (OR = 9.32; 95% CI = 1.16-74.69) and 50 (OR = 8.73; 95% CI = 1.17-65.10) from DMD gene presented higher risk for IR than noncarriers. We observed a greater staining of cytoplasmic aggregates for GLUT4 in muscle biopsies than healthy muscle tissue. Conclusion. Obesity, hyperinsulinemia, and IR were observed in DMD/BMD patients and are independent of corticosteroids treatment. Carriers of deletion in exons 45 or 50 from DMD gene are at risk for developing IR. It is suggested that alteration in GLUT4 in muscle fibers from DMD patients could be involved in IR.

Evidence of Insulin Resistance and Other Metabolic Alterations in Boys with Duchenne or Becker Muscular Dystrophy

PubMed Central

Rodríguez-Cruz, Maricela; Sanchez, Raúl; Escobar, Rosa E.; Cruz-Guzmán, Oriana del Rocío; López-Alarcón, Mardia; Bernabe García, Mariela; Coral-Vázquez, Ramón; Matute, Guadalupe; Velázquez Wong, Ana Claudia

2015-01-01

Aim. Our aim was (1) to determine the frequency of insulin resistance (IR) in patients with Duchenne/Becker muscular dystrophy (DMD/BMD), (2) to identify deleted exons of DMD gene associated with obesity and IR, and (3) to explore some likely molecular mechanisms leading to IR. Materials and Methods. In 66 patients with DMD/BMD without corticosteroids treatment, IR, obesity, and body fat mass were evaluated. Molecules involved in glucose metabolism were analyzed in muscle biopsies. Results show that 18.3%, 22.7%, and 68% were underweight, overweight, or obese, and with high adiposity, respectively; 48.5% and 36.4% presented hyperinsulinemia and IR, respectively. Underweight patients (27.3%) exhibited hyperinsulinemia and IR. Carriers of deletions in exons 45 (OR = 9.32; 95% CI = 1.16–74.69) and 50 (OR = 8.73; 95% CI = 1.17–65.10) from DMD gene presented higher risk for IR than noncarriers. We observed a greater staining of cytoplasmic aggregates for GLUT4 in muscle biopsies than healthy muscle tissue. Conclusion. Obesity, hyperinsulinemia, and IR were observed in DMD/BMD patients and are independent of corticosteroids treatment. Carriers of deletion in exons 45 or 50 from DMD gene are at risk for developing IR. It is suggested that alteration in GLUT4 in muscle fibers from DMD patients could be involved in IR. PMID:26089900

KENNEDY SPACE CENTER, FLA. - KSC External Relations and Business Development Director JoAnn Morgan speaks to attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the group’s Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing women’s accomplishments.

NASA Image and Video Library

2003-06-07

KENNEDY SPACE CENTER, FLA. - KSC External Relations and Business Development Director JoAnn Morgan speaks to attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the group’s Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing women’s accomplishments.

Duchenne/Becker muscular dystrophy: correlation of phenotype by electroretinography with sites of dystrophin mutations.

PubMed

Pillers, D A; Fitzgerald, K M; Duncan, N M; Rash, S M; White, R A; Dwinnell, S J; Powell, B R; Schnur, R E; Ray, P N; Cibis, G W; Weleber, R G

1999-01-01

The dark-adapted electroretinogram (ERG) of patients with Duchenne and Becker muscular dystrophy (DMD/BMD) shows a marked reduction in b-wave amplitude. Genotype-phenotype studies of mouse models for DMD show position-specific effects of the mutations upon the phenotype: mice with 5' defects of dystrophin have normal ERGs, those with defects in the central region have a normal b-wave amplitude associated with prolonged implicit times for both the b-wave and oscillatory potentials, and mice with 3' defects have a phenotype similar to that seen in DMD/BMD patients. The mouse studies suggest a key role for the carboxyl terminal dystrophin isoform, Dp260, in retinal electrophysiology. We have undertaken a systematic evaluation of DMD/BMD patients through clinical examination and review of the literature in order to determine whether the position-specific effects of mutations noted in the mouse are present in man. We have found that, in man, a wider variation of DMD defects correlate with reductions in the b-wave amplitude. Individuals with normal ERGs have mutations predominantly located 5' of the transcript initiation site of Dp260. Our results suggest that the most important determinant in the ERG b-wave phenotype is the mutation position, rather than muscle disease severity. Forty-six per cent of patients with mutations 5' of the Dp260 transcript start site have abnormal ERGs, as opposed to 94% with more distal mutations. The human genotype-phenotype correlations are consistent with a role for Dp260 in normal retinal electrophysiology and may also reflect the expression of other C-terminal dystrophin isoforms and their contributions to retinal signal transmission.

Clinical characterisation of Becker muscular dystrophy patients predicts favourable outcome in exon-skipping therapy.

PubMed

van den Bergen, J C; Schade van Westrum, S M; Dekker, L; van der Kooi, A J; de Visser, M; Wokke, B H A; Straathof, C S; Hulsker, M A; Aartsma-Rus, A; Verschuuren, J J; Ginjaar, H B

2014-01-01

Duchenne and Becker muscular dystrophy (DMD/BMD) are both caused by mutations in the DMD gene. Out-of-frame mutations in DMD lead to absence of the dystrophin protein, while in-frame BMD mutations cause production of internally deleted dystrophin. Clinically, patients with DMD loose ambulance around the age of 12, need ventilatory support at their late teens and die in their third or fourth decade due to pulmonary or cardiac failure. BMD has a more variable disease course. The disease course of patients with BMD with specific mutations could be very informative to predict the outcome of the exon-skipping therapy, aiming to restore the reading-frame in patients with DMD. Patients with BMD with a mutation equalling a DMD mutation after successful exon skipping were selected from the Dutch Dystrophinopathy Database. Information about disease course was gathered through a standardised questionnaire. Cardiac data were collected from medical correspondence and a previous study on cardiac function in BMD. Forty-eight patients were included, representing 11 different mutations. Median age of patients was 43 years (range 6-67). Nine patients were wheelchair users (26-56 years). Dilated cardiomyopathy was present in 7/36 patients. Only one patient used ventilatory support. Three patients had died at the age of 45, 50 and 76 years, respectively. This study provides mutation specific data on the course of disease in patients with BMD. It shows that the disease course of patients with BMD, with a mutation equalling a 'skipped' DMD mutation is relatively mild. This finding strongly supports the potential benefit of exon skipping in patients with DMD.

Characterization of genetic deletions in Becker muscular dystrophy using monoclonal antibodies against a deletion-prone region of dystrophin

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

Thanh, L.T.; Man, Nguyen Thi; Morris, G.E.

1995-08-28

We have produced a new panel of 20 monoclonal antibodies (mAbs) against a region of the dystrophin protein corresponding to a deletion-prone region of the Duchenne muscular dystrophy gene (exons 45-50). We show that immunohistochemistry or Western blotting with these {open_quotes}exon-specific{close_quotes} mAbs can provide a valuable addition to Southern blotting or PCR methods for the accurate identification of genetic deletions in Becker muscular dystrophy patients. The antibodies were mapped to the following exons: exon 45 (2 mAbs), exon 46 (6), exon 47 (1), exons 47/48 (4), exons 48-50 (6), and exon 50 (1). PCR amplification of single exons or groupsmore » of exons was used both to produce specific dystrophin immunogens and to map the mAbs obtained. PCR-mediated mutagenesis was also used to identify regions of dystrophin important for mAb binding. Because the mAbs can be used to characterize the dystrophin produced by individual muscle fibres, they will also be useful for studying {open_quotes}revertant{close_quotes} fibres in Duchenne muscle and for monitoring the results of myoblast therapy trials in MD patients with deletions in this region of the dystrophin gene. 27 refs., 7 figs., 3 tabs.« less

Patients with Duchenne and Becker muscular dystrophies are not more asymmetrical than healthy controls on timed performance of upper limb tasks

PubMed Central

Artilheiro, M.C.; Sá, C.S.C.; Fávero, F.M.; Caromano, F.A.; Voos, M.C.

2017-01-01

This study aimed to investigate possible asymmetries and relationships between performance of dominant and non-dominant upper limbs (UL) in patients with Duchenne and Becker muscular dystrophies (DMD/BMD), to compare UL performance of patients and healthy subjects and to investigate the relationship between timed performance of UL and age, motor function and muscle strength in DMD/BMD patients. Sixteen patients with DMD and 3 with BMD were evaluated with Jebsen-Taylor Test (timed performance), Vignos scale and Dimension 3 of Motor Function Measure (motor function), and Medical Research Council scale (muscle strength) on a single session. ANOVA showed no asymmetry between dominant and non-dominant UL, except in the writing subtest, in patients and in healthy controls. There were relationships between dominant and non-dominant UL performances. Correlations between timed performance, motor function and muscle strength were found, but age was not correlated with these variables. These findings may reduce the assessment time, prevent fatigue and provide more accurate clinical reasoning involving UL in DMD/BMD treatment. PMID:28746422

Identification of leaf volatiles from olive (Olea europaea) and their possible role in the ovipositional preferences of olive fly, Bactrocera oleae (Rossi) (Diptera: Tephritidae).

PubMed

Malheiro, Ricardo; Casal, Susana; Cunha, Sara C; Baptista, Paula; Pereira, José Alberto

2016-01-01

The olive fly, Bactrocera oleae (Rossi), is a monophagous pest that displays an oviposition preference among cultivars of olive (Olea europaea L.). To clarify the oviposition preference, the olive leaf volatiles of three olive cultivars (Cobrançosa, Madural and Verdeal Transmontana) were assessed by headspace solid-phase microextraction gas chromatography-mass spectrometry (HS-SPME-GC/MS) at six different periods of olive fruit maturation and degrees of infestation. A total of 39 volatiles were identified, mainly esters and alcohols, with a minor percentage of aldehydes, ketones and terpenic compounds, including sesquiterpenes. At sampling dates with higher degrees of infestation, cv. Cobrançosa had, simultaneously, significantly lower infestation degrees and higher volatile amounts than the other two cultivars, with a probable deterrent effect for oviposition. The green leaf volatiles (GLVs) (Z)-3-hexen-1-ol and (Z)-3-hexen-1-ol acetate) were the main compounds identified in all cultivars, together with toluene. The abundance of GLVs decreased significantly throughout maturation, without significant differences among cultivars, while toluene showed a general increase and positive correlation with olive fly infestation levels. The results obtained could broaden our understanding of the roles of various types and amounts of olive volatiles in the environment, especially in olive fly host selection and cultivar preference. Copyright © 2015 Elsevier Ltd. All rights reserved.

KENNEDY SPACE CENTER, FLA. - KSC External Relations and Business Development Director JoAnn Morgan (sixth from right) joins other attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the group’s Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing women’s accomplishments.

NASA Image and Video Library

2003-06-07

KENNEDY SPACE CENTER, FLA. - KSC External Relations and Business Development Director JoAnn Morgan (sixth from right) joins other attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the group’s Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing women’s accomplishments.

Exonization of an Intronic LINE-1 Element Causing Becker Muscular Dystrophy as a Novel Mutational Mechanism in Dystrophin Gene

PubMed Central

Gonçalves, Ana; Coelho, Teresa; Melo-Pires, Manuel; Sousa, Mário

2017-01-01

A broad mutational spectrum in the dystrophin (DMD) gene, from large deletions/duplications to point mutations, causes Duchenne/Becker muscular dystrophy (D/BMD). Comprehensive genotyping is particularly relevant considering the mutation-centered therapies for dystrophinopathies. We report the genetic characterization of a patient with disease onset at age 13 years, elevated creatine kinase levels and reduced dystrophin labeling, where multiplex-ligation probe amplification (MLPA) and genomic sequencing failed to detect pathogenic variants. Bioinformatic, transcriptomic (real time PCR, RT-PCR), and genomic approaches (Southern blot, long-range PCR, and single molecule real-time sequencing) were used to characterize the mutation. An aberrant transcript was identified, containing a 103-nucleotide insertion between exons 51 and 52, with no similarity with the DMD gene. This corresponded to the partial exonization of a long interspersed nuclear element (LINE-1), disrupting the open reading frame. Further characterization identified a complete LINE-1 (~6 kb with typical hallmarks) deeply inserted in intron 51. Haplotyping and segregation analysis demonstrated that the mutation had a de novo origin. Besides underscoring the importance of mRNA studies in genetically unsolved cases, this is the first report of a disease-causing fully intronic LINE-1 element in DMD, adding to the diversity of mutational events that give rise to D/BMD. PMID:28972564

Exonization of an Intronic LINE-1 Element Causing Becker Muscular Dystrophy as a Novel Mutational Mechanism in Dystrophin Gene.

PubMed

Gonçalves, Ana; Oliveira, Jorge; Coelho, Teresa; Taipa, Ricardo; Melo-Pires, Manuel; Sousa, Mário; Santos, Rosário

2017-10-03

A broad mutational spectrum in the dystrophin ( DMD ) gene, from large deletions/duplications to point mutations, causes Duchenne/Becker muscular dystrophy (D/BMD). Comprehensive genotyping is particularly relevant considering the mutation-centered therapies for dystrophinopathies. We report the genetic characterization of a patient with disease onset at age 13 years, elevated creatine kinase levels and reduced dystrophin labeling, where multiplex-ligation probe amplification (MLPA) and genomic sequencing failed to detect pathogenic variants. Bioinformatic, transcriptomic (real time PCR, RT-PCR), and genomic approaches (Southern blot, long-range PCR, and single molecule real-time sequencing) were used to characterize the mutation. An aberrant transcript was identified, containing a 103-nucleotide insertion between exons 51 and 52, with no similarity with the DMD gene. This corresponded to the partial exonization of a long interspersed nuclear element (LINE-1), disrupting the open reading frame. Further characterization identified a complete LINE-1 (~6 kb with typical hallmarks) deeply inserted in intron 51. Haplotyping and segregation analysis demonstrated that the mutation had a de novo origin. Besides underscoring the importance of mRNA studies in genetically unsolved cases, this is the first report of a disease-causing fully intronic LINE-1 element in DMD , adding to the diversity of mutational events that give rise to D/BMD.

The ultrasound-guided nerve blocks of abdominal wall contributed to anesthetic management of cholecystectomy in a patient with Becker muscular dystrophy without using muscle relaxants.

PubMed

Iwata, Masato; Kuzumoto, Naoya; Akasaki, Yuka; Morioka, Masayo; Nakayama, Kana; Matsuzawa, Nobuyoshi; Kimoto, Katsuhiro; Shimomura, Toshiyuki

2017-01-01

Becker muscular dystrophy (BMD) is a progressive neuromuscular disorder caused by mutations in the dystrophin gene. The sensitivity to non-depolarizing muscle relaxant in a patient with muscle dystrophy is reportedly higher than that in normal individuals, and the duration of the effect is known to be prolonged. In this report, we present the case of a 58-year-old man with BMD who underwent laparoscopic cholecystectomy for symptomatic cholelithiasis under total intravenous anesthesia without the use of muscle-relaxant drugs and supplemented with regional anesthesia. Anesthesia was induced and maintained with propofol, remifentanil, and fentanyl; ultrasound-guided bilateral rectus sheath block (RSB) and right-sided subcostal transversus abdominis plane block (TAP) were performed. The procedure required conversion to open surgery because of hard conglutination; intraoperative and postoperative periods were uneventful. Adequate analgesia was maintained after extubation because of the effect of RSB and TAP.

A rare subclinical or mild type of Becker muscular dystrophy caused by a single exon 48 deletion of the dystrophin gene.

PubMed

Zimowski, Janusz G; Pilch, Jacek; Pawelec, Magdalena; Purzycka, Joanna K; Kubalska, Jolanta; Ziora-Jakutowicz, Karolina; Dudzińska, Magdalena; Zaremba, Jacek

2017-08-01

In the material of 227 families with Becker muscular dystrophy (BMD), we found nine non-consanguineous families with 17 male individuals carrying a rare mutation-a single exon 48 deletion of the dystrophin gene-who were affected with a very mild or subclinical form of BMD. They were usually detected thanks to accidental findings of elevated serum creatine phosphokinase (sCPK). A thorough clinical analysis of the carriers, both children (12) and adults (5), revealed in some of them muscle hypotonia (10/17) and/or very mild muscle weakness (9/17), as well as decreased tendon reflexes (6/17). Adults, apart from very mild muscle weakness and calf hypertrophy in some, had no significant abnormalities on neurological assessments and had good exercise tolerance. Parents of the children carriers of the exon 48 deletion are usually unaware of their children being affected, and possibly at risk of developing life-threatening cardiomyopathy. The same concerns the adult male carriers. Therefore, the authors postulate undertaking preventive measures such as cascade screening of the relatives of the probands. Newborn screening programmes of Duchenne muscular dystrophy (DMD)/BMD based on sCPK marked increase may be considered.

ROSSI X-RAY TIMING EXPLORER OBSERVATIONS OF THE LOW-MASS X-RAY BINARY 4U 1608-522 IN THE UPPER-BANANA STATE

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

Takahashi, Hiromitsu; Sakurai, Soki; Makishima, Kazuo, E-mail: hirotaka@hep01.hepl.hiroshima-u.ac.jp

To investigate the physics of mass accretion onto weakly magnetized neutron stars (NSs), 95 archival Rossi X-Ray Timing Explorer data sets of an atoll source 4U 1608-522, acquired over 1996-2004 in the so-called upper-banana state, were analyzed. The object meantime exhibited 3-30 keV luminosity in the range of {approx}< 10{sup 35}-4 x 10{sup 37} erg s{sup -1}, assuming a distance of 3.6 kpc. The 3-30 keV Proportional Counter Array spectra, produced one from each data set, were represented successfully with a combination of a soft and a hard component, the presence of which was revealed in a model-independent manner bymore » studying spectral variations among the observations. The soft component is expressed by the so-called multi-color disk model with a temperature of {approx}1.8 keV, and is attributed to the emission from an optically thick standard accretion disk. The hard component is a blackbody (BB) emission with a temperature of {approx}2.7 keV, thought to be emitted from the NS surface. As the total luminosity increases, a continuous decrease is observed in the ratio of the BB luminosity to that of the disk component. This property suggests that it gradually becomes difficult for the matter flowing through the accretion disk to reach the NS surface, presumably forming outflows driven by the increased radiation pressure. On timescales of hours to days, the overall source variability was found to be controlled by two independent variables: the mass accretion rate and the innermost disk radius, which changes both physically and artificially.« less

Duchenne/Becker muscular dystrophy: A report on clinical, biochemical, and genetic study in Gujarat population, India.

PubMed

Rao, Mandava V; Sindhav, Gaurang M; Mehta, Jitendra J

2014-07-01

In India, various groups have studied different regions to find out deletion pattern of dystrophin gene. We have investigated its deletion pattern among Duchenne/Becker muscular dystrophy (D/BMD) patients across Gujarat. Moreover, in this study we also correlate the same with reading frame rule. However, we too consider various clinicopathological features to establish as adjunct indices when deletion detection fails. In this pilot study, a total of 88 D/BMD patients consulting at our centers in Gujarat, India were included. All patients were reviewed on basis of their clinical characteristics, tested by three primer sets of 10-plex, 9-plex, and 7-plex polymerase chain reaction (PCR) for genetic analysis; whereas, biochemical indices were measured using automated biochemical analyzers. The diagnosis of D/BMD was confirmed by multiplex-PCR (M-PCR) in D/BMD patients. A number of 65 (73.86%) out of 88 patients showed deletion in dystrophin gene. The exon 50 (58.46%) was the most frequent deletion found in our study. The mean age of onset of DMD and BMD was 4.09 ± 0.15 and 7.14 ± 0.55 years, respectively. In patients, mean creatine phosphokinase (CPK), lactate dehydrogenase (LDH), and myoglobin levels were elevated significantly (P < 0.05) in comparison to controls. Addition to CPK, LDH and myoglobin are good adjunct when deletion detection failed. These data are further in accordance with world literature when correlated with frame rule. The analysis has been carried out for the first time for a total of 88 D/BMD patients particularly from Gujarat, India. More research is essential to elucidate specific mutation pattern in association with management and therapies of proband.

Generation of induced pluripotent stem cells from a Becker muscular dystrophy patient carrying a deletion of exons 45-55 of the dystrophin gene (CCMi002BMD-A-9 ∆45-55).

PubMed

Gowran, Aoife; Spaltro, Gabriella; Casalnuovo, Federica; Vigorelli, Vera; Spinelli, Pietro; Castiglioni, Elisa; Rovina, Davide; Paganini, Stefania; Di Segni, Marina; Gervasini, Cristina; Nigro, Patrizia; Pompilio, Giulio

2018-04-01

Becker muscular dystrophy (BMD) is a dystrophinopathy caused by mutations in the dystrophin gene on chromosome Xp21. BMD mutations result in truncated semi-functional dystrophin isoforms. Consequently, less severe clinical symptoms become apparent later in life compared to Duchenne muscular dystrophy. Dermal fibroblasts from a BMD patient were electroporated with episomal plasmids containing reprogramming factors to create the induced pluripotent stem cell line: CCMi002BMD-A-9 that showed pluripotent markers, were karyotypically normal and capable of trilineage differentiation. MLPA analyses performed on DNA extracted from CCMi002BMD-A-9 showed an in-frame deletion of exons 45 to 55 (CCMi002BMD-A-9 Δ45-55). Copyright © 2018 The Author(s). Published by Elsevier B.V. All rights reserved.

Pattern of deletions of the dystrophin gene in Mexican Duchenne/Becker muscular dystrophy patients: the use of new designed primers for the analysis of the major deletion "hot spot" region.

PubMed

Coral-Vazquez, R; Arenas, D; Cisneros, B; Peñaloza, L; Salamanca, F; Kofman, S; Mercado, R; Montañez, C

1997-06-13

We have analyzed 59 unrelated Mexican Duchenne/Becker muscular dystrophy patients (DMD/BMD) using PCR analysis of the 2 prone deletion regions in the DMD gene. Thirty one (52%) of the patients had a deletion of one or several of the exons. Most of the alterations (87%) were clustered in exons 44-52, this being the highest percentage reported until now. In order to improve the molecular diagnosis in the Mexican population, we designed a new multiplex assay to PCR amplify exons 44-52. This assay allowed for the identification of a greater number of deletions in this region compared with the 9 and 5-plex assays previously described and to determine most of the deletion end boundaries. This is a reliable alternative for the initial screening of the DMD patients in the Mexican population.

Energy scaling of the "heartbeat" pulse width of GRS 1915+105, IGR J17091-3624, and MXB 1730-335 from Rossi-XTE observations

NASA Astrophysics Data System (ADS)

Maselli, A.; Capitanio, F.; Feroci, M.; Massa, F.; Massaro, E.; Mineo, T.

2018-04-01

We investigate some key aspects of the "heartbeat" variability consisting of series of bursts with a slow rise and a fast decay, thus far detected only in GRS 1915+105, IGR J17091-3624, and MXB 1730-335. A previous analysis based on BeppoSAX data of GRS 1915+105 revealed a hard-X delay (HXD), that is a lag of the burst rise at higher energies with respect to lower ones; this leads to narrower pulse widths, w, at higher energies. We here use some light curves of Rossi-XTE observations of GRS 1915+105 for a deeper analysis of this effect and search for its presence in those extracted from some IGR J17091-3624 and MXB 1730-335 observations performed with the same satellite. Our results show that, at variance with GRS 1915+105, no HXD is evident in the light curves of MXB 1730-335 and only a marginal HXD may be argued for IGR J17091-3624. For GRS 1915+105 we find a decreasing trend of the pulse width with energy following a power law w = A ṡ E-s with an index s ≈ 0.8. Furthermore, we confirm the increase of the HXD with the recurrence time Trec of the bursts in each series that was already found in previous works using BeppoSAX data. Based on a spectral analysis of these three sources we conclude that the differences highlighted in the properties of the "heartbeat" variability are probably related to the different accreting compact object and the eventual presence of a corona in these binary interacting systems.

Interpretation of acid α-glucosidase activity in creatine kinase elevation: A case of Becker muscular dystrophy.

PubMed

Oitani, Yoshiki; Ishiyama, Akihiko; Kosuga, Motomichi; Iwasawa, Kentaro; Ogata, Ayako; Tanaka, Fumiko; Takeshita, Eri; Shimizu-Motohashi, Yuko; Komaki, Hirofumi; Nishino, Ichizo; Okuyama, Torayuki; Sasaki, Masayuki

2018-05-16

Diagnosis of Pompe disease is sometimes challenging because it exhibits clinical similarities to muscular dystrophy. We describe a case of Becker muscular dystrophy (BMD) with a remarkable reduction in activity of the acid α-glucosidase (GAA) enzyme, caused by a combination of pathogenic mutation and polymorphism variants resulting in pseudodeficiency in GAA. The three-year-old boy demonstrated asymptomatic creatine kinase elevation. Neither exon deletion nor duplication was detected on multiplex ligation-dependent probe amplification (MLPA) of DMD. GAA enzyme activity in both dried blood spots and lymphocytes was low, at 11.7% and 7.7% of normal, respectively. However, genetic analysis of GAA detected only heterozygosity for a nonsense mutation (c.118C > T, p.Arg40 ∗ ). Muscle pathology showed no glycogen deposits and no high acid phosphatase activity. Hematoxylin-eosin staining detected scattered regenerating fibers; the fibers were faint and patchy on immunochemistry staining of dystrophin. The amount of dystrophin protein was reduced to 11.8% of normal, on Western blotting analysis. Direct sequencing analysis of DMD revealed hemizygosity for a nonsense mutation (c.72G > A, p.Trp24 ∗ ). The boy was diagnosed with BMD, despite remarkable reduction in GAA activity; further, he demonstrated heterozygosity for [p.Gly576Ser; p.Glu689Lys] polymorphism variants that indicated pseudodeficiency on another allele in GAA. Pseudodeficiency alleles are detected in approximately 4% of the Asian population; these demonstrate low activity of acid α-glucosidase (GAA), similar to levels found in Pompe disease. Clinicians should be careful in their interpretations of pseudodeficiency alleles that complicate diagnosis in cases of elevated creatine kinase. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Duchenne/Becker muscular dystrophy: A report on clinical, biochemical, and genetic study in Gujarat population, India

PubMed Central

Rao, Mandava V.; Sindhav, Gaurang M.; Mehta, Jitendra J.

2014-01-01

Objective: In India, various groups have studied different regions to find out deletion pattern of dystrophin gene. We have investigated its deletion pattern among Duchenne/Becker muscular dystrophy (D/BMD) patients across Gujarat. Moreover, in this study we also correlate the same with reading frame rule. However, we too consider various clinicopathological features to establish as adjunct indices when deletion detection fails. Materials and Methods: In this pilot study, a total of 88 D/BMD patients consulting at our centers in Gujarat, India were included. All patients were reviewed on basis of their clinical characteristics, tested by three primer sets of 10-plex, 9-plex, and 7-plex polymerase chain reaction (PCR) for genetic analysis; whereas, biochemical indices were measured using automated biochemical analyzers. Results: The diagnosis of D/BMD was confirmed by multiplex-PCR (M-PCR) in D/BMD patients. A number of 65 (73.86%) out of 88 patients showed deletion in dystrophin gene. The exon 50 (58.46%) was the most frequent deletion found in our study. The mean age of onset of DMD and BMD was 4.09 ± 0.15 and 7.14 ± 0.55 years, respectively. In patients, mean creatine phosphokinase (CPK), lactate dehydrogenase (LDH), and myoglobin levels were elevated significantly (P < 0.05) in comparison to controls. Addition to CPK, LDH and myoglobin are good adjunct when deletion detection failed. These data are further in accordance with world literature when correlated with frame rule. Conclusion: The analysis has been carried out for the first time for a total of 88 D/BMD patients particularly from Gujarat, India. More research is essential to elucidate specific mutation pattern in association with management and therapies of proband. PMID:25221400

Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

PubMed

Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

2015-03-01

We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

Serum Creatinine Distinguishes Duchenne Muscular Dystrophy from Becker Muscular Dystrophy in Patients Aged ≤3 Years: A Retrospective Study

PubMed Central

Wang, Liang; Chen, Menglong; He, Ruojie; Sun, Yiming; Yang, Juan; Xiao, Lulu; Cao, Jiqing; Zhang, Huili; Zhang, Cheng

2017-01-01

Here, we investigated correlations between serum creatinine (SCRN) levels and clinical phenotypes of dystrophinopathy in young patients. Sixty-eight patients with dystrophinopathy at the Neuromuscular Clinic, The First Affiliated Hospital, Sun Yat-sen University, were selected for this study. The diagnosis of dystrophinopathy was based on clinical manifestation, biochemical changes, and molecular analysis. Some patients underwent muscle biopsies; SCRN levels were tested when patients were ≤3 years old, and reading frame changes were analyzed. Each patient was followed up, and motor function and clinical phenotype were assessed when the same patients were ≥4 years old. Our findings indicated that in young patients, lower SCRN levels were associated with increased disease severity (p < 0.01) and that SCRN levels were the highest in patients exhibiting mild Becker muscular dystrophy (BMD) (p < 0.001) and the lowest in patients with Duchenne muscular dystrophy (DMD) (p < 0.01) and were significantly higher in patients carrying in-frame mutations than in patients carrying out-of-frame mutations (p < 0.001). SCRN level cutoff values for identifying mild BMD [18 µmol/L; area under the curve (AUC): 0.947; p < 0.001] and DMD (17 µmol/L; AUC: 0.837; p < 0.001) were established. These results suggest that SCRN might be a valuable biomarker for distinguishing DMD from BMD in patients aged ≤3 years and could assist in the selection of appropriate treatment strategies. PMID:28533764

MLPA based detection of mutations in the dystrophin gene of 180 Polish families with Duchenne/Becker muscular dystrophy.

PubMed

Zimowski, Janusz G; Massalska, Diana; Holding, Mariola; Jadczak, Sylwia; Fidziańska, Elżbieta; Lusakowska, Anna; Kostera-Pruszczyk, Anna; Kamińska, Anna; Zaremba, Jacek

2014-01-01

Duchenne/Becker muscular dystrophy (DMD/BMD) is a recessive, X-linked disorder caused by a mutation in the dystrophin gene. Deletions account for approximately 60-65% of mutations, duplications for 5-10%. The remaining cases are mainly point mutations. According to Monaco theory clinical form of the disease depends on maintaining or disrupting the reading frame. The purpose of the study was to determine frequency and location of deletions and duplications in the dystrophin gene, to determine the compliance between maintaining/disrupting the reading frame and clinical form of the disease and to check the effectiveness of MLPA (multiplex ligation-dependent probe amplification) in the detection of these mutations in hemizygous patients and heterozygous female carriers. The material is composed of combined results of molecular diagnosis carried out in years 2009-2012 in 180 unrelated patients referred with the diagnosis of DMD/BMD tested by use of MLPA. We identified 110 deletions, 22 duplication (in one patient two different duplications were detected) and 2 point mutations. Deletions involved mainly exons 45-54 and 3-21, whereas most duplications involved exons 3-18. The compliance with Monaco theory was 95% for deletions and 76% for duplications. Most of mutations in the dystrophin gene were localized in the hot spots - different for deletions and duplications. MLPA enabled their quick identification, exact localization and determination whether or not they maintained or disrupted the reading frame. MLPA was also effective in detection of deletions and duplications in female carriers. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Engaging patients and caregivers in prioritizing symptoms impacting quality of life for Duchenne and Becker muscular dystrophy.

PubMed

Hollin, Ilene L; Peay, Holly; Fischer, Ryan; Janssen, Ellen M; Bridges, John F P

2018-05-26

Patient preference information (PPI) have an increasing role in regulatory decision-making, especially in benefit-risk assessment. PPI can also facilitate prioritization of symptoms to treat and inform meaningful selection of clinical trial endpoints. We engaged patients and caregivers to prioritize symptoms of Duchenne and Becker muscular dystrophy (DBMD) and explored preference heterogeneity. Best-worst scaling (object case) was used to assess priorities across 11 symptoms of DBMD that impact quality of life and for which there is unmet need. Respondents selected the most and least important symptoms to treat among a subset of five. Relative importance scores were estimated for each symptom, and preference heterogeneity was identified using mixed logit and latent class analysis. Respondents included patients (n = 59) and caregivers (n = 96) affected by DBMD. Results indicated that respondents prioritized "weaker heart pumping" [score = 5.13; 95% CI (4.67, 5.59)] and pulmonary symptoms: "lung infections" [3.15; (2.80, 3.50)] and "weaker ability to cough" [2.65; (2.33, 2.97)] as the most important symptoms to treat and "poor attention span" as the least important symptom to treat [- 5.23; (- 5.93, - 4.54)]. Statistically significant preference heterogeneity existed (p value < 0.001). At least two classes existed with different priorities. Priorities of the majority latent class (80%) reflected the aggregate results, whereas the minority latent class (20%) did not distinguish among pulmonary and other symptoms. Estimates of the relative importance for symptoms of Duchenne muscular dystrophy indicated that symptoms with direct links to morbidity and mortality were prioritized above other non-skeletal muscle symptoms. Findings suggested the existence of preference heterogeneity for symptoms, which may be related to symptom experience.

Serum Creatinine Distinguishes Duchenne Muscular Dystrophy from Becker Muscular Dystrophy in Patients Aged ≤3 Years: A Retrospective Study.

PubMed

Wang, Liang; Chen, Menglong; He, Ruojie; Sun, Yiming; Yang, Juan; Xiao, Lulu; Cao, Jiqing; Zhang, Huili; Zhang, Cheng

2017-01-01

Here, we investigated correlations between serum creatinine (SCRN) levels and clinical phenotypes of dystrophinopathy in young patients. Sixty-eight patients with dystrophinopathy at the Neuromuscular Clinic, The First Affiliated Hospital, Sun Yat-sen University, were selected for this study. The diagnosis of dystrophinopathy was based on clinical manifestation, biochemical changes, and molecular analysis. Some patients underwent muscle biopsies; SCRN levels were tested when patients were ≤3 years old, and reading frame changes were analyzed. Each patient was followed up, and motor function and clinical phenotype were assessed when the same patients were ≥4 years old. Our findings indicated that in young patients, lower SCRN levels were associated with increased disease severity ( p  < 0.01) and that SCRN levels were the highest in patients exhibiting mild Becker muscular dystrophy (BMD) ( p  < 0.001) and the lowest in patients with Duchenne muscular dystrophy (DMD) ( p  < 0.01) and were significantly higher in patients carrying in-frame mutations than in patients carrying out-of-frame mutations ( p  < 0.001). SCRN level cutoff values for identifying mild BMD [18 µmol/L; area under the curve (AUC): 0.947; p  < 0.001] and DMD (17 µmol/L; AUC: 0.837; p  < 0.001) were established. These results suggest that SCRN might be a valuable biomarker for distinguishing DMD from BMD in patients aged ≤3 years and could assist in the selection of appropriate treatment strategies.

Social involvement issues in patients with Becker muscular dystrophy: A questionnaire survey of subjects from a patient registry.

PubMed

Mori-Yoshimura, Madoka; Mizuno, Yukio; Yoshida, Sumiko; Minami, Narihiro; Yonemoto, Naohiro; Takeuchi, Fumi; Nishino, Ichizo; Murata, Miho; Takeda, Shin'ichi; Takahashi, Yuji; Kimura, En

2018-04-01

Little is known about the relationship between Becker Muscular Dystrophy (BMD) and developmental problems, school life, employment, and mental problems. We aimed to clarify whether BMD is a risk factor for developmental disorders, problematic behavior, psychiatric diseases, and other social difficulties in school life and employment. Adults with genetically or immunohistochemically confirmed BMD from the Registry of Muscular Dystrophy in Japan (REMUDY) were asked to complete a questionnaire regarding patient history, school life, employment, and mental problems. In total, 125 (68.3%) of 183 participants with BMD (median age, 37.2 years) completed the questionnaire. Of these, ten had developmental disorders (mental retardation, autism, and speech disturbance). Fifty-eight (44%) experienced bullying in school, and 39 felt the reason for bullying was physical handicap. Sixteen participants experienced problematic behavior such as cutting class, domestic violence, violent incidents, suicide attempts, or self-mutilation. Employment histories were noted by 92 (73%), of whom 15 could not continue to work due to physical handicaps. Fifteen participants had psychiatric disorders, with 5, 3 and 1 having neurosis, depression, and bipolar disorder, respectively. The other 6 participants with psychiatric disorders did not specify their diagnoses. Patients carrying a Dp140 expression change had significantly more incidences of developmental disorders, but not bullying, problematic behavior, workplace difficulties, or psychiatric disorders. Patients with BMD risk bullying and workplace difficulties, as well as developing psychiatric disorders. Parents, teachers, and supporters should be mindful of the daily environment of BMD patients and provide support to help them cope with stress. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Becker muscular dystrophy-like myopathy regarded as so-called "fatty muscular dystrophy" in a pig: a case report and its diagnostic method.

PubMed

Horiuchi, Noriyuki; Aihara, Naoyuki; Mizutani, Hiroshi; Kousaka, Shinichi; Nagafuchi, Tsuneyuki; Ochiai, Mariko; Ochiai, Kazuhiko; Kobayashi, Yoshiyasu; Furuoka, Hidefumi; Asai, Tetsuo; Oishi, Koji

2014-03-01

We describe a case of human Becker muscular dystrophy (BMD)-like myopathy that was characterized by the declined stainability of dystrophin at sarcolemma in a pig and the immunostaining for dystrophin on the formalin-fixed, paraffin-embedded (FFPE) tissue. The present case was found in a meat inspection center. The pig looked appeared healthy at the ante-mortem inspection. Muscular abnormalities were detected after carcass dressing as pale, discolored skeletal muscles with prominent fat infiltrations and considered so-called "fatty muscular dystrophy". Microscopic examination revealed following characteristics: diffused fat infiltration into the skeletal muscle and degeneration and regeneration of the remaining skeletal muscle fibers. Any lesions that were suspected of neurogenic atrophy, traumatic muscular degeneration, glycogen storage disease or other porcine muscular disorders were not observed. The immunostaining for dystrophin was conducted and confirmed to be applicable on FFPE porcine muscular tissues and revealed diminished stainability of dystrophin at the sarcolemma in the present case. Based on the histological observations and immunostaining results, the present case was diagnosed with BMD-like myopathy associated with dystrophin abnormality in a pig. Although the genetic properties were not clear, the present BMD-like myopathy implied the occurrence of dystrophinopathy in pigs. To the best of our knowledge, this is the first report of a natural case of myopathy associated with dystrophin abnormalities in a pig.

Ultrasound tissue characterization does not differentiate genotype, but indexes ejection fraction deterioration in becker muscular dystrophy.

PubMed

Giglio, Vincenzo; Puddu, Paolo Emilio; Holland, Mark R; Camastra, Giovanni; Ansalone, Gerardo; Ricci, Enzo; Mela, Julia; Sciarra, Federico; Di Gennaro, Marco

2014-12-01

The aims of the study were, first, to assess whether myocardial ultrasound tissue characterization (UTC) in Becker muscular dystrophy (BMD) can be used to differentiate between patients with deletions and those without deletions; and second, to determine whether UTC is helpful in diagnosing the evolution of left ventricular dysfunction, a precursor of dilated cardiomyopathy. Both cyclic variation of integrated backscatter and calibrated integrated backscatter (cIBS) were assessed in 87 patients with BMD and 70 controls. The average follow-up in BMD patients was 48 ± 12 mo. UTC analysis was repeated only in a subgroup of 40 BMD patients randomly selected from the larger overall group (15 with and 25 without left ventricular dysfunction). Discrimination between BMD patients with and without dystrophin gene deletion was not possible on the basis of UTC data: average cvIBS was 5.2 ± 1.2 and 5.5 ± 1.4 dB, and average cIBS was 29.9 ± 4.7 and 29.6 ± 5.8, respectively, significantly different (p < 0.001) only from controls (8.6 ± 0.5 and 24.6 ± 1.2 dB). In patients developing left ventricular dysfunction during follow-up, cIBS increased to 31.3 ± 5.4 dB, but not significantly (p = 0.08). The highest cIBS values (34.6 ± 5.3 dB, p < 0.09 vs. baseline, p < 0.01 vs BMD patients without left ventricular dysfunction) were seen in the presence of severe left ventricular dysfunction. Multivariate statistics indicated that an absolute change of 6 dB in cIBS is associated with a high probability of left ventricular dysfunction. UTC analysis does not differentiate BMD patients with or without dystrophin gene deletion, but may be useful in indexing left ventricular dysfunction during follow-up. Copyright © 2014 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

First checklist of the fruit flies of Morocco, including new records (Diptera, Tephritidae)

PubMed Central

Harym, Younes El; Belqat, Boutaïna

2017-01-01

Abstract The first checklist of the Tephritidae of Morocco, containing 59 species, is presented here. Out of 38 species collected during the present project, three (Campiglossa martii (Becker, 1908), Tephritis divisa (Rondani, 1871), and Terellia sp. near longicauda) present new records for North Africa, and ten (Carpomya incompleta (Becker, 1903), Chaetorellia conjuncta (Becker, 1913), Chetostoma curvinerve Rondani, 1856, Dacus frontalis (Becker, 1922), D. longistylus (Wiedemann, 1830), Dioxyna sororcula (Wiedemann, 1830), Ensina sonchi (Linnaeus, 1767), Myopites inulaedyssentericae Blot, 1827, M. stylatus Fabricius, 1794, and Tephritis vespertina (Loew, 1844)) are new for Morocco. PMID:29118602

Dystrophin Hot-Spot Mutants Leading to Becker Muscular Dystrophy Insert More Deeply into Membrane Models than the Native Protein.

PubMed

Ameziane-Le Hir, Sarah; Paboeuf, Gilles; Tascon, Christophe; Hubert, Jean-François; Le Rumeur, Elisabeth; Vié, Véronique; Raguénès-Nicol, Céline

2016-07-26

Dystrophin (DYS) is a membrane skeleton protein whose mutations lead to lethal Duchenne muscular dystrophy or to the milder Becker muscular dystrophy (BMD). One third of BMD "in-frame" exon deletions are located in the region that codes for spectrin-like repeats R16 to R21. We focused on four prevalent mutated proteins deleted in this area (called RΔ45-47, RΔ45-48, RΔ45-49, and RΔ45-51 according to the deleted exon numbers), analyzing protein/membrane interactions. Two of the mutants, RΔ45-48 and RΔ45-51, led to mild pathologies and displayed a similar triple coiled-coil structure as the full-length DYS R16-21, whereas the two others, RΔ45-47 and RΔ45-49, induced more severe pathologies and showed "fractional" structures unrelated to the normal one. To explore lipid packing, small unilamellar liposomes (SUVs) and planar monolayers were used at various initial surface pressures. The dissociation constants determined by microscale thermophoresis (MST) were much higher for the full-length DYS R161-21 than for the mutants; thus the wild type protein has weaker SUV binding. Comparing surface pressures after protein adsorption and analysis of atomic force microscopy images of mixed protein/lipid monolayers revealed that the mutants insert more into the lipid monolayer than the wild type does. In fact, in both models every deletion mutant showed more interactions with membranes than the full-length protein did. This means that mutations in the R16-21 part of dystrophin disturb the protein's molecular behavior as it relates to membranes, regardless of whether the accompanying pathology is mild or severe.

Skewed X-chromosome inactivation plays a crucial role in the onset of symptoms in carriers of Becker muscular dystrophy.

PubMed

Viggiano, Emanuela; Picillo, Esther; Ergoli, Manuela; Cirillo, Alessandra; Del Gaudio, Stefania; Politano, Luisa

2017-04-01

Becker muscular dystrophy (BMD) is an X-linked recessive disorder affecting approximately 1: 18.000 male births. Female carriers are usually asymptomatic, although 2.5-18% may present muscle or heart symptoms. In the present study, the role of the X chromosome inactivation (XCI) on the onset of symptoms in BMD carriers was analysed and compared with the pattern observed in Duchenne muscular dystrophy (DMD) carriers. XCI was determined on the lymphocytes of 36 BMD carriers (both symptomatic and not symptomatic) from 11 families requiring genetic advice at the Cardiomyology and Medical Genetics of the Second University of Naples, using the AR methylation-based assay. Carriers were subdivided into two groups, according to age above or below 50 years. Seven females from the same families known as noncarriers were used as controls. A Student's t-test for nonpaired data was performed to evaluate the differences observed in the XCI values between asymptomatic and symptomatic carriers, and carriers aged above or below 50 years. A Pearson correlation test was used to evaluate the inheritance of the XCI pattern in 19 mother-daughter pairs. The results showed that symptomatic BMD carriers had a skewed XCI with a preferential inactivation of the X chromosome carrying the normal allele, whereas the asymptomatic carriers and controls showed a random XCI. No concordance concerning the XCI pattern was observed between mothers and related daughters. The data obtained in the present study suggest that the onset of symptoms in BMD carriers is related to a skewed XCI, as observed in DMD carriers. Furthermore, they showed no concordance in the XCI pattern inheritance. Copyright © 2017 John Wiley & Sons, Ltd.

Characterization of Ignition and Combustion Properties of Nanowire-based Energetics

DTIC Science & Technology

2013-07-23

Sannia, A. Cincotti, G. Cao, Chem. Eng. Sci. 54 (15–16) (1999) 3053–3061. [4] C. Rossi, K. Zhang, D. Esteve, P. Alphonse , P. Tailhades, C. Vahlas, J...94 (5) (2003) 2923–2929. [30] K. Zhang, C. Rossi, G.A.A. Rodriguez, C. Tenail- leau, P. Alphonse , Appl. Phys. Lett. 91 (11) (2007) 113117/1–113117/3...Pister, Smart Mater. Struct. 10(6), 1145 (2001). 8C. Rossi, K. Zhang, D. Esteve, P. Alphonse , P. Tailhades, and C. Vahlas, J. Microelectromech. Syst. 16

Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study.

PubMed

Magliano, Lorenza; D'Angelo, Maria Grazia; Vita, Giuseppe; Pane, Marika; D'Amico, Adele; Balottin, Umberto; Angelini, Corrado; Battini, Roberta; Politano, Luisa; Patalano, Melania; Sagliocchi, Alessandra; Civati, Federica; Brighina, Erika; Vita, Gian Luca; Messina, Sonia; Sframeli, Maria; Lombardo, Maria Elena; Scalise, Roberta; Colia, Giulia; Catteruccia, Maria; Berardinelli, Angela; Motta, Maria Chiara; Gaiani, Alessandra; Semplicini, Claudio; Bello, Luca; Astrea, Guja; Zaccaro, Antonella; Scutifero, Marianna

2014-12-01

This study explored the burden in parents and healthy siblings of 4-17 year-old patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies, and whether the burden varied according to clinical aspects and social resources. Data on socio-demographic characteristics, patient's clinical history, parent and healthy children burden, and on parent's social resources were collected using self-reported questionnaires administered to 336 parents of patients with DMD (246) and BMD (90). Parents of patients with DMD reported higher burden than those of patients with BMD, especially concerning feeling of loss (84.3% DMD vs. 57.4% BMD), stigma (44.2% DMD vs. 5.5% BMD) and neglect of hobbies (69.0% DMD vs. 32.5% BMD). Despite the burden, 66% DMD and 62.4% BMD parents stated the caregiving experience had a positive impact on their lives. A minority of parents believed MD has a negative influence on the psychological well-being (31.0% DMD vs. 12.8% BMD), and social life of unaffected children (25.7% vs. 18.4%). In the DMD group, burden correlated with duration of illness and parent age, and burden was higher among parents with lower social contacts and support in emergencies. In DMD, difficulties among healthy children were reported as higher by parents who were older, had higher burden and lower social contacts. In both groups, burden increased in relation to patient disability. These findings underline that the psychological support to be provided to parents of patients with MD, should take into account clinical features of the disease.

Comparative cost of illness analysis and assessment of health care burden of Duchenne and Becker muscular dystrophies in Germany.

PubMed

Schreiber-Katz, Olivia; Klug, Constanze; Thiele, Simone; Schorling, Elisabeth; Zowe, Janet; Reilich, Peter; Nagels, Klaus H; Walter, Maggie C

2014-12-18

Our study aimed to determine the burden of illness in dystrophinopathy type Duchenne (DMD) and Becker (BMD), both leading to progressive disability, reduced working capacity and high health care utilization. A micro-costing method was used to examine the direct, indirect and informal care costs measuring the economic burden of DMD in comparison to BMD on patients, relatives, payers and society in Germany and to determine the health care burden of these diseases. Standardized questionnaires were developed based on predefined structured interview guidelines to obtain data directly from patients and caregivers using the German dystrophinopathy patient registry. The health-related quality of life (HRQOL) was analyzed using PedsQL™ Measurement Model. In total, 363 patients with genetically confirmed dystrophinopathies were enrolled. Estimated annual disease burden including direct medical/non-medical, indirect and informal care costs of DMD added up to € 78,913 while total costs in BMD were € 39,060. Informal care costs, indirect costs caused by loss of productivity and absenteeism of patients and caregivers as well as medical costs of rehabilitation services and medical aids were identified as the most important cost drivers. Total costs notably increased with disease progression and were consistent with the clinical severity; however, patients' HRQOL declined with disease progression. In conclusion, early assessments of economic aspects and the disease burden are essential to gain extensive knowledge of a distinct disease and above all play an important role in funding drug development programs for rare diseases. Therefore, our results may help to accelerate payer negotiations such as the pricing and reimbursement of new therapies, and will hopefully contribute to facilitating the efficient translation of innovations from clinical research over marketing authorization to patient access to a causative treatment.

Molecular Analysis-Based Genetic Characterization of a Cohort of Patients with Duchenne and Becker Muscular Dystrophy in Eastern China.

PubMed

Zhao, Hui-Hui; Sun, Xue-Ping; Shi, Ming-Chao; Yi, Yong-Xiang; Cheng, Hong; Wang, Xing-Xia; Xu, Qing-Cheng; Ma, Hong-Ming; Wu, Hao-Quan; Jin, Qing-Wen; Niu, Qi

2018-04-05

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are common X-linked recessive neuromuscular disorders caused by mutations in dystrophin gene. Multiplex polymerase chain reaction (multiplex PCR) and multiplex ligation-dependent probe amplification (MLPA) are the most common methods for detecting dystrophin gene mutations. This study aimed to contrast the two methods and discern the genetic characterization of patients with DMD/BMD in Eastern China. We collected 121 probands, 64 mothers of probands, and 15 fetuses in our study. The dystrophin gene was detected by multiplex PCR primarily in 28 probands, and MLPA was used in multiplex PCR-negative cases subsequently. The dystrophin gene of the remaining 93 probands and 62 female potential carriers was tested by MLPA directly. In fetuses, multiplex PCR and MLPA were performed on 4 fetuses and 10 fetuses, respectively. In addition, sequencing was also performed in 4 probands with negative MLPA. We found that 61.98% of the subjects had genetic mutations including deletions (50.41%) and duplications (11.57%). There were 43.75% of mothers as carriers of the mutation. In 15 fetuses, 2 out of 7 male fetuses were found to be unhealthy and 2 out of 8 female fetuses were found to be carriers. Exons 3-26 and 45-52 have the maximum frequency in mutation regions. In the frequency of exons individually, exon 47 and exon 50 were the most common in deleted regions and exons 5, 6, and 7 were found most frequently in duplicated regions. MLPA has better productivity and sensitivity than multiplex PCR. Prenatal diagnosis should be applied in DMD high-risk fetuses to reduce the disease incidence. Furthermore, it is the responsibility of physicians to inform female carriers the importance of prenatal diagnosis.

[Comparison analysis of muscle enzymes in children with myocarditis and Duchene/Becker muscular dystrophy].

PubMed

Zhang, Yali; Wang, Hong; Yu, Xuexin; Xing, Yanlin; Wang, Ce; He, Rong

2016-09-28

To compare the changes in muscle enzyme between children with myocarditis and Duchene/Becker muscular dystrophy (DMD/BMD), and to seek the explanations for variation.
 The retrospective analysis for 83 myocarditis children (myocarditis group) and 69 DMD/BMD children (DMD/BMD group), who were collected from Department of Pediatric of Shengjing Hospital affiliated to China Medical University since January 2008 to May 2015, was carried out. At the same time, 24 healthy children from the Department of Pediatric Development served as a control group. The examination indexes included creatine kinase (CK), creatine kinase-isoenzyme MB (CK-MB), creatine kinase isoenzyme MB mass (CK-MB mass), cardiac troponin I (cTnI) and high-sensitive-cTnT (hs-cTnT).
 1) In the myocarditis group, the CK increased from 100 to 1 000 U/L, reached a peak after 5 days, which lasted for a week and then dropped to the normal; the CK-MB reached a peak after 5 to 7 days and dropped to the normal a month later; the CK-MB mass reached a peak on the first day and dropped to the normal after 3 weeks; the cTn reached to a peak after 5 days and dropped to the normal after about 17 days; hs-cTnT reached to a peak on the first day and dropped to the normal after about 19 days. 2) In the DMD/BMD group, the CK increased significantly and 27 cases had a CK value of more than 10 000 U/L. After the treatment for 1 to 2 weeks, their enzyme rose again after a slight drop. In terms of cTnI, 6 cases showed a moderate increase, 5 of them couldn't drop to the normal level until more than 3 weeks later; the hs-cTnT increased in the 45 cases, which lasted for more than 3 weeks in the 31 cases of them and showed a tendency of persisting increase.
 The cTnI and hs-cTnT rise significantly and possess wider observation window than CK and CK-MB mass in myocarditis children, with more sensitive and specific changes. The myocardial damage can occur before myasthenia and keep this trend for a long time in the DMD

Molecular characterization of Babesia species in wild animals and their ticks in Turkey.

PubMed

Orkun, Ömer; Karaer, Zafer

2017-11-01

To date, no study has investigated Babesia ecology in wild boars, hares or foxes in Turkey. This study aimed to determine and characterize Babesia spp. in wild animals and their ticks. We identified a novel Babesia genotype and four known Babesia species in wild animals and their ticks. We detected Babesia spp. molecularly in hares for the first time. In addition, we identified B. vulpes in foxes for the first time in Turkey. The presence of B. rossi, B. crassa and B. occultans was also revealed in ticks collected from wild boars and hares. This is only the second report of B. rossi in ticks outside of Africa and suggests that B. rossi is circulating in ticks in Turkey. Therefore B. rossi poses a significant threat to domestic dogs. Here we demonstrate the role of wild animals in the life cycle of Babesia species in Turkey and contribute to Babesia ecological and taxonomic information. Copyright © 2017 Elsevier B.V. All rights reserved.

Duchenne Muscular Dystrophy and Becker Muscular Dystrophy Confirmed by Multiplex Ligation-Dependent Probe Amplification: Genotype-Phenotype Correlation in a Large Cohort.

PubMed

Vengalil, Seena; Preethish-Kumar, Veeramani; Polavarapu, Kiran; Mahadevappa, Manjunath; Sekar, Deepha; Purushottam, Meera; Thomas, Priya Treesa; Nashi, Saraswathi; Nalini, Atchayaram

2017-01-01

Studies of cases of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) confirmed by multiplex ligation-dependent probe amplification (MLPA) have determined the clinical characteristics, genotype, and relations between the reading frame and phenotype for different countries. This is the first such study from India. A retrospective genotype-phenotype analysis of 317 MLPA-confirmed patients with DMD or BMD who visited the neuromuscular clinic of a quaternary referral center in southern India. The 317 patients comprised 279 cases of DMD (88%), 32 of BMD (10.1%), and 6 of intermediate phenotype (1.9%). Deletions accounted for 91.8% of cases, with duplications causing the remaining 8.2%. There were 254 cases of DMD (91%) with deletions and 25 (9%) due to duplications, and 31 cases (96.8%) of BMD with deletions and 1 (3.2%) due to duplication. All six cases of intermediate type were due to deletions. The most-common mutation was a single-exon deletion. Deletions of six or fewer exons constituted 68.8% of cases. The deletion of exon 50 was the most common. The reading-frame rule held in 90% of DMD and 94% of BMD cases. A tendency toward a lower IQ and earlier wheelchair dependence was observed with distal exon deletions, though a significant correlation was not found. The reading-frame rule held in 90% to 94% of children, which is consistent with reports from other parts of the world. However, testing by MLPA is a limitation, and advanced sequencing methods including analysis of the structure of mutant dystrophin is needed for more-accurate assessments of the genotype-phenotype correlation.

Transplantation of human umbilical cord-derived mesenchymal stems cells for the treatment of Becker muscular dystrophy in affected pedigree members.

PubMed

Li, Pang; Cui, Kai; Zhang, Bo; Wang, Zhendan; Shen, Yangyang; Wang, Xiangyu; Zhang, Jianbo; Tong, Feng; Li, Sheng

2015-04-01

The regeneration of muscle tissue has been achieved using multipotent mesenchymal stem cells in mouse models of injured skeletal muscle. In the present study, the utility of multipotent human umbilical cord-derived mesenchymal stem cells (hUC-MSCs) in the treatment of Becker muscular dystrophy (BMD), a genetic disease where muscle tissue fails to regenerate, was examined in members from a pedigree affected by BMD. The disease status was evaluated in 4 affected pedigree members (II1, II2, II3 and III2; aged 50, 46, 42 and 6 years, respectively). The transplantation of the hUC‑MSCs (performed on 3 patients, I2, II3 and III2) was performed by infusion with an intravenous drip over a 30‑min period, and the patients were evaluated at 1, 3, 4 and 12 weeks following the procedure. The evaluation was based on physical characteristics, as well as on molecular testing for serum creatine kinase (CK) and lactate dehydrogenase (LDH) levels and a histological examination of muscle biopsies. The patients suffered no adverse reactions in response to the transplantation of the hUC‑MSCs. At 1 week following transplantation all 3 patients showed improvement in the muscle force of the limbs, muscle size and daily activity. The walking gait of patient III2 had improved by 1 week post-transplantation and reached a normal status by 12 weeks. Serum CK and LDH levels were decreased relative to the baseline levels. A histological examination of muscle biopsies displayed no obvious tissue regeneration. In conclusion, the treatment of patients with BMD using hUC-MSCs was safe and of therapeutic benefit that lasted for up to 12 weeks. hUC-MSCs are, therefore, a potential cell therapy-based treatment option for patients with muscular dystrophies.

Mouse models of two missense mutations in actin-binding domain 1 of dystrophin associated with Duchenne or Becker muscular dystrophy.

PubMed

McCourt, Jackie L; Talsness, Dana M; Lindsay, Angus; Arpke, Robert W; Chatterton, Paul D; Nelson, D'anna M; Chamberlain, Christopher M; Olthoff, John T; Belanto, Joseph J; McCourt, Preston M; Kyba, Michael; Lowe, Dawn A; Ervasti, James M

2018-02-01

Missense mutations in the dystrophin protein can cause Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) through an undefined pathomechanism. In vitro studies suggest that missense mutations in the N-terminal actin-binding domain (ABD1) cause protein instability, and cultured myoblast studies reveal decreased expression levels that can be restored to wild-type with proteasome inhibitors. To further elucidate the pathophysiology of missense dystrophin in vivo, we generated two transgenic mdx mouse lines expressing L54R or L172H mutant dystrophin, which correspond to missense mutations identified in human patients with DMD or BMD, respectively. Our biochemical, histologic and physiologic analysis of the L54R and L172H mice show decreased levels of dystrophin which are proportional to the phenotypic severity. Proteasome inhibitors were ineffective in both the L54R and L172H mice, yet mice homozygous for the L172H transgene were able to express even higher levels of dystrophin which caused further improvements in muscle histology and physiology. Given that missense dystrophin is likely being degraded by the proteasome but whole body proteasome inhibition was not possible, we screened for ubiquitin-conjugating enzymes involved in targeting dystrophin to the proteasome. A myoblast cell line expressing L54R mutant dystrophin was screened with an siRNA library targeting E1, E2 and E3 ligases which identified Amn1, FBXO33, Zfand5 and Trim75. Our study establishes new mouse models of dystrophinopathy and identifies candidate E3 ligases that may specifically regulate dystrophin protein turnover in vivo. © The Author(s) 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Egg parasitoid of Saccharosydne subandina (Hemiptera: Delphacidae) in Neuquen, Argentina

USDA-ARS?s Scientific Manuscript database

Saccharosydne subandina Remes Lenicov & Rossi Batiz is a recently described planthopper from Argentina which is known to feed on garlic, rye, and pampas grass (de Remes-Lenicov & Rossi-Batiz 2010). During a trip to Neuquén Province in February 2007, we noticed a heavy infestation of pampas grass, Co...

Putting Science FIRST: Memories of Family Science Experiences.

ERIC Educational Resources Information Center

Science and Children, 1996

1996-01-01

Presents anecdotes from prominent citizens including Bill Clinton, Alan Alda, Carl Sagan, Gerald Wheeler, JoAnne Vasquez, and Lynn Margulis in which they reminisce about interesting science experiences with their families. (JRH)

Accelerating research through reagent repositories: the genome editing example.

PubMed

Joung, J Keith; Voytas, Daniel F; Kamens, Joanne

2015-11-20

Keith Joung, Dan Voytas and Joanne Kamens share insights into how the genome editing field was advanced by early access to biological resources and the role in this process that plasmid repositories play.

Patients with Duchenne muscular dystrophy are significantly shorter than those with Becker muscular dystrophy, with the higher incidence of short stature in Dp71 mutated subgroup.

PubMed

Matsumoto, Masaaki; Awano, Hiroyuki; Lee, Tomoko; Takeshima, Yasuhiro; Matsuo, Masafumi; Iijima, Kazumoto

2017-11-01

Duchenne and Becker muscular dystrophy (DMD/BMD) are caused by mutations in the dystrophin gene and are characterized by severe and mild progressive muscle wasting, respectively. Short stature has been reported as a feature of DMD in the Western hemisphere, but not yet confirmed in Orientals. Height of young BMD has not been fully characterized. Here, height of ambulant and steroid naive Japanese 179 DMD and 42 BMD patients between 4 and 10 years of age was retrospectively examined using height standard deviation score (SDS). The mean height SDS of DMD was -1.08 SD that was significantly smaller than normal (p < 0.001), indicating short stature of Japanese DMD. Furthermore, the mean height SDS of BMD was -0.27 SD, suggesting shorter stature than normal. Remarkably, the mean height SDS of DMD was significantly smaller than that of BMD (p < 0.0001). In DMD higher incidence of short stature (height SDS < -2.5 SD) was observed in Dp71 subgroup having mutations in dystrophin exons 63-79 than others having mutations in exons 1-62 (27.8% vs. 7.5%, p = 0.017). These suggested that height is influenced by dystrophin in not only DMD but also BMD and that dystrophin Dp71 has a role in height regulation. Copyright © 2017 Elsevier B.V. All rights reserved.

Cardiac profile of asymptomatic children with Becker and Duchenne muscular dystrophy under treatment with steroids and with/without perindopril.

PubMed

Mavrogeni, Sophie; Giannakopoulou, Aikaterini; Papavasiliou, Antigoni; Markousis-Mavrogenis, George; Pons, Roser; Karanasios, Evangelos; Noutsias, Michel; Kolovou, Genovefa; Papadopoulos, George

2017-07-24

To evaluate cardiovascular function in boys with Duchenne (DMD) and Becker (BMD) muscular dystrophy, using cardiac magnetic resonance (CMR). This is a single point cross sectional study of twenty-four boys with genetically ascertained DMD, and 10 with BMD, aged 10.5 ± 1.5 years (range 9-13), were prospectively evaluated by a 1.5 T system and compared with those of age-sex matched controls. The DMD patients were divided in 2 groups. Group A (N = 12) were under treatment with both deflazacort and perindopril, while Group B (n = 12) were under treatment with deflazacort, only. BMD patients did not take any medication. Biventricular function was assessed using a standard SSFP sequence. Late gadolinium enhancement (LGE) was assessed from T1 images taken 15 min after injection of 0.2 mg/Kg gadolinium DTPA using a 3D-T1-TFE sequence. Group A and BMDs were asymptomatic with normal ECG, 24 h ECG recording and echocardiogram. Group B were asymptomatic but 6/12 had abnormal ECG and mildly impaired LVEF. Their 24 h ECG recording revealed supraventricular and ventricular extrasystoles (all at 12-13 yrs). LV indices in Group A and BMD did not differ from those of controls. However, LV indices in Group B were significantly impaired compared with controls, Group A and BMDs (p < 0.001). An epicardial LGE area = 3 ± 0.5% of LV mass was identified in the posterolateral wall of LV only in 6/12 patients of Group B, but in not in any BMD or Group A. Children with either BMD or DMD under treatment with both deflazacort and perindopril present preserved LV function and lack of LGE. However, further large scale multicenter studies are warranted to confirm these data, including further CMR mapping approaches.

75 FR 4449 - Requested Administrative Waiver of the Coastwise Trade Laws

Federal Register 2010, 2011, 2012, 2013, 2014

2010-01-27

... electronic version of this document and all documents entered into this docket is available on the World Wide Web at http://www.regulations.gov . FOR FURTHER INFORMATION CONTACT: Joann Spittle, U.S. Department of...

Pinworms

MedlinePlus

... still be around. How Do I Keep Pinworms Away? The best way to keep from getting pinworms ... these simple steps, you can keep the worms away! Reviewed by: Joanne Murren-Boezem, MD Date reviewed: ...

The 6-minute walk test, motor function measure and quantitative thigh muscle MRI in Becker muscular dystrophy: A cross-sectional study.

PubMed

Fischer, Dirk; Hafner, Patricia; Rubino, Daniela; Schmid, Maurice; Neuhaus, Cornelia; Jung, Hans; Bieri, Oliver; Haas, Tanja; Gloor, Monika; Fischmann, Arne; Bonati, Ulrike

2016-07-01

Becker muscular dystrophy (BMD) has an incidence of 1 in 16 000 male births. This cross-sectional study investigated the relation between validated functional scores and quantitative MRI (qMRI) of thigh muscles in 20 ambulatory BMD patients, aged 18.3-60 years (mean 31.2; SD 11.1). Clinical assessments included the motor function measure (MFM) and its subscales, as well as timed function tests such as the 6-minute walk test (6MWT) and the timed 10-m run/walk test. Quantitative MRI of the thigh muscles included the mean fat fraction (MFF) using a 2-point Dixon (2-PD) technique, and transverse relaxation time (T2) measurements. The mean MFM value was 80.4%, SD 9.44 and the D1 subscore 54.5%, SD 19.9. The median 6MWT was 195m, IQR 160-330.2. The median 10-m run/walk test was 7.4 seconds, IQR 6.1-9.3. The mean fat fraction of the thigh muscles was 55.6%, SD 17.4%, mean T2 relaxation times of all muscles: 69.9 ms, SD 14.4. The flexors had the highest MFF and T2 relaxation times, followed by the extensors and the adductors. MFF and global T2 relaxation times were highly negatively correlated with the MFM total, D1-subscore and 6MWT, and positively correlated with the 10 m run/walk test time (p < 0.01). Age was not correlated with MFF, global T2 relaxation time or clinical assessments. Both MFF and T2 measures in the thigh muscle were well correlated with clinical function in BMD and may serve as a surrogate outcome measure in clinical trials. Copyright © 2016 Elsevier B.V. All rights reserved.

Genetics Home Reference: phosphoribosylpyrophosphate synthetase superactivity

MedlinePlus

... Sources for This Page Ahmed M, Taylor W, Smith PR, Becker MA. Accelerated transcription of PRPS1 in ... 11):7482-8. Citation on PubMed Becker MA, Smith PR, Taylor W, Mustafi R, Switzer RL. The ...

78 FR 67333 - Performance Review Board Membership

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2013-11-12

.... Arnold William G. Bostic, Jr. Stephen B. Burke Joanne Buenzli Crane Susan R. Helper Ron S. Jarmin Enrique... Stephens Sonja Steptoe Frank A. Vitrano Katherine K. Wallman Adam Wilczewski The term of each PRB member...

Phytophthora capsici - Loss of Heterozygosity (LOH): A Widespread Mechanism for Rapid Adaptation (7th Annual SFAF Meeting, 2012)

ScienceCinema

Mudge, Joanne

2018-01-15

Joanne Mudge on "Phytophthora capsici - Loss of Heterozygosity (LOH): A Widespread Mechanism for Rapid Mutation" at the 2012 Sequencing, Finishing, Analysis in the Future Meeting held June 5-7, 2012 in Santa Fe, New Mexico.

The Impact of Nanotechnology Energetics on the Department of Defense by 2035

DTIC Science & Technology

2010-02-17

Kaili Zhang, Daniel Esteve, Pierre Alphonse , Philippe Tailhades and Constantin Vahlas. “Nano-Energetic Materials for MEMS: A Review.” Journal of...on impact and the energetic compounds react. 16 Rossi, Carole, Kaili Zhang, Daniel Esteve, Pierre Alphonse , Philippe Tailhades and Constantin Vahlas...Rossi, Carole, Kaili Zhang, Daniel Esteve, Pierre Alphonse , Philippe Tailhades and Constantin Vahlas. “Nano-Energetic Materials for

Using Arrays to Build towards Multiplicative Thinking in the Early Years

ERIC Educational Resources Information Center

Jacob, Lorraine; Mulligan, Joanne

2014-01-01

In this article, Lorraine Jacob and Joanne Mulligan discuss how arrays can be used to promote students' early learning in relation to multiplication and division. They provide examples of activities that can be used from Foundation to Year 5.

IEEE International Semiconductor Laser Conference (14th) Held in Maui, Hawaii on September 19-23, 1994

DTIC Science & Technology

1994-09-23

subcommittee chairs, Tom Koch, James Whiteaway and Yasuhiko Arakawa, for their efforts. The other Conference Committee members, Gary Evans, Joanne...Academy of Sciences, St. Petersburg, Russia Asia end Ausat/agla K. Ikeda Chair: Yasuhiko Arakawa Mitsubishi Electric Corporation, Hyogo, Japan University

[Recreation for Youth with Deaf Blindness.

ERIC Educational Resources Information Center

Carr, Theresa, Ed.

1992-01-01

This newsletter special issue focuses on recreational programming for people with deaf blindness. The following individual articles are presented: "Strategies To Promote Community Integrated Recreation: Guidelines for Leisure Coaches" by Joann Enos (which lists seven such strategies); "Assessing Recreation and Leisure…

Natalist Policies in the United States

ERIC Educational Resources Information Center

Lopoo, Leonard M.; Raissian, Kerri M.

2012-01-01

This retrospective reviews the policies that affect the fertility of American women, both policies designed to alter fertility intentionally as well as those that change childbearing unintentionally. Becker's seminal work on the economics of fertility serves as the theoretical foundation for this literature. After describing Becker's economic…

Comparison of Serum rAAV Serotype-Specific Antibodies in Patients with Duchenne Muscular Dystrophy, Becker Muscular Dystrophy, Inclusion Body Myositis, or GNE Myopathy.

PubMed

Zygmunt, Deborah A; Crowe, Kelly E; Flanigan, Kevin M; Martin, Paul T

2017-09-01

Recombinant adeno-associated virus (rAAV) is a commonly used gene therapy vector for the delivery of therapeutic transgenes in a variety of human diseases, but pre-existing serum antibodies to viral capsid proteins can greatly inhibit rAAV transduction of tissues. Serum was assayed from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), inclusion body myositis (IBM), and GNE myopathy (GNE). These were compared to serum from otherwise normal human subjects to determine the extent of pre-existing serum antibodies to rAAVrh74, rAAV1, rAAV2, rAAV6, rAAV8, and rAAV9. In almost all cases, patients with measurable titers to one rAAV serotype showed titers to all other serotypes tested, with average titers to rAAV2 being highest in all instances. Twenty-six percent of all young normal subjects (<18 years old) had measurable rAAV titers to all serotypes tested, and this percentage increased to almost 50% in adult normal subjects (>18 years old). Fifty percent of all IBM and GNE patients also had antibody titers to all rAAV serotypes, while only 18% of DMD and 0% of BMD patients did. In addition, serum-naïve macaques treated systemically with rAAVrh74 could develop cross-reactive antibodies to all other serotypes tested at 24 weeks post treatment. These data demonstrate that most DMD and BMD patients should be amenable to vascular rAAV-mediated treatment without the concern of treatment blockage by pre-existing serum rAAV antibodies, and that serum antibodies to rAAVrh74 are no more common than those for rAAV6, rAAV8, or rAAV9.

76 FR 16432 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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2011-03-23

..., Neuroscience Center, 6001 Executive Boulevard, Rockville, MD 20852 (Virtual Meeting). Contact Person: JoAnn.../ NIH/DHHS/Neuroscience Center, 6001 Executive Boulevard, Suite 3208, MSC 9529, Bethesda, MD 20892-9529..., Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences...

Recreation and Natural Area Needs Assessment (GREAT III)

DTIC Science & Technology

1982-01-01

1970 The Pennsylvania State University: Research and Computer Technician for Dr. E. L. Bergman, Department of Horticulture . Education B.S. The...Publication 1974 Becker, R. H. and R. 0. Ray. "Accessibility: An Application of the New Technology." Therapeutic Recreation Journal, Vol. 8, No. 4. 1976 Becker

High School Students' Use of Paper-Based and Internet-Based Information Sources in the Engineering Design Process

ERIC Educational Resources Information Center

Pieper, Jon; Mentzer, Nathan

2013-01-01

Mentzer and Becker (2011) and Becker and Mentzer (2012) demonstrated that high school students engaged in engineering design problems spent more time accessing information and spent more time designing when provided with Internet access. They studied high school students engaged in an engineering design challenge. The two studies attempted to…

Expectations and experiences of investigators and parents involved in a clinical trial for Duchenne/Becker muscular dystrophy

PubMed Central

Peay, Holly L; Tibben, Aad; Fisher, Tyler; Brenna, Ethan; Biesecker, Barbara B

2014-01-01

Background The social context of rare disease research is changing, with increased community engagement around drug development and clinical trials. This engagement may benefit patients and families, but may also lead to heightened trial expectations and therapeutic misconception. Clinical investigators are also susceptible to harboring high expectations. Little is known about parental motivations and expectations for clinical trials for rare pediatric disorders. Purpose We describe the experience of parents and clinical investigators involved in a phase II clinical trial for Duchenne and Becker muscular dystrophy: their expectations, hopes, motivations, and reactions to the termination of the trial. Methods This qualitative study was based on interviews with clinical investigators and parents of sons with DBMD who participated in the phase IIa or IIb ataluren clinical trial in the United States. Interviews were transcribed and coded for thematic analysis. Results Participants were twelve parents of affected boys receiving active drug and nine clinical investigators. High trial expectations of direct benefit were reported by parents and many clinicians. Investigators described monitoring and managing parents’ expectations; several worried about their own involvement in increasing parents’ expectations. Most parents were able to differentiate their expectations from their optimistic hopes for a cure. Parents’ expectations arose from other parents, advocacy organizations, and the sponsor. All parents reported some degree of clinical benefit to their children. Secondary benefits were hopefulness and powerful feelings associated with active efforts to affect the disease course. Parents and clinical investigators reported strong, close relationships that were mutually important. Parents and clinicians felt valued by the sponsor for the majority of the trial. When the trial abruptly stopped, they described loss of engagement, distress, and feeling unprepared for

Adventures in Assessment: Learner-Centered Approaches to Assessment and Evaluation in Adult Literacy, 2002.

ERIC Educational Resources Information Center

Cora, Marie, Ed.

2002-01-01

This journal presents the following articles: "Introduction: Volume 14--Examining Performance" (Marie Cora) "Fair Assessment Practices: Giving Students Equitable Opportunities to Demonstrate Learning" (Linda Suskie); "Assessing Oral Communication at the Community Learning Center Development of the OPT (Oral Proficiency Test)" (JoAnne Hartel and…

Important Evidence Highlights the Meaning of Teacher-Child Relationships for Child Development. Commentary on: "Formations of Attachment Relationships towards Teachers Lead to Conclusions for Public Child Care"

ERIC Educational Resources Information Center

Sagi-Schwartz, Abraham

2016-01-01

In this commentary, Sagi-Schwartz evaluates the article by Beckh and Becker-Stoll (2016) on attachment relationships with non-parental caregivers and how it may contribute to public child care. Beckh and Becker-Stoll first describe important background about research on early parent-child relationships, and how their nature and quality might…

Whose Side Are We on and for Whom Do We Write? Notes on Issues and Challenges Facing Those Researching and Evaluating Public Policy

ERIC Educational Resources Information Center

Warren, Jon; Garthwaite, Kayleigh

2015-01-01

Becker (1967) poses the question "Whose side are we on?," a question which has become an enduring part of discussions within social scientific methodology. This paper explores the key issues in Becker's argument and considers its relevance to researchers today, locating this within a consideration of evaluation-based research and policy.…

Travel to Learn: How, When, and Why to Apply for Overseas Professional Development

ERIC Educational Resources Information Center

Cook, Erika; Becker, Aaron

2013-01-01

In this article, Erika Cook and Aaron Becker suggest teachers travel to "unfamiliar" lands to not only breathe life into global lessons but also to gain new perspectives and experiences as educators. For teachers who dread teaching about an unfamiliar place or culture, Cook and Becker recommend considering the very real opportunities to…

Definitions and Omissions of Heroism

ERIC Educational Resources Information Center

Martens, Jeffrey W.

2005-01-01

This article presents comments on "The Heroism of Women and Men" by Selwyn W. Becker and Alice H. Eagly. Their article specifically addressed the "cultural association of heroism with men and masculinity . . . in natural settings." Becker and Eagly evidenced roughly equivalent rates of heroism by women and men in a variety of settings. However,…

Knowledge and Intellectual Capital. Symposium 13. [Concurrent Symposium Session at AHRD Annual Conference, 2000.

ERIC Educational Resources Information Center

2000

Three presentations are provided from Symposium 13, Knowledge and Intellectual Capital, of the Academy of Human Resource Development (HRD) 2000 Conference proceedings. "Human Capital Measurement" (Joanne Provo) begins with a literature review that provides a context for understanding how investments in human capital add value to the…

76 FR 52287 - Political Activity-Federal Employees Residing In Designated Localities

Federal Register 2010, 2011, 2012, 2013, 2014

2011-08-22

... 21, 2011. ADDRESSES: Comments may be mailed to Elaine Kaplan, General Counsel, Room 7355, United... CONTACT: Jo-Ann Chabot, Office of the General Counsel, United States Office of Personnel Management, (202... Government of the District of Columbia. Section 7323(a) generally permits Federal employees who are not...

[Youth Smoking.

ERIC Educational Resources Information Center

Stare, Russell K., Ed.

1994-01-01

This issue of the newsletter "Prevention Forum" focuses on smoking among adolescents. The articles are as follows: (1) "Where There's Smoke--Will Prevention Put Out the Fire?" (Joanne Burgess), an overview of the Surgeon General's report "Preventing Tobacco Use among Young People," including interviews with prevention…

Making the Connection: Coordinating Education and Training for a Skilled Workforce. A Report on the Proceedings of the National Conference for State Leaders (Washington, D.C., July 8-10, 1991).

ERIC Educational Resources Information Center

Department of Health and Human Services, Washington, DC.

The following presentations are included in this document: "A Nation of Students" (Lamar Alexander); "Businesses Are Clamoring for Skilled Workers" (Betsy Brand); "We Need to Be Partners in Reality" (Roberts Jones); "The Value of a Working Parent as a Role Model" (JoAnne Barnhart); "Human Capital Is…

78 FR 36231 - 60-Day Notice of Proposed Information Collection: FHA-Disclosure of Adjustable Rate Mortgages...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-06-17

... Relay Service at (800) 877-8339. FOR FURTHER INFORMATION CONTACT: Joanne Kuzma, Director, Office of... calling the toll-free Federal Relay Service at (800) 877-8339. Copies of available documents submitted to... techniques or other forms of information technology, e.g., permitting electronic submission of responses. HUD...

Transfer and Dispositions in Writing Centers: A Cross-Institutional, Mixed-Methods Study

ERIC Educational Resources Information Center

Bromley, Pam; Northway, Kara; Schonberg, Eliana

2016-01-01

Taking a dispositional view of transfer and applying Joanne Lobato's "actor-oriented transfer perspective," we analyze student perceptions of writing center visits at three very different institutions. We find that, as a nonevaluative space where university students can develop metacognitive awareness across disciplines and over time,…

Becker muscular dystrophy

MedlinePlus

The health care provider will do a nervous system (neurological) and muscle exam. A careful medical history is also important, ... the legs and pelvis, then moves to the muscles of the shoulders, ... respiratory system Tests that may be done include: CPK blood ...

Koenraad Beckers | NREL

Science.gov Websites

postdoctoral researcher working on geothermal energy and CSP projects. His interests include heat and mass geothermal energy systems modeling, reservoir simulation, and economic analysis, as well as on the design and transfer, energy conversion and storage systems, reservoir modeling, and direct-use applications of thermal

Human Immunodeficiency Virus (HIV) Infections; Strain and Type Variations; Diagnosis and Prevention.

DTIC Science & Technology

1992-10-26

Scarlatti et al. 1992 (41) 1 1 Arendrup et al. 1992 (42) SIVsm/monkey 7 0 Zhang et al. manuscript (43) B) Sequential samples: serum collected >6 months...983-990. 1991. 12. Scarlatti , G, Lombardi, V, Plebani, A, Principi, N, Chiara, V, Ferraris, G, Bucceri, A, Feny6, E M, Wigzell, H, Rossi, P, and...envelope glycoprotein gp125 of human immunodeficiency virus type2. Manuscript. M2. Scarlatti , G, Albert, J, Rossi, P, Hodara, V, Biraghi, P, Muggiasca

Normal and Differential SAR Interferometry

DTIC Science & Technology

2007-02-01

incorporating the use of a rough DEM. [ Adragna 1995]. The same technique is also used for flat Earth removal, and for differential Interferometry (Chap..5...available at http://www.estec.esa.nl/confannoun/99b02/index.html Massonnet, D., K. Feigi, M. Rossi, and F. Adragna , 1994. Radar Interferometric Mapping...of Deformation in the Year After the Landers Earthquake, Nature, Vol. 369, pp. 227-230 Massonnet, D., M. Rossi, C. Carmona, F. Adragna , G. Peltzer

Comparing the Heroism of Women and Men

ERIC Educational Resources Information Center

Eagly, Alice H.; Becker, Selwyn W.

2005-01-01

This article presents comments on "Definitions and Omissions of Heroism" by Jeffery W. Martens which is a comment on the original article "The Heroism of Women and Men" by Selwyn W. Becker and Alice H. Eagly. Becker and Eagly welcome the opportunity to discuss the questions about defining heroism that Martens raised in his comment on their…

A Survey of Principles Instructors: Why Lecture Prevails

ERIC Educational Resources Information Center

Goffe, William L.; Kauper, David

2014-01-01

For many years, surveys have shown that lecture is the dominant method for teaching principles of economics (Watts and Schaur 2011; Watts and Becker 2008; Becker and Watts 1996, 2001a, b). The authors confirm this and augment it by asking why principles instructors teach the way they do. The respondents, 340 principles instructors at the 2012…

Digital data sets that describe aquifer characteristics of the Rush Springs Aquifer in western Oklahoma

USGS Publications Warehouse

Runkle, D.L.; Becker, M.F.; Rea, Alan

1997-01-01

This diskette contains digitized aquifer boundaries and maps of hydraulic conductivity, recharge, and ground-water level elevation contours for the Rush Spring aquifer in western Oklahoma. This area encompasses all or part of Blaine, Caddo, Canadian, Comanche, Custer, Dewey, Grady, Stephens, and Washita Counties. These digital data sets were developed by Mark F. Becker to use as input into a computer model that simulated ground-water flow in the Rush Springs aquifer (Mark F. Becker, U.S. Geological Survey, written commun., 1997). For the purposes of modeling the ground-water flow in the Rush Springs aquifer, Mark F. Becker (written commun., 1997) defined the Rush Springs aquifer to include the Rush Springs Formation, alluvial and terrace deposits along major streams, and parts of the Marlow Formations, particularly in the eastern part of the aquifer boundary area. The Permian-age Rush Springs Formation consists of highly cross-bedded sandstone with some interbedded dolomite and gypsum. The Rush Springs Formation is overlain by Quaternary-age alluvial and terrace deposits that consist of unconsolidated clay, silt, sand, and gravel. The Rush Springs Formation is underlain by the Permian-age Marlow Formation that consists of interbedded sandstones, siltstones, mudstones, gypsum-anhydrite, and dolomite beds (Mark F. Becker, written commun., 1997). The parts of the Marlow Formation that have high permeability and porosity are where the Marlow Formation is included as part of the Rush Springs aquifer. The Rush Springs aquifer underlies about 2,400 square miles of western Oklahoma and is an important source of water for irrigation, livestock, industrial, municipal, and domestic use. Irrigation wells are reported to have well yields greater than 1,000 gallons per minute (Mark F. Becker, written commun., 1997). Mark F. Becker created some of the aquifer boundaries, hydraulic conductivity, and recharge data sets by digitizing parts of previously published surficial geology

Understanding Our Gifted, Volume 2, Issues 1-6, September 1989-August 1990.

ERIC Educational Resources Information Center

Silverman, Linda, Ed.

1990-01-01

This document consists of six issues (all of volume 2) of a newsletter subtitled "Dedicated to Helping Gifted Children Reach their Full Potential". These issues deal with all aspects of parenting and educating gifted children. Major articles include: "Re-examining the Concept of Underachievement" (Joanne Rand Whitmore);…

ABCs of Being Smart: S Is for Supporting

ERIC Educational Resources Information Center

Foster, Joanne

2014-01-01

Joanne Foster's article "R We There Yet?" was first published in "Parenting for High Potential" ("PHP") in 2006, which became the springboard for the "ABCs of Being Smart" series of columns. At that time, Foster invited "PHP" readers to think about their own versions of the "ABCs of Being…

Enhancing the Breadth and Efficacy of Therapeutic Vaccines for Breast Cancer

DTIC Science & Technology

2017-01-01

research professor, 1 post-doctoral fellow, and 3 research associates. We have worked closely with our surgery, medical oncology , and pathology...MD – project PI (40% effort) Personnel 2. John Yim, MD – CoH Surgical Oncology (5% effort) 3. Joanne Mortimer, MD – CoH Medical Oncology (no

Impact: Advising Makes the Difference. Proceedings of the National Conference on Academic Advising (2nd, Memphis, Tennessee, October 8-11, 1978).

ERIC Educational Resources Information Center

Chando, Carl M., Ed.

Summaries of papers from the 1978 National Conference on Academic Advising are presented. Among the 58 papers are the following: "Faculty Advising as Faculty Development" (Howard Kramer); "Student Self-Assessment Systems and Academic Advising" (John H. Clarke, Christopher Robles, Joanne Yphantis); "Development and Utilization of a Peer Advising…

Use of capillary Western immunoassay (Wes) for quantification of dystrophin levels in skeletal muscle of healthy controls and individuals with Becker and Duchenne muscular dystrophy.

PubMed

Beekman, Chantal; Janson, Anneke A; Baghat, Aabed; van Deutekom, Judith C; Datson, Nicole A

2018-01-01

Duchenne muscular dystrophy (DMD) is a neuromuscular disease characterized by progressive weakness of the skeletal and cardiac muscles. This X-linked disorder is caused by open reading frame disrupting mutations in the DMD gene, resulting in strong reduction or complete absence of dystrophin protein. In order to use dystrophin as a supportive or even surrogate biomarker in clinical studies on investigational drugs aiming at correcting the primary cause of the disease, the ability to reliably quantify dystrophin expression in muscle biopsies of DMD patients pre- and post-treatment is essential. Here we demonstrate the application of the ProteinSimple capillary immunoassay (Wes) method, a gel- and blot-free method requiring less sample, antibody and time to run than conventional Western blot assay. We optimized dystrophin quantification by Wes using 2 different antibodies and found it to be highly sensitive, reproducible and quantitative over a large dynamic range. Using a healthy control muscle sample as a reference and α-actinin as a protein loading/muscle content control, a panel of skeletal muscle samples consisting of 31 healthy controls, 25 Becker Muscle dystrophy (BMD) and 17 DMD samples was subjected to Wes analysis. In healthy controls dystrophin levels varied 3 to 5-fold between the highest and lowest muscle samples, with the reference sample representing the average of all 31 samples. In BMD muscle samples dystrophin levels ranged from 10% to 90%, with an average of 33% of the healthy muscle average, while for the DMD samples the average dystrophin level was 1.3%, ranging from 0.7% to 7% of the healthy muscle average. In conclusion, Wes is a suitable, efficient and reliable method for quantification of dystrophin expression as a biomarker in DMD clinical drug development.

Characteristics of Japanese Patients with Becker Muscular Dystrophy and Intermediate Muscular Dystrophy in a Japanese National Registry of Muscular Dystrophy (Remudy): Heterogeneity and Clinical Variation.

PubMed

Mori-Yoshimura, Madoka; Mitsuhashi, Satomi; Nakamura, Harumasa; Komaki, Hirofumi; Goto, Kanako; Yonemoto, Naohiro; Takeuchi, Fumi; Hayashi, Yukiko K; Murata, Miho; Takahashi, Yuji; Nishino, Ichizo; Takeda, Shin'ichi; Kimura, En

2018-01-01

Obtaining an adequate number of patients to conduct a natural history study for rare diseases such as Becker muscular dystrophy (BMD) is difficult. The present study used data from Remudy, a national registry for neuromuscular diseases in Japan, to conduct a phenotypic analysis of BMD. We analyzed Remudy data of participants with dystrophinopathy. All participants who were aged 17 and older and were ambulant at age 13 were included in this study. Participants were divided into two groups: those with BMD who were ambulant at age 17, and those with intermediate muscular dystrophy (IMD) who lost ambulation by age 17. Frequent mutations were analyzed by age at ambulation, cardiopulmonary function, and genotype. For clinical comparisons, participants who were administered steroids were excluded. From July 2009 through September 2015, 192 participants had registered with Remudy. Mean participant age was 34.80±13.3 (range, 17-78) years, and 52.1% of participants were ambulant. Of the entire study population, 50.5% had cardiomyopathy and 35.9% had respiratory failure. Three participants required invasive ventilation and 30 required non-invasive ventilation. Nineteen of the 30 non-invasive ventilator users were part-time users. In total, 138 (71.9%) had BMD and 54 (28.1%) had IMD. The most frequent mutation was ex45_ex47del (36 participants). Among participants with frequent in-frame mutations, those with the ex45-49del mutation lost their ambulation earlier than those with the ex45_ex47del mutation. A total of 67 different exon deletions and duplications were identified in the study population. We clarified the clinical phenotypes of Japanese patients with BMD/IMD using data from Remudy. Our results suggest that not only IMD but also BMD are associated with risk of respiratory dysfunction.

Interpreting the rational addiction model.

PubMed

Ferguson, B S

2000-10-01

The rational addiction (RA) model of Becker and Murphy (Becker GS, Murphy KM. A theory of rational addiction. J Pol Econ 1988; 96(4): 675-700) has rapidly become one of the standard models in the literature on addictive behaviour. This paper reviews some theoretical issues surrounding its use, and indicates areas in which caution should be used in applying this model.

Moments in the Life of a Scientist

NASA Astrophysics Data System (ADS)

Rossi, Bruno

1990-08-01

Bruno Rossi has long been an influential figure in diverse areas of physics and in this volume he presents a fascinating account of his life and work as an experimental physicist. He discusses his scientific contributions, from experiments that played a major role in establishing the nature and properties of cosmic rays to those establishing the existence of a solar wind and others that laid the foundations of X-ray astronomy. Rossi provides close insight into his actual experiences as a scientist and the motivations that gave direction to his research, and he recounts the beginning of very significant stages in high energy physics and space research. He writes evocatively of the many places where he worked--of Florence, Arcetri, Padua, and Venice, of the mountains of Colorado and the deserts of New Mexico. His narrative also provides insight into the life of a Jewish family in fascist Italy. The text is accompanied by photographs taken throughout Rossi's career.

Deaf Role Models Making a Critical Difference in New Mexico

ERIC Educational Resources Information Center

Abrams, Stacy; Gallegos, Rosemary

2011-01-01

In this issue of "Odyssey," Joanne Corwin describes New Mexico's statewide partnership among several agencies for the provision of early intervention services to infants and children who are deaf or hard of hearing and their families (Effective Partnering of State Agencies to Achieve Early Hearing Detection and Intervention Benchmarks,…

2007 NCTE Presidential Address: Where Ignorant Armies Clash by Night

ERIC Educational Resources Information Center

Yatvin, Joanne

2008-01-01

This article presents the text of Joanne Yatvin's presidential address, delivered at the NCTE Annual Convention in New York City in November 2007. The title of her presidential address, "Where Ignorant Armies Clash by Night," was taken from Matthew Arnold's (1867) poem "Dover Beach." Yatvin states that the federal government…

Seeing History: Malaika Favorite's "Furious Flower Poetry Quilt" Painting and Pan-African Memory

ERIC Educational Resources Information Center

Shanahan, Maureen G.

2010-01-01

Malaika Favorite's "Furious Flower Poetry Quilt" (2004) is an acrylic painting that depicts 24 portraits of leading poets of the African Diaspora. Commissioned by Dr Joanne Gabbin, English professor and director of the Furious Flower Poetry Center at James Madison University, the painting is part of a larger programme of poetry…

The Air Land Sea Bulletin. Issue No. 2009-3, September 2009

DTIC Science & Technology

2009-09-01

mote control system at Balad Air Base, Iraq, in support of Operation IRAQI FREEDOM. (Photo by SSgt Cohen A. Young, USAF) Recently there has been a...to a UAS launch and recovery site on Forward Operating Base Hunter, Iraq recently. (Photo by 1 LT Joanne Cotton , USA) The Department of Homeland

A Team Approach to Quality Programming for Deaf and Hard of Hearing Students

ERIC Educational Resources Information Center

Gutierrez, Priscilla Shannon

2011-01-01

In this issue of "Odyssey," Joanne Corwin describes New Mexico's statewide partnership among several agencies for the provision of early intervention services to infants and children who are deaf or hard of hearing and their families (Effective Partnering of State Agencies to Achieve Early Hearing Detection and Intervention Benchmarks,…

Elastic-Plastic Behavior of Cyclotrimethylene Trinitramine Single Crystals Under Spherical Indentation: Modeling and Simulation

DTIC Science & Technology

2012-04-01

M. Winey and Y. M. Gupta, J. Appl. Phys. 107, 103505 (2010). 13R. Becker, Int. J. Plast. 20, 1983 (2004). 14B. Olinger, B. Roof, and H. H. Cady ...f011g, f021g (010), f011g, 021ð Þ 063512-8 J. D. Clayton and R. Becker J. Appl. Phys. 111, 063512 (2012) 18H. H. Cady , J. Chem. Eng. Data 17, 369

Teacher Supervision: If It Ain't Working...

ERIC Educational Resources Information Center

Rooney, Joanne

2005-01-01

When Joanne Rooney, a principal, asked 17 tenured teachers who were due for their formal supervisory visits at Pleasant Hill School in Palatine, Illinois whether her annual visits and follow-up conferences help them become better teachers," her question was met with muffled laughter. They knew that her rushed, mandatory visits and conferences…

Pewaukee School District, Wisconsin. Case Study: Measures of Academic Progress

ERIC Educational Resources Information Center

Northwest Evaluation Association, 2015

2015-01-01

For more than a decade, Pewaukee School District Superintendent JoAnn Sternke has watched her district get better and better at its mission: opening the door to each student's future. The Wisconsin district began using Measures of Academic Progress® (MAP®) computer adaptive interim assessments from Northwest Evaluation Association™ (NWEA™) in 2004…

Postcode Criminals

ERIC Educational Resources Information Center

Hiett, Sandra; Kushner, Joann

2013-01-01

Postcode Criminals was the second phase of an international participatory community arts project challenging negative stereotypes of urban youth. Concerned with the impact of zero tolerance community policing strategies in the UK and USA, artists Joann Kushner and Dread Scott developed an art-based project with a social justice agenda. To give…

ABCs of Being Smart: T Is for Tips for Working with Teachers

ERIC Educational Resources Information Center

Foster, Joanne

2015-01-01

As part of her series, "ABCs of Being Smart," Joanne Foster presents time-tested tips for parents of toddlers to teens. Categories include: traits to tap when meeting with teachers to strengthen home and school connections or resolve any issues; strategies for parents to add to their "toolbox"; and tactical measures to consider…

Developing "Argumentation" with the 4-11 Age Range

ERIC Educational Resources Information Center

Russell, Terry; McGuigan, Linda; Hamilton, Janet; Geldard, Joanne

2016-01-01

Terry Russell and Linda McGuigan of the University of Liverpool draw on their classroom research to offer their thoughts on argumentation, an aspect of "working scientifically," with contributions from two recent research collaborators: Janet Hamilton teaching 4-6 year-olds and Joanne Geldard teaching science to 10-11 year-olds. Between…

Two Tools Shaping NCDA's Place

ERIC Educational Resources Information Center

Feller, Rich, Ed.; Furbish, Dale, Ed.

2013-01-01

The authors are pleased to introduce the second pair of articles in the National Career Development Association (NCDA) Centennial Special Series celebrating the 100th anniversary of NCDA. In this issue, JoAnn Harris-Bowlsbey reviews the development and use of computer-assisted career guidance systems, and Juliette N. Lester, James Woods, and…

Handbook of Parenting. Volume 3: Being and Becoming a Parent. Second Edition.

ERIC Educational Resources Information Center

Bornstein, Marc H., Ed.

Highlighting the specific as well as common characteristics of different types of parents, this volume, the third of five volumes on parenting, deals specifically with parental status and the social conditions of parenting. The volume consists of 20 chapters as follows: (1) "Mothering" (Kathryn E. Barnard and JoAnne E. Solchany); (2)…

Hantavirus Pulmonary Syndrome in Central Bolivia: Relationships Between Reservoir Hosts, Habitats, and Viral Genotypes

DTIC Science & Technology

2005-01-01

in Brazil. J Med Virol 59: 527–535. 7. Lopez N, Padula P, Rossi C, Lazaro ME, Franze-Fernandez MT, 1996. Genetic identification of a new hantavirus...177: 529–538. 10. Toro J, Vega JD, Khan AS, Mills JN, Padula P, Terry W, Yadon Z, Valderrama R, Ellis BA, Pavletic C, Cerda R, Zaki S, Wun- Ju S, Meyer...Chile, 1997. Emerg Infect Dis 4: 687–694. 11. Padula PJ, Rossi CM, Della Valle MO, Martinez PV, Colavecchia SB, Edelstein A, Miguel SD, Rabinovich RD

Optically Based Rapid Screening Method for Proven Optimal Treatment Strategies Before Treatment Begins

DTIC Science & Technology

2015-08-01

lifetime ( t2 ) corresponds to protein- bound NADH (23). Conversely, protein-bound FAD corre- sponds to the short lifetime, whereas free FAD corresponds...single photon counting (TCSPC) electronics (SPC-150, Becker and Hickl). TCSPC uses a fast detector PMT to measure the time between a laser pulse and... Becker and Hickl). A binning of nine surrounding pixels was used. Then, the fluorescence lifetime components were computed for each pixel by deconvolving

A Randomized, Controlled Trial of Intranasal Oxytocin as an Adjunct to Behavioral Therapy for Autism Spectrum Disorder

DTIC Science & Technology

2015-10-01

Fellowship of the Alexander von Humboldt Foundation , MIT Name: Dina Hirshfeld- Becker , PhD Project Role: Independent Evaluator Researcher Identifier...e.g. ORCID ID): Nearest person month worked: 3 Contribution to Project: Dr. Hirshfeld- Becker has been an independent evaluator and has evaluated ...either intranasal oxytocin or placebo. Participants and evaluators will be blind to treatment condition. In year 1 of the study, we set up the study

The Skin That We Speak: Thoughts on Language and Culture in the Classroom.

ERIC Educational Resources Information Center

Delpit, Lisa, Ed.; Dowdy, Joanne Kilgour, Ed.

This book explores the many Englishes that are spoken in the classroom and the layers of politics, power, and identity that those different forms carry. There are 12 papers in three parts. Part 1, "Language and Identity," includes: "Ovuh Dyuh" (Joanne Kilgour Dowdy); and "Ebonics: A Case History" (Ernie Smith). Part 2, "Languages in the…

Forum: Knowledge, Action, Involvement

ERIC Educational Resources Information Center

Weinberger, JoAnn

2015-01-01

St. Clair (EJ1072357) provides a summary and lays out some of the important issues inherent in the broad strategies articulated in "Making Skills Everyone's Business: A Call to Transform Adult Learning in the United States" (MSEB) (United States Department of Education [USDoE], 2015) (see ED558793). In this commentary, JoAnn Weinberger…

Indigenous Storywork: Educating the Heart, Mind, Body, and Spirit

ERIC Educational Resources Information Center

Archibald, Jo-ann

2008-01-01

Indigenous oral narratives are an important source for, and component of, Coast Salish knowledge systems. Stories are not only to be recounted and passed down; they are also intended as tools for teaching. Jo-ann Archibald worked closely with Elders and storytellers, who shared both traditional and personal life-experience stories, in order to…

Tenure in the Sacred Grove: Issues and Strategies for Women and Minority Faculty. SUNY Series in Women in Education.

ERIC Educational Resources Information Center

Cooper, Joanne E., Ed.; Stevens, Dannelle D., Ed.

Designed to help women and minority college faculty navigate a path to tenure, this book looks at the political, scholarly, personal, and interpersonal issues. The chapters of part 1, Surveying the Landscape of the Sacred Grove, are: (1) The Journey toward Tenure (Joanne E. Cooper and Dannelle D. Stevens); (2) Case Studies: Learning from Others…

Exploring Diversity at GCSE: Making a First World War Battlefields Visit Meaningful to All Students

ERIC Educational Resources Information Center

Philpott, Joanne; Guiney, Daniel

2011-01-01

Having already reflected on ways of improving their students' understanding of historical diversity at Key Stage 3, Joanne Philpott and Daniel Guiney set themselves the challenge of extending this to post-14 students by means of fieldwork activities at First World War battlefields sites. In addition, they wanted to link the study of past diversity…

Indigenous Educational Models for Contemporary Practice: In Our Mother's Voice. Sociocultural, Political, and Historical Studies in Education.

ERIC Educational Resources Information Center

Nee-Benham, Maenette Kape'ahiokalani Padeken Ah, Ed.

This book presents a collection of papers on the rights of indigenous students to an equal education. The 15 chapters include: (1) "Gathering Together To Travel to the Source: A Vision for a Language and Culture-Based Educational Model" (Maenette Kape'ahiokalani Padeken Ah Nee-Benham and Joanne Elizabeth Cooper); (2) "Building a Child-Centered…

Caring for the Caregiver: The Use of Music and Music Therapy in Grief and Trauma

ERIC Educational Resources Information Center

Loewy, Joanne V., Ed.; Hara, Andrea Frisch, Ed.

2002-01-01

A collection of reflections on music therapy interventions provided as a part of the New York City Music Therapy Relief Project, sponsored by AMTA and the Recording Academy after September 11th, 2001. Edited by Joanne V. Loewy and Andrea Frisch Hara. Each chapter is written by a different therapist involved in the project.

Exploring Literacy. Nineteenth Yearbook: A Peer Reviewed Publication of the College Reading Association, 1997.

ERIC Educational Resources Information Center

Linek, Wayne M., Ed.; Sturtevant, Elizabeth G., Ed.

This book presents 25 essays and studies that explore the interplay of a variety of factors that provide the fuel for individual and collaborative explorations into the nature of literacy. Essays and studies in the book are "Patterns of Response: Struggling Readers Respond to a Real Book during Transactional Literature Discussion" (JoAnn Rubino…

Academic Libraries: Their Rationale and Role in American Higher Education. Contributions in Librarianship and Information Science, No. 84.

ERIC Educational Resources Information Center

McCabe, Gerald B., Ed.; Person, Ruth J., Ed.

This book is designed to educate campus administrators on the issues facing their libraries, and the role that a library should have on the campus. Chapters are: (1) "The Academic Library: Its Place and Role in the Institution" (Joanne R. Euster); (2) "What Community Colleges Need from Their Libraries" (David R. Dowell & Jack A. Scott); (3)…

Literacy Development in the First Year of Schooling.

ERIC Educational Resources Information Center

Hill, Susan; Louden, William

Drawing on the research study, "100 Children Go to School: Connections between Literacy Development in the Prior to School Period and the First Year of Schooling," conducted from 1996-1998 by a team made up of Susan Hill, Barbara Comber, William Louden, Judith Rivalland, and Jo-Anne Reid, this paper discusses the findings of the study,…

Our Visions of Possibility for Literacy

ERIC Educational Resources Information Center

O'Keefe, Tim; Reinier, Rise; Gallagher, Kevin; Morgan, Bruce; Lopez-Robertson, Julia; Santman, Donna; Wong-Kam, JoAnn; Hill, Sharon; Christensen, Linda

2006-01-01

Tim O'Keefe, Rise Reinier and Kevin Gallagher, Bruce Morgan, Julia Lopez-Robertson, Donna Santman, JoAnn Wong-Kam, Sharon Hill, and Linda Christensen provide short essays describing their personal visions of possibility about literacy and how they maintain that passion and vision. Across a range of contexts, they reflect on the ways in which their…

Where Are We?: The Place of Women in History Curricula

ERIC Educational Resources Information Center

Pearson, Joanne

2012-01-01

Joanne Pearson reflects on her experiences as a history teacher and teacher educator, considering the ways in which she has seen women represented in the history curricula of different schools in England. She makes the case that greater attention needs to be paid by history teachers to the criteria against which they make decisions about the…

History of Chandra X-Ray Observatory

NASA Image and Video Library

2002-01-23

Leon Van Speybroeck of the Harvard-Smithsonian Center for Astrophysics in Cambridge Massachusetts was awarded the 2002 Bruno Rossi Prize of the High-Energy Astrophysics Division of the American Astronomy Society. The Rossi Prize is an arnual recognition of significant contributions in high-energy astrophysics in honor of the Massachusetts Institute of Technology's late Professor Bruno Rossi, an authority on cosmic ray physics and a pioneer in the field of x-ray astronomy. Van Speybroeck, who led the effort to design and make the x-ray mirrors for NASA's premier Chandra X-Ray Observatory, was recognized for a career of stellar achievements in designing precision x-ray optics. As Telescope Scientist for Chandra, he has worked for more than 20 years with a team that includes scientists and engineers from the Harvard-Smithsonian, NASA's Marshall Space Flight Center, TRW, Inc., Huhes-Danbury (now B.F. Goodrich Aerospace), Optical Coating Laboratories, Inc., and Eastman-Kodak on all aspects of the x-ray mirror assembly that is the heart of the observatory.

Year Five of Southeast Atlantic Coastal Ocean Observing System (SEACOOS) Implementation

DTIC Science & Technology

2007-12-15

137 total]. Alvera -Azcarate, A., A. Barth, J.M. Beckers, and R.H. Weisberg, 2007. Multivariate reconstruction of missing data in sea surface...temperature, chlorophyll and wind satellite fields. Jour. Geophys. Res., 112, C03008, doi: 10.1029/2006JC003660. Alvera -Azcarate, A., A. Barth, and R.H...A., J.-M. Beckers, A. Alvera -Azcarate, and R. H. Weisberg, 2007. Filtering inertia-gravity waves from the initial conditions of the linear shallow

PARK2, a Large Common Fragile Site Gene, is Part of a Stress Response Network in Normal Cells That is Disrupted During the Development of Ovarian Cancer

DTIC Science & Technology

2007-01-01

from chromosomal regions known to contain a CFS and identified a number of other large CFS genes. This includes the Duchene Muscular Dystrophy (DMD...Callahan G, Becker NA, Phillips LA, Smith DI. Characterization of FRA6E and its potential role in autosomal recessive juvenile parkinsonism and...Department of Defense Support 1) Denison SR, Wang F, Becker NA, Schule B, Kock N, Phillips LA, Klein C, Smith DI. Alterations in the common fragile site

Proceedings of the Scientific Conference on Obscuration and Aerosol Research Held in Aberdeen Maryland on 27-30 June 1989

DTIC Science & Technology

1990-08-01

corneal structure for both normal and swollen corneas. Other problems of future interest are the understanding of the structure of scarred and dystrophied ...METHOD AND RESULTS The system of equations is solved numerically on a Cray X-MP by a finite element method with 9-node Lagrange quadrilaterals ( Becker ...Appl. Math., 42, 430. Becker , E. B., G. F. Carey, and J. T. Oden, 1981. Finite Elements: An Introduction (Vol. 1), Prentice- Hall, Englewood Cliffs, New

Estrogen Receptor Mutants/Variants in Human Breast Cancer.

DTIC Science & Technology

1995-12-01

Effect of dystrophin gene deletions on mRNA levels and processing in Duchenne and Becker dystrophies . Cell 1990, 63:1239-1248 4 Patriotis C, Makris A...the most common inherited disorder of platelets, Aspartylblucosaminuria, 2 an inherited lysosomal storage disorder, Duchenne and Becker muscular... dystrophies 3 or cancer progression.4m 5 Several estrogen receptor (ER) variant mRNAs have also been identified in human breast cancer biopsies.6,7,8, 9

Virus isolations and high population density implicate Culex antennatus (Becker) (Diptera: Culicidae) as a vector of Rift Valley Fever virus during an outbreak in the Nile Delta of Egypt.

PubMed

Hanafi, Hanafi A; Fryauff, David J; Saad, Magdi D; Soliman, Atef K; Mohareb, Emad W; Medhat, Iman; Zayed, Abdel Basset; Szumlas, Daniel E; Earhart, Kenneth C

2011-08-01

In June, 2003, Egypt's hospital-based electronic disease surveillance system began to record increased cases of acute febrile illness from governorates in the Nile Delta. In response to a request for assistance from the Egyptian Ministry of Health and the World Health Organization (WHO), the U.S. Naval Medical Research Unit No. 3 (NAMRU-3) provided assistance in identifying the cause and extent of this outbreak. Testing of human clinical samples (n=375) from nine governorates in Egypt identified 29 cases of RVF viremia that spanned the period of June to October, and a particular focus of disease in Kafr el Sheikh governorate (7.7% RVF infection rate). Veterinary samples (n=101) collected during this time in Kafr el Sheikh and screened by immunoassay for RVFV-specific IgM identified probable recent infections in cattle (10.4%) and sheep (5%). Entomologic investigations that focused in rural, rice growing villages in the Sidi Salim District of Kafr el Sheikh during August-September, 2003, collected, identified, and tested host-seeking female mosquitoes for the presence of pathogenic viruses. Three isolates of RVF virus (RVFV) were obtained from 297 tested pools of female mosquitoes and all three RVFV isolates came from Cx. antennatus (Becker). While Cx. pipiens has been considered the primary vector of RVF virus in Egypt and is often the most common man-biting species found, Cx. antennatus was the dominant species captured at the 2003 outbreak location in Kafr el Sheikh governorate. This is the first time that Cx. antennatus has been found naturally infected with RVFV in Egypt. Published by Elsevier B.V.

Comparison Of A Neutron Kinetics Parameter For A Polyethylene Moderated Highly Enriched Uranium System

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

McKenzie, IV, George Espy; Goda, Joetta Marie; Grove, Travis Justin

This paper examines the comparison of MCNP® code’s capability to calculate kinetics parameters effectively for a thermal system containing highly enriched uranium (HEU). The Rossi-α parameter was chosen for this examination because it is relatively easy to measure as well as easy to calculate using MCNP®’s kopts card. The Rossi-α also incorporates many other parameters of interest in nuclear kinetics most of which are more difficult to precisely measure. The comparison looks at two different nuclear data libraries for comparison to the experimental data. These libraries are ENDF/BVI (.66c) and ENDF/BVII (.80c).

PARK2, a Large Common Fragile Site Gene, is Part of a Stress Response Network in Normal Cells that is Disrupted During the Development of Ovarian Cancer

DTIC Science & Technology

2008-01-01

Muscular Dystrophy (DMD) gene and the immediately adjacent large 7 Il1RAPL1 gene in FRAXC, LARGE in FRA22B (which is associated with myodystrophy when...OF THIS GRANT 1) Denison SR, Wqang F, Becker NA, Schule B, Kock N, Phillips LA, Klein C, Smith DI. Alterations in the common fragile site gene...apoptotic and stress responses. Biochem Pharmacol 2003; 66: 1347-1354. 13) Denison SR, Callahan G, Becker NA, Phillips LA, Smith DI. Alterations in

Journal of Rehabilitation Research and Development. Volume 27 Number 3, Summer 1990

DTIC Science & Technology

1990-01-01

First 119. Reflex Sympathetic Dystrophy in an Amputee: Case St., Springfield, IL 62794-9265 Study. Odderson IR. Czerniecki JM, Arch Ph\\vs Med Rehabil 71...60206 Compiegne cedex. France Persons with Disabilities. Becker HA. et al.. hit J Rehabil Res 12(3):235-250. 1989. 201. Psychological Aspects of...Hearing Aid Treatment. Contact: Heather A. Becker . University of Texas at Austin. Borre S. Courtois J. hit J Rehabil Res 12(3):347-348. 1989. School of

Cardiac involvement in female Duchenne and Becker muscular dystrophy carriers in comparison to their first-degree male relatives: a comparative cardiovascular magnetic resonance study.

PubMed

Florian, Anca; Rösch, Sabine; Bietenbeck, Michael; Engelen, Markus; Stypmann, Jörg; Waltenberger, Johannes; Sechtem, Udo; Yilmaz, Ali

2016-03-01

Duchenne (DMD) and Becker (BMD) muscular dystrophies are X-linked recessive disorders associated with both skeletal myopathy and progressive cardiomyopathy in males. Female DMD/BMD carriers (DMDc/BMDc) are mostly free of skeletal muscle symptoms, but they are also prone to cardiomyopathy. The aim of the current study was to characterize the frequency, pattern, and extent of cardiomyopathy in female DMD/BMD carriers (DMDc/BMDc) in comparison to their first-degree male MD relatives. Thirty-six (age 44 ± 14 years) female MD carriers (20 DMDc and 16 BMDc) constituted the 'MD carrier group' and were prospectively enrolled. All MD carriers underwent a complete CMR study comprising cine- and late gadolinium enhancement (LGE) imaging. In 22 of these women ('female MD carrier comparison group', 7 DMD and 15 BMD), at least one first-degree male relative with a previously established diagnosis of MD underwent the same CMR protocol and was assigned to the 'male MD comparison group' (n = 24, 6 DMD and 18 BMD). In the total MD carrier group, 17 (47%) MD carriers had at least one pathological CMR finding [5 (14%) with a reduced left ventricular ejection fraction (LV-EF) and 16 (44%) with the presence of LGE]. All LGE-positive patients (n = 16) showed non-ischaemic LGE with subepicardial involvement of the LV lateral free wall being the most frequent pattern (13/16, 81%). Compared with BMDc, DMDc demonstrated more frequently a pathological CMR result (65 vs. 19%; P = 0.008)--in spite of being significantly younger (40 ± 11 vs. 50 ± 16 years, P = 0.038). In the male MD comparison group, the same LGE pattern as in female carriers was seen, but with a significantly higher prevalence of cardiac abnormalities compared with their female carrier relatives constituting the female MD comparison group (75 vs. 27%; P = 0.003). Cardiac involvement is a frequent finding in female carriers of DMD, but less frequently observed in carriers of BMD. Those DMDc and BMDc with cardiac involvement

Year Five of Southeast Atlantic Coastal Ocean Observing System (SEACOOS) Implementation

DTIC Science & Technology

2008-09-30

published or in press that acknowledge SEACOOS support (2003-2008) [137 total]. Alvera -Azcárate, A., A. Barth, J.M. Beckers, and R.H. Weisberg...C03008, doi:10.1029/2006JC003660. Alvera -Azcárate, A., A. Barth, and R.H. Weisberg, 2008. The surface circulation of the Caribbean Sea and the Gulf of...reef fishes on the continental shelf. Marine Technology Society Journal 39(2): 110-118. Barth, A., J.-M. Beckers, A. Alvera -Azcárate, and R. H

Description of Fundulus Heteroclitus Ventilatory Data and Water Quality Parameters: A Feasibility Study for Predicting Toxic Pfiesteria Piscicida and P. Piscicida-like Events in Estuarine Environments

DTIC Science & Technology

2000-06-30

Center for Environmental Health Research (USACEHR), the United States Environmental Protection Agency (USEPA), Johns Hopkins University Applied Physics...Joanne M. Burkholder and fellow North Carolina State researchers in 1988, has the ability to assume a toxic life cycle stage under appropriate...P. piscicida form. If the toxic form ofP. piscicida is present in the optimal quantity, the fish \\vill usually die within an hour. Dr. Burkholder

Three novel serum biomarkers, miR-1, miR-133a, and miR-206 for Limb-girdle muscular dystrophy, Facioscapulohumeral muscular dystrophy, and Becker muscular dystrophy.

PubMed

Matsuzaka, Yasunari; Kishi, Soichiro; Aoki, Yoshitsugu; Komaki, Hirofumi; Oya, Yasushi; Takeda, Shin-Ichi; Hashido, Kazuo

2014-11-01

Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies. We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay. Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively. Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as

Bubble Memory Module.

DTIC Science & Technology

1980-12-01

I AD-A093 642 ROCKWELL INTERNATIONAL ANAHEIM CA AUOEISSTAE-T F/S V/2 I BU13LE MEMORY MODULE. (U) DEC 80 0 0 BOHNING. F J BECKER NASI -14174...Cde under Contract NASI -14174 Dist’m/o National Aeronautics and Space Administration Scientific and Technical Information BranchA 1980 J Approvod ior...9/ BUBBLE M MORY MODULE.(U) DEC 80 0 BHNING, F J BECKER NASI -14174 NCLASSIFIED CB-569/201 NASA-CR-3380 ML22-fllfllf ll l ff mmlmmmmm.l®fmmM EEmmEI

SimCoach Evaluation: A Virtual Human Intervention to Encourage Service-Member Help-Seeking for Posttraumatic Stress Disorder and Depression

DTIC Science & Technology

2015-01-01

Sciences and director of the Trauma and Anxiety Recovery Program at Emory School of Medicine • JoAnn Difede, attending psychologist at the NewYork...page promoting herbal supplement use, among other strategies, as a remedy). The incorporation of SME expertise is consistent with best practices...of SimCoach Beta recommendations was that links to websites dis- cussing non–evidence-based strategies for addressing depression (e.g., herbal remedies

Free-lance nursing in Italy.

PubMed

Galli, E; Lindsay, J S

1993-01-01

In 1987 Registered Nurses Ernesta 'Tina' Galli and Joanne Lindsay embarked on a unique venture--a private agency* organized and run by nurses that engages free-lance nurses to fill the need for nursing assistance both at home and in public and private institutions. The response has been overwhelming. Today they have branches throughout Italy and have expanded their services to include research and primary health care. Below, their story.

High-Power Ultrasound for Disinfection of Graywater and Ballast Water: A Beaker-Scale and Pilot-Scale Investigation

DTIC Science & Technology

2006-06-01

The authors thank Denise Aylor (613) and Erick Satchell (613) for performing the cavitation erosion measurements and JoAnn Burkholder (North Carolina...20376 CODE 613 (AYLOR) 1 CODE 613 (SATCHELL) 1 COMMANDER CODE 617 (LEE, JOHN ) 1 NAVAL SURFACE WARFARE CENTER CODE 617 (BRIZZOLARA) 10 DAHLGREN...WUN-FOGLE) 10 CODE 702 (STRASBORG) 1 DEFENSE TECHNICAL INFORMATION CODE 3442 (TIC) 1 CENTER 8725 JOHN KINGMAN ROAD SUITE 0944 FORT BELVOIR VA 22060

Defense Infrastructure: Documentation Lacking to Fully Support How DOD Determined Specifications for the Landstuhl Replacement Medical Center

DTIC Science & Technology

2012-05-01

LEED system awards points for meeting a variety of standards and certifies buildings as silver, gold , or platinum. The replacement medical center’s...indicate that it may qualify for a “ gold ” certification once it has met the more stringent German design requirements. For example, the project will... Joanne Landesman assisted in the message and report development, Amie Steele assisted in developing the report’s tables and graphics, Jennifer Echard

The Results of a Laboratory Feasibility Study for the Biological Treatment of Umatilla Groundwater

DTIC Science & Technology

2012-01-01

high fructose corn syrup Kroger brand lactose Columbia River Processors, Boardman, OR cheese whey Columbia River Processors, Boardman, OR lactate...Processing Roy Dugan 541·481-3771 79588 Rippee Road 55 High Fructose Corn Syrup Malt Products Corp. Joanne McGuire 530-677-8282 #677 Blackstrap...communication with experts) tested in Run 1 were: • high - fructose corn sugar (based on promising results obtained using soft drink by-products

Fighting in the Gray Zone: A Strategy to Close the Preemption Gap

DTIC Science & Technology

2004-06-12

FIGHTING IN THE GRAY ZONE: A STRATEGY TO CLOSE THE PREEMPTION GAP Joanne M. Fish, Commander, USN Samuel J. McCraw, Lieutenant Colonel...SUBTITLE FIGHTING IN THE GRAY ZONE: A STRATEGY TO CLOSE THE PREEMPTION GAP 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6...Classical) Forcible Counterproliferation Figure 10: WMD Acquisition in the Gray Zone FORCIBLE COUNTERPROLIFERATION: CLOSING THE STRATEGY GAP The

Health behind bars: can exploring the history of prison health systems impact future policy?

PubMed

Weston, Kathryn M; McCarthy, Louella R; Meyering, Isobelle Barrett; Hampton, Stephen; Mackinnon, Tobias

2018-02-01

The value of history is, indeed, not scientific but moral … it prepares us to live more humanely in the present, and to meet rather than to foretell, the future - Carl Becker. Becker's quote reminds us of the importance of revealing and understanding historical practices in order to influence actions in the future. There are compelling reasons for uncovering this history, in particular to better inform government policy makers and health advocates, and to address the impacts of growing community expectations to 'make the punishment fit the crime'. Copyright © 2018 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

White Paper To William Becker

EPA Pesticide Factsheets

This document may be of assistance in applying the Title V air operating permit regulations. This document is part of the Title V Policy and Guidance Database available at www2.epa.gov/title-v-operating-permits/title-v-operating-permit-policy-and-guidance-document-index. Some documents in the database are a scanned or retyped version of a paper photocopy of the original. Although we have taken considerable effort to quality assure the documents, some may contain typographical errors. Contact the office that issued the document if you need a copy of the original.

Duchenne and Becker Muscular Dystrophies

MedlinePlus

... trial in boys with the disease. In another approach, MDA-supported researchers at a biotechnology company are ... respira- tory or speech therapy consultations • annual flu shots • support groups for those affected, spouses, parents or ...

Building Language Skills and Cultural Competencies in the Military: DOD’s Challenge in Today’s Educational Environment

DTIC Science & Technology

2008-11-01

cultures and to communicate directly with local populations. These skills save lives. They can save lives when the military is performing traditional ...with the Mayor of Hammam Al Alill, Iraq, October 27, 2008. USAF Photo/ Staff Sergeant JoAnn S. Makinano. THE ROADMAP VISION FOR TRANSFORMATION 31...skills. The importance of these skills may be more obvious in the irregular warfare setting, but are as important in the more traditional combat arena

Nebraska Prostate Cancer Research Program

DTIC Science & Technology

2012-05-01

Powell. (2012). Dioxin exposure enhances nuclear localization of androgen receptor. The 8th Annual National Symposium on Prostate Cancer by CCRTD...cholesterol. Mol . Cellu. Endo. 295:115-120. 2. Siegel, R., Naishadham, D., and Jemal, A. (2012). Cancer Statistics, 2012. CA Cancer J Clin 62: 10-29...Ul DIOXIN J!1XPOSURE EN CES NUCLEAR LOCALIZATION OF ANDROGEN RECEPTOR\\~f..aTayia Aaron, nd Joann Powell, Center for Cancer Research and Therapeutic

Annual Conference on Human Retrovirus Testing (7th) held in Chicago, IL on March 3-5, 1992

DTIC Science & Technology

1993-04-05

Ph.D.; Charles Sehable, M.S.; Judith Wethers , M.S.; Judith Wilber, Ph.D. This conference was supported, in part, by a firancial graot from the...Westerman Bill Roberts Judith Wethers , M.S. U Col Chester Roberts, Ph.D. Julianne Wiese Jeanette V Roman Helen Wiprud Gene F Robertson, Ph.D. JoAnn L Yee...RHgmor Thorstensson, Ph.D., National Judith Wethers , M.S., Director of Testing Bacteriological Lab Services. Retrovirology Lab.. New York Ralph

KSC-03pd0705

NASA Image and Video Library

2003-03-14

KENNEDY SPACE CENTER, Fla. - In the RLV Hangar, Mike Leinbach, Shuttle launch director, describes some of the debris to U.S. Representative Tom Feeney (second from left), who is visiting KSC to see the Columbia debris collected in the hangar. At right, from KSC, are JoAnn Morgan, director of External Relations and Business Development; Greg Katnik, technical manager, Space Shuttle Program Launch Integration Office; and John Halsema, Chief/Federal & International Liaison, Government Relations Office.

Lewis Wolpert discusses development and depression. Interview by Joanne Clough.

PubMed

Wolpert, Lewis

2004-06-01

Lewis Wolpert is Professor of Biology as Applied to Medicine in the Department of Anatomy and Developmental Biology at University College, London, UK. His research interests focus on the mechanisms that are involved in embryonic development. Lewis originally trained as a civil engineer in South Africa but in 1955 made the move to research in cellular biology at King's College, London. He was made a Fellow of the Royal Society in 1980 and awarded the CBE in 1990. Lewis was also made a Fellow of the Royal Society of Literature in 1999 and has presented science on both radio and TV for several years. He was awarded The Royal Society Michael Faraday Prize in 2000 for his contribution to the public understanding of science, most notably through his Chairmanship of the Committee for the Public Understanding of Science (COPUS; 1993-1998). He is the author of numerous books, including Malignant Sadness: The Anatomy of Depression, Principles of Development, The Unnatural Nature of Science and The Triumph of the Embryo. He also writes a regular column for The Independent.

Biotechnology

NASA Image and Video Library

2001-08-04

In August 2001, principal investigator Jeanne Becker sent human ovarian tumor cells to the International Space Station (ISS) aboard the STS-105 mission. The tumor cells were cultured in microgravity for a 14 day growth period and were analyzed for changes in the rate of cell growth and synthesis of associated proteins. In addition, they were evaluated for the expression of several proteins that are the products of oncogenes, which cause the transformation of normal cells into cancer cells. This photo, which was taken by astronaut Frank Culbertson who conducted the experiment for Dr. Becker, shows two cell culture bags containing LN1 ovarian carcinoma cell cultures.

Ovarian Tumor Cells Studied Aboard the International Space Station (ISS)

NASA Technical Reports Server (NTRS)

2001-01-01

In August 2001, principal investigator Jeanne Becker sent human ovarian tumor cells to the International Space Station (ISS) aboard the STS-105 mission. The tumor cells were cultured in microgravity for a 14 day growth period and were analyzed for changes in the rate of cell growth and synthesis of associated proteins. In addition, they were evaluated for the expression of several proteins that are the products of oncogenes, which cause the transformation of normal cells into cancer cells. This photo, which was taken by astronaut Frank Culbertson who conducted the experiment for Dr. Becker, shows two cell culture bags containing LN1 ovarian carcinoma cell cultures.

KSC-03PD-1866

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. KSC External Relations and Business Development Director JoAnn Morgan speaks to attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the groups Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing womens accomplishments.

KSC-03PD-1865

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. KSC External Relations and Business Development Director JoAnn Morgan speaks to attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the groups Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing womens accomplishments.

KSC-00pp0099

NASA Image and Video Library

2000-01-24

JoAnn Morgan, associate director for Advanced Development and Shuttle Upgrades at KSC, studies posters of space-related news stories in the mobile exhibition called "NewsCapade with Al Neuharth." The exhibit started its cross-country tour in San Francisco in April. It is a traveling version of the Newseum in Arlington, Va. Morgan was among four speakers discussing "Space, the Media and the Millennium" at a reception Jan. 24 kicking off the display at KSC

KSC00pp0099

NASA Image and Video Library

2000-01-24

JoAnn Morgan, associate director for Advanced Development and Shuttle Upgrades at KSC, studies posters of space-related news stories in the mobile exhibition called "NewsCapade with Al Neuharth." The exhibit started its cross-country tour in San Francisco in April. It is a traveling version of the Newseum in Arlington, Va. Morgan was among four speakers discussing "Space, the Media and the Millennium" at a reception Jan. 24 kicking off the display at KSC

CrossTalk: The Journal of Defense Software Engineering. Volume 18, Number 2

DTIC Science & Technology

2005-02-01

Richard J. Adams , Sergio Alvarado, Suellen Eslinger, and Joanne Tagami all with The Aerospace Corporation, and Scott A. Whitmire at ODS Software...Kiczales, G., and M. Kersten . “Show Me the Structure.” Software Develop- ment Apr. 2000. Notes 1. Please note that the M1-M11 number- ing of concepts did...VA: Integrated Computer Engineering, Inc., 2 Aug. 2000 (http://www.spmn.com). 3 Adams , Richard J., Suellen Eslinger, Karen L. Owens, and Mary A. Rich

KSC-03PD-1867

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. KSC External Relations and Business Development Director JoAnn Morgan (sixth from right) joins other attendees of The Florida Commission on the Status of Women held June 7 at the Debus Conference Facility. Morgan is a member of the groups Hall of Fame. The commission, through coordinating, researching, communicating, and encouraging legislation, is dedicated to empowering women from all walks of life in achieving their fullest potential, to eliminating barriers to that achievement, and to recognizing womens accomplishments.

KSC-2012-3111

NASA Image and Video Library

2012-05-23

CAPE CANAVERAL, Fla. – Joanne Maguire, executive vice president of Space Systems for Lockheed Martin, and Kennedy Space Center Director Bob Cabana, far right, are accompanied by Lockheed Martin and NASA personnel during a tour of Kennedy's Operations and Checkout Building. Lockheed Martin is the prime contractor to NASA for the Orion Multi-Purpose Crew Vehicle, which will be processed in the refurbished Operations and Checkout building. For more information, visit http://www.nasa.gov/exploration/systems/ground. Photo credit: NASA/Jim Grossmann

KSC-2012-3112

NASA Image and Video Library

2012-05-23

CAPE CANAVERAL, Fla. – Lockheed Martin and NASA personnel accompany Joanne Maguire, executive vice president of Space Systems for Lockheed Martin, and Kennedy Space Center Director Bob Cabana, far right, during a tour of Kennedy's Operations and Checkout Building. Lockheed Martin is the prime contractor to NASA for the Orion Multi-Purpose Crew Vehicle, which will be processed in the refurbished Operations and Checkout building. For more information, visit http://www.nasa.gov/exploration/systems/ground. Photo credit: NASA/Jim Grossmann

Applications of a Wage-Turnover Model to the Shipbuilding Industry.

DTIC Science & Technology

1980-02-08

wage differential leaving a firm indifferent between the two groups is 1 3 J2 Because older workers have a shorter work- life expectancy, their rate...discussion of the relationship between current and future wage rates over a worker’s life cycle, see Joanne Salop and Steven Salop, "Self Selection...existing wage scales. 24- F4or an optimal solution to the problem of life -cycle wage Fates from the perspective of the firm, Information is needed on

Measuring and Modeling Cosmic Ray Showers with an MBL System: An Undergraduate Project.

ERIC Educational Resources Information Center

Jackson, David P.; Welker, Matthew T.

2001-01-01

Describes a novel method for inducing and measuring cosmic ray showers using a low-cost, microcomputer-based laboratory system. Uses low counting-rate radiation monitors in the reproduction of Bruno Rossi's classic experiment. (Contains 16 references.) (Author/YDS)

Genetics Home Reference: multiple myeloma

MedlinePlus

... percent of cancers of the blood and blood-forming tissues, and between one and two percent of ... Jurado M, Landi S, Rossi AM, Lesueur F, Marques H, Dudziński M, Wątek M, Moreno V, Orciuolo E, ...

Genetics Home Reference: myotonia congenita

MedlinePlus

... Manual Consumer Version: Congenital Myopathies Orphanet: Thomsen and Becker disease Patient Support and Advocacy Resources (3 links) Muscular Dystrophy Association National Organization for Rare Disorders (NORD) Resource ...

Fullerenes, fulleranes and polycyclic aromatic hydrocarbons in the Allende meteorite

NASA Technical Reports Server (NTRS)

Becker, L.; Bunch, T. E.

1997-01-01

In this paper, we confirm our earlier observations of fullerenes (C60 and C70) in the Allende meteorite (Becker et al., 1994a, 1995). Fullerene C60 was also detected in two separate C-rich (approximately 0.5-1.0%) dark inclusions (Heymann et al., 1987) that were hand picked from the Allende sample. The amounts of C60 detected were approximately 5 and approximately 10 ppb, respectively, which is considerably less than what was detected in the Allende 15/21 sample (approximately 100 ppb; Becker et al., 1994a, 1995). This suggests that fullerenes are heterogeneously distributed in the meteorite. In addition, we present evidence for fulleranes, (C60Hx), detected in separate samples by laser desorption (reflectron) time-of-flight (TOF) mass spectrometry (LDMS). The LDMS spectra for the Allende extracts were remarkably similar to the spectra generated for the synthetic fullerane mixtures. Several fullerane products were synthesized using a Rh catalyst (Becker et al., 1993a) and separated using high-performance liquid chromatography (HPLC). Polycyclic aromatic hydrocarbons (PAHs) were also observed ppm levels) that included benzofluoranthene and corannulene, a cup-shaped molecule that has been proposed as a precursor molecule to the formation of fullerenes in the gas phase (Pope et al., 1993).

Genetics Home Reference: atelosteogenesis type 2

MedlinePlus

... 49-53. Review. Citation on PubMed or Free article on PubMed Central Rossi A, Superti-Furga A. Mutations in the diastrophic dysplasia sulfate transporter (DTDST) gene (SLC26A2): 22 novel mutations, mutation review, associated skeletal ...

Taking a position on intramembrane proteolysis.

PubMed

Lemieux, M Joanne

2018-03-30

Decades of work have contributed to our in-depth mechanistic understanding of soluble proteases, but much less is known about the catalytic mechanism of intramembrane proteolysis due to inherent difficulties in both preparing and analyzing integral membrane enzymes and transmembrane substrates. New work from Naing et al. tackles this challenge by examining the catalytic parameters of an aspartyl intramembrane protease homologous to the enzyme that cleaves amyloid precursor protein, finding that both chemistry and register contribute to specificity in substrate cleavage. © 2018 Joanne Lemieux.

Juno Jupiter Into the Unknown

NASA Image and Video Library

2016-06-23

A look into the JOI Mission; Narrated by Heidi Becker, Juno Radiation Monitoring Investigation Lead: Scott Bolton, Juno Principal Investigator; Steven Levin, Juno Project Scientist; Rick Nybakken, Juno Project Manager.

Myotonia Congenita

MedlinePlus

... increased. There are two forms of the disorder: Becker-type, which is the most common form; and Thomsen’s disease, which is ... Association National Institute of Arthritis and Musculoskeletal and ...

Identification of tick-borne pathogens in ticks feeding on humans in Turkey.

PubMed

Orkun, Ömer; Karaer, Zafer; Çakmak, Ayşe; Nalbantoğlu, Serpil

2014-08-01

The importance of tick-borne diseases is increasing all over the world, including Turkey. The tick-borne disease outbreaks reported in recent years and the abundance of tick species and the existence of suitable habitats increase the importance of studies related to the epidemiology of ticks and tick-borne pathogens in Turkey. The aim of this study was to investigate the presence of and to determine the infection rates of some tick-borne pathogens, including Babesia spp., Borrelia burgdorferi sensu lato and spotted fever group rickettsiae in the ticks removed from humans in different parts of Ankara. A total of 169 ticks belonging to the genus Haemaphysalis, Hyalomma, Ixodes and Rhipicephalus were collected by removing from humans in different parts of Ankara. Ticks were molecularly screened for Babesia spp., Borrelia burgdorferi sensu lato and spotted fever group rickettsiae by PCR and sequencing analysis. We detected 4 Babesia spp.; B. crassa, B. major, B. occultans and B. rossi, one Borrelia spp.; B. burgdorferi sensu stricto and 3 spotted fever group rickettsiae; R. aeschlimannii, R. slovaca and R. hoogstraalii in the tick specimens analyzed. This is the report showing the presence of B. rossi in a region that is out of Africa and in the host species Ha. parva. In addition, B. crassa, for which limited information is available on its distribution and vector species, and B. occultans, for which no conclusive information is available on its presence in Turkey, were identified in Ha. parva and H. marginatum, respectively. Two human pathogenic rickettsia species (R. aeschlimannii and R. slovaca) were detected with a high prevalence in ticks. Additionally, B. burgdorferi sensu stricto was detected in unusual tick species (H. marginatum, H. excavatum, Hyalomma spp. (nymph) and Ha. parva). This study investigates both the distribution of several tick-borne pathogens affecting humans and animals, and the presence of new tick-borne pathogens in Turkey. More

Eliciting and Measuring Betrayal Aversion using the BDM Mechanism.

PubMed

Quercia, Simone

2016-05-01

Betrayal aversion has been operationalized as the evidence that subjects demand a higher risk premium to take social risks compared to natural risks. This evidence has been first shown by Bohnet and Zeckhauser (2004) using an adaptation of the Becker - DeGroot - Marschak mechanism (BDM, Becker et al. (1964)). We compare their implementation of the BDM mechanism with a new version designed to facilitate subjects' comprehension. We find that, although the two versions produce different distributions of values, the size of betrayal aversion, measured as an average treatment difference between social and natural risk settings, is not different across the two versions. We further show that our implementation is preferable to use in practice as it reduces substantially subjects' mistakes and the likelihood of noisy valuations.

Characterizations of double pulsing in neutron multiplicity and coincidence counting systems

DOE PAGES

Koehler, Katrina E.; Henzl, Vladimir; Croft, Stephen; ...

2016-06-29

Passive neutron coincidence/multiplicity counters are subject to non-ideal behavior, such as double pulsing and dead time. It has been shown in the past that double-pulsing exhibits a distinct signature in a Rossi-alpha distribution, which is not readily noticed using traditional Multiplicity Shift Register analysis. But, it has been assumed that the use of a pre-delay in shift register analysis removes any effects of double pulsing. Here, we use high-fidelity simulations accompanied by experimental measurements to study the effects of double pulsing on multiplicity rates. By exploiting the information from the double pulsing signature peak observable in the Rossi-alpha distribution, themore » double pulsing fraction can be determined. Algebraic correction factors for the multiplicity rates in terms of the double pulsing fraction have been developed. We also discuss the role of these corrections across a range of scenarios.« less

Populations of Bactrocera oleae (Diptera: Tephritidae) and Its Parasitoids in Himalayan Asia

USDA-ARS?s Scientific Manuscript database

For a biological control program against olive fruit fly, Bactrocera oleae Rossi, olives were collected in the Himalayan foothills (China, Nepal, India, and Pakistan) to discover new natural enemies. Wild olives, Olea europaea ssp. cuspidata (Wall ex. G. Don), were sparsely distributed and fly-infes...

Populations of Bactrocera oleae (Diptera: Tephritidae) and Its Parasitoids in Himalayan Asia

USDA-ARS?s Scientific Manuscript database

For a biological control program against olive fruit fly, Bactrocera oleae Rossi, olives were collected in the Himalayan foothills (China, Nepal, India, and Pakistan) to discover new natural enemies. Wild olives, Olea europaea ssp. cuspidata (Wall ex. G. Don), were sparsely distributed and fly-infe...

Mobility of olive fruit fly (Diptera: Tephritidae) late third instars and teneral adults in test arenas

USDA-ARS?s Scientific Manuscript database

The mobility of olive fruit fly, Bactrocera oleae (Rossi), late third instars before pupation, teneral adults before flight, and mature adults restricted from flight was studied under mulches in greenhouse cage tests, in horizontal pipes, vertical bottles and pipes filled with sand, and by observati...

Classical biological control of the olive fruit fly, Bactrocera olea (Diptera: Tephritidae), using the exotic parasitoie, Psyttalia lounsburyi (Hymenoptera: Braconidae) in France.

USDA-ARS?s Scientific Manuscript database

The olive fruit fly, Bactrocera oleae (Rossi) (Diptera: Tephritidae) is an important pest of olives which is worldwide distributed and responsible for economic losses of approximately US$800 million per year. Since the 2000s both economical and environmental concerns have raised interested in clas...

Evaluation of Limb-Girdle Muscular Dystrophy

ClinicalTrials.gov

2014-03-06

Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

Muscular Dystrophy

MedlinePlus

... Gardner-Medwin D. Variability in clinical, genetic and protein abnormalities in manifesting carriers of Duchenne and Becker muscular dystrophy . Neuromuscul. Disord. Jan 1993;3(1):57-64. 3. Bushby KM, Appleton R, Anderson ...

KSC-03pd1204

NASA Image and Video Library

2003-04-23

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

KSC-03pd1203

NASA Image and Video Library

2003-04-23

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

KSC-03pd1205

NASA Image and Video Library

2003-04-23

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

Base Realignment and Closure: An Evolution for Harvesting Efficiencies

DTIC Science & Technology

2012-06-15

Master’s Thesis 3. DATES COVERED (From - To) 25-07-2011 to 15-06-2012 4. TITLE AND SUBTITLE Base Realignment and Closure: An Evolution for...made to c lo~e and rea l ign bases in a timely manner. ACKNOWLEDGEl\\IIENT Although one name appears on the cover of thi.s thesi ~. thi~ paper i the...wisdom to endure to the end. • M y fami ly ror your constcu1t encourugcment and support . • C’ A PT Joanne Fish. my thesi ~ advi!’>ur who provided the

KSC-03PD-1203

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

KSC-03PD-1205

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

KSC-03PD-1204

NASA Technical Reports Server (NTRS)

2003-01-01

KENNEDY SPACE CENTER, FLA. - JoAnn Morgan, director, External Relations and Business Development, speaks to the students of MESA, the New Mexico Mathematics, Engineering and Science Achievement Program. The students are visiting KSC, touring facilities and meeting with mentors. MESA students, high school seniors who hold grade-point averages of at least 3.2 and who tutor other students in math and science, have made the spring trip for the past 14 years. The MESA program has close ties to the NASA Training Project at the University of New Mexico.

Synthesis Laboratory for the U.S. Army Medical Research Institute of Infectious Diseases Selection Panel

DTIC Science & Technology

1991-01-14

McCain 27 1 M. D. Ochs 73.5 4 C Richards 203.25 11 Dr. J. M. Riordan 108.5 6 Glassware Technicians: A. D. Jackson 322 18 W. Johnson 172 10 R...Kissinger. L. W.; Von. 1. / Med. Chem., 1*4*. 68, 1220- 1225. 3. Robins, R. K; Christensen, L. F. /. Med. Chem., 1978, 21, 742 4. Branson , J. (Bristol...Myers), personal communication. 5. Branson , Joanne J.; Ghazzouli, I.; Hitchcock, M. J. M; Webb, R. R.; Martin, J. C, J. Med. Chem., 1989. 32, 1457-1463

KSC-02pd1646

NASA Image and Video Library

2002-10-21

KENNEDY SPACE CENTER, FLA. - Representatives from the Merritt Island National Wildlife Refuge (MINWR) and KSC unveil a plaque dedicating the Sendler Education Outpost, located at Dummit Cove on the Refuge. Fourth from right is Acting Deputy Director JoAnn Morgan. The outpost is a resource for environmental educational students in the Central Florida area. It is named for Karl Sendler, a space pioneer and manager under Dr. Kurt Debus, KSC's first center director. Funding for the facility was provided by the Merritt Island Wildlife Association with assistance from MINWR and Kennedy Space Center.

Eliciting and Measuring Betrayal Aversion using the BDM Mechanism*

PubMed Central

Quercia, Simone

2016-01-01

Betrayal aversion has been operationalized as the evidence that subjects demand a higher risk premium to take social risks compared to natural risks. This evidence has been first shown by Bohnet and Zeckhauser (2004) using an adaptation of the Becker – DeGroot – Marschak mechanism (BDM, Becker et al. (1964)). We compare their implementation of the BDM mechanism with a new version designed to facilitate subjects’ comprehension. We find that, although the two versions produce different distributions of values, the size of betrayal aversion, measured as an average treatment difference between social and natural risk settings, is not different across the two versions. We further show that our implementation is preferable to use in practice as it reduces substantially subjects’ mistakes and the likelihood of noisy valuations. PMID:27366658

The Multileveled Regulation of the Human Cholinesterase Genes and Their Protein Products

DTIC Science & Technology

1993-09-30

s that are involved in binding and penetration of ligands. In essence , binding affinity consists of ligand penetration in addition to its binding to...J. Cell Biol. 110, 715-719. Rotundo RL, Jasmin BJ, Lee RK, Rossi SG (1992) Compartmentalization of acetylcholinesterase mENA and protein expression

International Conference on Conducting Materials (ICoCom2010) Held in Sousse, Tunisia on November 3-7, 2010

DTIC Science & Technology

2010-11-01

Microscopy measurements on operating Pentacene Thin Film Transistor Rossi (Brazil) Organic Vertical Field Effect Transistor using DPIF as organic...Conductors and Related Quantum Matter Oral Session OSC7 12:00-12:20 12:20-12:40 Zulkifli (Malaysia) In-situ imaging of Structural Inhomogeneity and Local Jc

The Effect of Audio and Tactile Cues on Soldier Decision Making and Navigation in Complex Simulation Scenarios

DTIC Science & Technology

2008-04-01

Gray, A.; Tolentino, A.; Jagusztyn, N.; Stilson, F.; Klein, R.; Willis , T.; Rossi, M.; Redden, E.; Elliott, L. Guiding Principles for Tactile...7290 1 COMMANDANT USAADASCH ATTN ATSA CD ATTN AMSRD ARL HR ME DR HAWLEY 5800 CARTER RD FT BLISS TX 79916-3802 NO. OF COPIES

77 FR 2083 - Notice of Inventory Completion: Minnesota Indian Affairs Council, Bemidji, MN

Federal Register 2010, 2011, 2012, 2013, 2014

2012-01-13

... the north side of Height of Land Lake, site 21BK48, by Mr. William Krause during road construction and... near Ice Cracking Lake during road construction and donated to the Becker County Historical Society (HR...

Genetics Home Reference: epilepsy-aphasia spectrum

MedlinePlus

... Van Paesschen W, Caraballo R, Fejerman N, Weckhuysen S, De Jonghe P, Larsen J, Møller RS, Hjalgrim H, ... B, Kurlemann G, Kluger G, Hahn A, Haberlandt DE, Kutzer C, Sperner J, Becker F, Weber YG, ...

Moments in the Life of a Scientist

NASA Astrophysics Data System (ADS)

Rossi, Bruno

2008-07-01

List of plates; Foreword; Preface; Prehistory; 1. Arcetri (1928-32); 2. Padua, Copenhagen, Manchester; 3. Physics of elementary particles in the Age of Innocence (1939-46); 4. Los Alamos (1943-46); 5. Cosmic rays at MIT (1946- ); 6. Physics in space; Postscript; As for me … Nora Rossi; Index.

Biological control of olive fruit fly in California – release, establishment and impact of Psyttalia lounsburyi and Psyttalia humilis

USDA-ARS?s Scientific Manuscript database

The invasive olive fruit fly Bactrocera oleae (Rossi) (Diptera: Tephritidae) likely originated in sub-Saharan Africa, where the wild olive Olea europaea cuspidata L. (Wall. ex G. Don) is found and from which the domesticated olive O. europaea europaea L. was derived. Following the path of olive cult...

The Attentional Boost Effect with Verbal Materials

ERIC Educational Resources Information Center

Mulligan, Neil W.; Spataro, Pietro; Picklesimer, Milton

2014-01-01

Study stimuli presented at the same time as unrelated targets in a detection task are better remembered than stimuli presented with distractors. This attentional boost effect (ABE) has been found with pictorial (Swallow & Jiang, 2010) and more recently verbal materials (Spataro, Mulligan, & Rossi-Arnaud, 2013). The present experiments…

HOMOGENEOUS CATALYTIC OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENTS

EPA Science Inventory

Homogeneous Catalytic Oxidations of Hydrocarbons in Alternative Solvent Systems

Michael A. Gonzalez* and Thomas M. Becker, Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26 West Martin Luther King Drive, ...

HOMOGENEOUS CATALYSTS FOR THE PARTIAL-OXYGENATION OF SATURATED HYDROCARBONS WITH HYDROGEN PERIOXIDE

EPA Science Inventory

A Methodology for the Evaluation of Process Sustainability
Michael Gonzalez*, Raymond Smith and Thomas Becker

United States Environmental Protection Agency; Office of Research and Development; Sustainable Technologies Division; 26 West Martin Luther King Drive; Cincinna...

Identification of Tick-Borne Pathogens in Ticks Feeding on Humans in Turkey

PubMed Central

Orkun, Ömer; Karaer, Zafer; Çakmak, Ayşe; Nalbantoğlu, Serpil

2014-01-01

Background The importance of tick-borne diseases is increasing all over the world, including Turkey. The tick-borne disease outbreaks reported in recent years and the abundance of tick species and the existence of suitable habitats increase the importance of studies related to the epidemiology of ticks and tick-borne pathogens in Turkey. The aim of this study was to investigate the presence of and to determine the infection rates of some tick-borne pathogens, including Babesia spp., Borrelia burgdorferi sensu lato and spotted fever group rickettsiae in the ticks removed from humans in different parts of Ankara. Methodology/Principal Findings A total of 169 ticks belonging to the genus Haemaphysalis, Hyalomma, Ixodes and Rhipicephalus were collected by removing from humans in different parts of Ankara. Ticks were molecularly screened for Babesia spp., Borrelia burgdorferi sensu lato and spotted fever group rickettsiae by PCR and sequencing analysis. We detected 4 Babesia spp.; B. crassa, B. major, B. occultans and B. rossi, one Borrelia spp.; B. burgdorferi sensu stricto and 3 spotted fever group rickettsiae; R. aeschlimannii, R. slovaca and R. hoogstraalii in the tick specimens analyzed. This is the report showing the presence of B. rossi in a region that is out of Africa and in the host species Ha. parva. In addition, B. crassa, for which limited information is available on its distribution and vector species, and B. occultans, for which no conclusive information is available on its presence in Turkey, were identified in Ha. parva and H. marginatum, respectively. Two human pathogenic rickettsia species (R. aeschlimannii and R. slovaca) were detected with a high prevalence in ticks. Additionally, B. burgdorferi sensu stricto was detected in unusual tick species (H. marginatum, H. excavatum, Hyalomma spp. (nymph) and Ha. parva). Conclusions/Significance This study investigates both the distribution of several tick-borne pathogens affecting humans and animals, and

The dynamic relationships between union dissolution and women's employment: a life-history analysis of 16 countries.

PubMed

van Damme, Maike; Kalmijn, Matthijs

2014-11-01

The specialization theory from Gary Becker is often used to explain the effect of women's work on the risk of divorce. The main argument is that women with little work experience have higher economic costs to exit marriage. Using the Fertility and Family Surveys, we test for 16 countries to what extent women's employment increases the risk of separation. We also more directly examine the role of economic exit costs in separation by investigating the effect of separated women's work history during the union on women's post-separation employment. The results imply that Becker was right to some extent, especially in contexts with little female employment support. However, in settings where women's employment opportunities are more ample, sociological or psychological theories have probably more explanatory power to explain the causes and consequences of union dissolution. Copyright © 2014 Elsevier Inc. All rights reserved.

GREENING OF OXIDATION CATALYSIS THROUGH IMPROVED CATALYST AND PROCESS DESIGN

EPA Science Inventory

Greening of Oxidation Catalysis Through Improved Catalysts and Process Design
Michael A. Gonzalez*, Thomas Becker, and Raymond Smith

United State Environmental Protection Agency, Office of Research and Development, National Risk Management Research Laboratory, 26 W...

A METHODOLOGY FOR THE EVALUATION OF PROCESS SUSTAINABILITY

EPA Science Inventory

The twelve principles of green chemistry (Anastas and Warner, 1998) provide a foundation and pathway which allows researchers to incorporate greenness into existing reactions or when developing new technologies. Research from our laboratory (Gonzalez and Becker, 2002) has adopted...

GREEN CATALYZED OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENT SYSTEMS GENERATED BY PARIS II

EPA Science Inventory

Green Catalyzed Oxidation of Hydrocarbons in Alternative Solvent Systems Generated by PARIS II

Michael A. Gonzalez*, Thomas M. Becker, and Paul F. Harten; Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26...

GREEN CATALYZED OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENT SYSTEMS GENERATED BY PARIS II DECHEMA; GREEN SOLVENTS FOR CATALYSIS - ENVIRONMENTALLY BENIGN REACTION MEDIA

EPA Science Inventory

Green catalyzed oxidation of hydrocarbons in alternative solvent systems generated by PARIS II
Thomas M. Becker, Michael A. Gonzalez, Paul F. Harten; Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26 West Mar...

OXIDATION OF CYCLOHEXANE WITH AIR CATALYZED BY A STERICALLY HINDERED IRON (II) COMPLEX

EPA Science Inventory

Oxidation of Cyclohexane with Air Catalyzed by a Sterically Hindered Iron(II) Complex.


Thomas M. Becker, Michael A. Gonzalez*

United States Environmental Protection Agency; National Risk Management Research Laboratory; Sustainable Technology Division; Clean Pr...

KSC-02pd0974

NASA Image and Video Library

2002-06-13

KENNEDY SPACE CENTER, FLA. -- The 2002 Florida Press Association and Florida Society of Newspaper Editors Convention offers a panel on space. At the podium is Bob Stover, managing editor, Florida Today. Panel participants enjoying a laugh are (left to right) Craig Covault, senior editor, Aviation Week; Howard Benedict, retired AP reporter; JoAnn Morgan, director, External Relations and Business Development, Kennedy Space Center; Marcia Dunn, AP reporter. The convention was held at the Debus Center, KSC Visitors Complex. Also speaking at the convention were Center Director Roy Bridges and NASA Associate Deputy Administrator Dr. Daniel Mulville

ROLE OF MONOCYTES AND EOSINOPHILS IN RESPIRATORY SYNCTIAL VIRUS (RSV) INFECTION

EPA Science Inventory

Role of Monocytes and Eosinophils
in Respiratory Syncytial Virus (RSV) Infection

Joleen M. Soukup and Susanne Becker

US Environmental Protection Agency, National Health and Environmental
Effects Research Laboratory, Research Triangle Park, NC 27711;
...

LANTHANIDE ENHANCE LUMINESCENCE (LEL) WITH ONE AND TWO PHOTON EXCITATION OF QUANTUM DYES LANTHANIDE (III) - MACROCYCLES

EPA Science Inventory

Title: Lanthanide Enhance Luminescence (LEL) with one and two photon excitation of Quantum Dyes? Lanthanide(III)-Macrocycles
Principal Author:
Robert C. Leif, Newport Instruments
Secondary Authors:
Margie C. Becker, Phoenix Flow Systems
Al Bromm, Virginia Commonw...

Derivation of Human Induced Pluripotent Stem (iPS) Cells to Heritable Cardiac Arrhythmias

ClinicalTrials.gov

2017-08-10

Inherited Cardiac Arrythmias; Long QT Syndrome (LQTS); Brugada Syndrome (BrS); Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT); Early Repolarization Syndrome (ERS); Arrhythmogenic Cardiomyopathy (AC, ARVD/C); Hypertrophic Cardiomyopathy (HCM); Dilated Cardiomyopathy (DCM); Muscular Dystrophies (Duchenne, Becker, Myotonic Dystrophy); Normal Control Subjects

HOMOGENEOUS AIR OXIDATION OF HYDROCARBONS UTILIZING MN AND CO CATALYSTS

EPA Science Inventory

Homogeneous Air Oxidation of Hydrocarbons Utilizing Mn and Co Catalysts

Thomas M. Becker and Michael A. Gonzalez*, Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26 West Martin Luther King Drive, Mail Sto...

Using Films to Learn about the Nature of Cross-Cultural Stereotypes in Intercultural Business Communication Courses

ERIC Educational Resources Information Center

Cardon, Peter W.

2010-01-01

Instructors of intercultural business communication courses inevitably face the challenge of providing cross-cultural experiences in the classroom, and students are eager to have real exposure to other cultures. One way of simulating the feel of entering another culture is through films. As Mallinger and Rossy (2003) state, films are a "uniquely…

A Technology Integration Education (TIE) Model: Millennial Preservice Teachers' Motivations about Technological, Pedagogical, and Content Knowledge (TPACK) Competencies

ERIC Educational Resources Information Center

Holland, Denise D.; Piper, Randy T.

2014-01-01

Nobel laureates Schultz (1971) and Becker (1964, 1993) reinvigorated the analysis of education investments. Human capital investments that improve cognitive skills for elementary and secondary students have important economic implications. An interdisciplinary, 12-construct technology integration education (TIE) model was developed. The sample…

NITROTYROSINE ATTENUATES RSV-INDUCED INFLAMMATION IN AIRWAY EPITHELIAL CELLS

EPA Science Inventory

Nitrotyrosine attenuates RSV-induced inflammation in airway epithelial cells. Joleen Soukup, Zuowei Li, Susanne Becker and Yuh-Chin Huang. NHEERL, ORD, USEPA, RTP, North Carolina, CEMALB, University of North Carolina, Chapel Hill, North Carolina

Nitrotyrosine (NO2Tyr) is a...

ROLE OF MONOCYTES IN RESPIRATORY SYNCTIAL VIRUS (RSV) INFECTION.

EPA Science Inventory

ROLE OF MONOCYTES IN RESPIRATORY SYNCYTIAL VIRUS (RSV) INFECTION.
Joleen M. Soukup and Susanne Becker, National Health and Environmental Effects Research
Laboratory, US EPA, Research Traingle Park, NC USA.

RSV infection in airway epithelial cells (EC) results i...

The Use of Physarum for Testing of Toxicity/Mutagenicity

DTIC Science & Technology

1984-04-19

grade and sup- pliers were as follows: ethanol, U.S. Industrial Co.; hydrazine dihydrochloride Fisher Chemical Co.; hydrocarbons, Alltech Co. and Theta...procedure had its own particular advantages and limitations. The microplasmodial growth inhibition system (Becker et al., 1963) was convenient because it

Shedding a New Light on the Universe: An Information and Activity Booklet. Grades 9-12.

ERIC Educational Resources Information Center

Masetti, Maggie

This activity booklet is divided into two parts. Part One presents basic information about the electromagnetic spectrum and multiwavelength astronomy with an emphasis on X-ray astronomy. Part Two describes X-ray detectors at a more advanced level. An introduction to the Rossi X-ray Timing Explorer (RXTE) and its contributions to science is…

Monitoring X-Ray Emission from X-Ray Bursters

NASA Technical Reports Server (NTRS)

Halpern, Jules P.; Kaaret, Philip

1999-01-01

The scientific goal of this project was to monitor a selected sample of x-ray bursters using data from the All-Sky Monitor (ASM) on the Rossi X-Ray Timing Explorer together with data from the Burst and Transient Source Experiment (BATSE) on the Compton Gamma-Ray Observatory to study the long-term temporal evolution of these sources in the x-ray and hard x-ray bands. The project was closely related to "Long-Term Hard X-Ray Monitoring of X-Ray Bursters", NASA project NAG5-3891, and and "Hard x-ray emission of x-ray bursters", NASA project NAG5-4633, and shares publications in common with both of these. The project involved preparation of software for use in monitoring and then the actual monitoring itself. These efforts have lead to results directly from the ASM data and also from Target of Opportunity Observations (TOO) made with the Rossi X-Ray Timing Explorer based on detection of transient hard x-ray outbursts with the ASM and BATSE.

The pornographic anatomy book? The curious tale of the Anatomical Basis of Medical Practice.

PubMed

Halperin, Edward C

2009-02-01

Studying the history of medical education helps teach us that medicine is a social activity that occurs in the context of social mores and customs. In 1971, a major new anatomy textbook aimed at first-year medical students was published. The Anatomical Basis of Medical Practice, written by Professors R. Frederick Becker, James S. W. Wilson, and John A. Gehweiler, emphasized surface anatomy, embryology, and radiographic anatomy. At multiple places in the text, the authors used sexually suggestive and "cheeky" comments about women. A small fraction of the illustrations were stylized, posed female nude photographs purchased from California photographer Peter Gowland. These photographs, of a type typically seen in Playboy centerfolds or "pin-up girl" calendars, produced a firestorm of controversy. The book was criticized in the press and in reviews in scholarly journals, and a boycott was organized by the Association of Women in Science. The publisher received negative feedback from consumers, and the book was withdrawn from the market. The book is now a minor collector's item. Professors Becker and Wilson vigorously responded. They laid blame for the debacle on the publisher and also claimed they were the victims of a witch hunt by feminists. The Anatomical Basis of Medical Practice appeared as the women's movement became part of the American popular consciousness. It was also an era in which the public began to grapple with how to define pornography. Professor Becker and his coauthors thought that they were writing a witty, engaging, and funny book. Their detractors thought the book denigrated women.

A Survey of Economic Models of Criminal Behavior

DTIC Science & Technology

1987-10-01

Economic Research, 1-54. Bentham , Jeremy (1931). Theory of Legislation. New York: Harcourt Brace. Block, Michael K. and J. M. Heineke (1975). A Labor...criminology in the eighteenth and nineteenth centuries, Beccaria and Bentham , explicitly applied an economic framework. Becker sees his work as re

EFFECTS OF DIESEL EXHAUST PARTICLES ON HUMAN ALVEOLAR MACROPHAGE RESPONSIVENESS TO LIPOPOLYSACCHARIDE

EPA Science Inventory

Effects of diesel exhaust particles on human alveolar macrophage responsiveness to lipopolysaccharide
S. Mundandhara1 , S. Becker2 and M. Madden2, 1UNC Center for Environmental Medicine, Asthma, and Lung Biology, 2US EPA, NHEERL, HSD, Chapel Hill, NC, US

Epidemiological...

Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin-glycoprotein complex assembly in pig skeletal muscle

USDA-ARS?s Scientific Manuscript database

Duchenne muscular dystrophy (DMD) is caused by a dystrophin deficiency while Becker muscular dystrophy (BMD) is caused by a dystrophin insufficiency or expression of a partially functional protein product. Both of these dystrophinopathies are most commonly studied using the mdx mouse and a golden r...

Global Education.

ERIC Educational Resources Information Center

Longstreet, Wilma S., Ed.

1988-01-01

This issue contains an introduction ("The Promise and Perplexity of Globalism," by W. Longstreet) and seven articles dedicated to exploring the meaning of global education for today's schools. "Global Education: An Overview" (J. Becker) develops possible definitions, identifies objectives and skills, and addresses questions and…

Methylene Blue Enteric Mapping for Intraoperative Localization in Obscure Small Bowel Hemorrhage: Report of a New Technique and Literature Review

DTIC Science & Technology

2012-07-12

Becker GJ, Park HM, O’Connor KW, Tarver RD, Scott JA, Jackson VP, Lappas JC, Broadie TA, Holden RW. Portal hypertension due to jejunal vascular...controlled hypertension and hyperlipidemia, a pri- or of laparoscopic cholecystectomy, and open small bowel resection. The patient reported melena and

National Rehabilitation Hospital Assistive Technology Research Center

DTIC Science & Technology

1995-10-01

methods of assembly by orthotists. Because of this, efforts have been made to overcome these challenges but have proved fruitless thus far. Becker ...people including those with muscular dystrophy , multiple sclerosis, brachial plexus injuries, and those who work in jobs requiring them to hold their

Discrimination against Facially Stigmatized Applicants in Interviews: An Eye-Tracking and Face-to-Face Investigation

ERIC Educational Resources Information Center

Madera, Juan M.; Hebl, Michelle R.

2012-01-01

Drawing from theory and research on perceived stigma (Pryor, Reeder, Yeadon, & Hesson-McInnis, 2004), attentional processes (Rinck & Becker, 2006), working memory (Baddeley & Hitch, 1974), and regulatory resources (Muraven & Baumeister, 2000), the authors examined discrimination against facially stigmatized applicants and the processes involved.…

Special Section: A Debate on Research Techniques in Economic Education

ERIC Educational Resources Information Center

Dawson, George G.; And Others

1976-01-01

Dawson introduces three articles which debate merits of research techniques in undergraduate economic education. William E. Becker criticizes John C. Soper's models, multicollinearity argument, and student incentives in a research project; Soper replies; Robert Highsmith critically analyzes strengths and weaknesses of each argument. (AV)

What Would Pete Say?

ERIC Educational Resources Information Center

Berk, Richard A.

2007-01-01

Peter Rossi's reputation was built on a pragmatic and careful approach to research practice. He cared most about facts: getting the facts right, and then communicating those facts in an earthy, accessible, and witty manner. At the same time, Pete had a brash interpersonal style and a taste for taking on some of the most controversial policy…

Examining the Effects of School-Provided E-Readers on Middle School Students' Reading Ability

ERIC Educational Resources Information Center

Brown, H. Quincy

2016-01-01

Nationwide, the increasing popularity of e-books is undeniable; sales of e-books increased an astounding 4,456% over a 5-year period. Researchers, Miranda, Johnson, and Rossi-Williams, determined that e-readers have a positive impact on students' desire to read. This study attempted to determine if the use of institution issued e-readers would…

Ebola Virus Genome Plasticity as a Marker of Its Passaging History: A Comparison of In Vitro Passaging to Non-Human Primate Infection

DTIC Science & Technology

2012-11-28

Simulation Sciences Branch, United States Army Research Laboratory , Aberdeen Proving Ground, Maryland, United States of America Abstract To identify...culture and may support filovirus stock standardization for medical countermeasure development. Citation: Kugelman JR, Lee MS, Rossi CA, McCarthy SE...support filovirus stock standardization for medical countermeasure development. 15. SUBJECT TERMS 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF

Our Gifted: Our Future. Proceedings of the Annual Northern Virginia Conference on Gifted/Talented Education (5th, Alexandria, VA, March 7-8, 1980).

ERIC Educational Resources Information Center

Orloff, Jeffrey H., Ed.

The document contains 13 selected papers from a conference on working with gifted students. Titles and authors include the following: "A Metacurriculum for the Future" (B. Hubbard); "Building a Curriculum to Train Leadership Abilities" (L. Addison); "Gifted Boys, Gifted Girls--What's the Difference" (B. Becker);…

"Build Your Social Confidence": A Social Anxiety Group for College Students

ERIC Educational Resources Information Center

Damer, Diana E.; Latimer, Kelsey M.; Porter, Sarah H.

2010-01-01

Social anxiety, a common concern among college students, carries significant negative consequences. Group therapy is an efficient and cost-effective way to provide treatment, and cognitive-behavioral group therapy (CBGT; Heimberg & Becker, 2002) is the most widely researched and empirically supported treatment for persons with social anxiety…

Body Mass Index, Nutrient Intakes, Health Behaviours and Nutrition Knowledge: A Quantile Regression Application in Taiwan

ERIC Educational Resources Information Center

Chen, Shih-Neng; Tseng, Jauling

2010-01-01

Objective: To assess various marginal effects of nutrient intakes, health behaviours and nutrition knowledge on the entire distribution of body mass index (BMI) across individuals. Design: Quantitative and distributional study. Setting: Taiwan. Methods: This study applies Becker's (1965) model of health production to construct an individual's BMI…

THE EFFECT OF SIZE FRACTIONED PARTICULATE MATTER ON HUMAN AIRWAY EPITHELIAL CELLS IN VITRO

EPA Science Inventory

THE EFFECT OF SIZE FRACTIONATED PARTICULATE MATTER ON HUMAN AIRWAY EPITHELIAL CELLS IN VITRO. LA Dailey1, C Sioutas2, JM Soukup1, S Becker1, RB Devlin1. 1National Health & Environmental Effects Research Laboratory, USEPA, RTP, NC,USA; 2USC, Civil & Environmental Engineering, LA, ...

A Critique of the Controversy about the Stability of Consumers' Tastes.

ERIC Educational Resources Information Center

Daniel, Coldwell, III

1988-01-01

Examines the role of the stability of consumer tastes in descriptive theory. Summarizes the traditional approach to the derivation of the consumer's preference structure, considers ways in which the conventional theory has been extended, presents the Stigler-Becker theory of consumer choice, and evaluates both approaches. (GEA)

Canadian Association for the Study of Adult Education. Proceedings of the Annual Conference (10th, Kingston, Ontario, Canada, June 1991).

ERIC Educational Resources Information Center

Baskett, H. K., Ed.

Among 8 French and 36 English papers are the following: "Confronting the Self in Research" (Baskett); "Learning Processes as They Occur in Groups" (Becker, Hill); "La pensee critique a-t-elle un sexe?" (Bedard, Ouellette); "The Effect of Literacy on Income and Duration of Employment" (Blunt); "Graduate…

Heterotopic Ossification Following Combat-Related Trauma

DTIC Science & Technology

2010-01-01

Nylen E, Brown TS, Rose MW, Stojadinovic A, Becker KL, McGuigan FX. Correlation of procalcitonin and cytokine expression with dehiscence of wartime...macular dystrophies . Arch Ophthalmol. 2003;121:967-72. 83. Schulmerich MV, Cole JH, Kreider JM, Esmonde-White F, Dooley KA, Goldstein SA, Morris MD

Awareness Programs and Change in Taste-Based Caste Prejudice

PubMed Central

Banerjee, Ritwik; Datta Gupta, Nabanita

2015-01-01

Becker's theory of taste-based discrimination predicts that relative employment of the discriminated social group will improve if there is a decrease in the level of prejudice for the marginally discriminating employer. In this paper we experimentally test this prediction offered by Garry Becker in his seminal work on taste based discrimination, in the context of caste in India, with management students (potential employers in the near future) as subjects. First, we measure caste prejudice and show that awareness through a TV social program reduces implicit prejudice against the lower caste and the reduction is sustained over time. Second, we find that the treatment reduces the prejudice levels of those in the left tail of the prejudice distribution - the group which can potentially affect real outcomes as predicted by the theory. And finally, a larger share of the treatment group subjects exhibit favorable opinion about reservation in jobs for the lower caste. PMID:25902290

Numerical design of an EBIS collector to optimize electron collection and ion extraction

NASA Astrophysics Data System (ADS)

Dietrich, Jürgen

1990-12-01

For the Frankfurt EBIS (R. Becker et al., Nucl. Instr. and Meth. B24/25 (1987) 838, ref. [1]), a new collector was designed using the relativistic electron optics program EGUN (W.B. Herrmannsfeldt, SLAC-331 (1988), ref. [2]) and the magnetic field program INTMAG (R. Becker, Nucl. Instr. and Meth. B42 (1989) 303, ref. [3]). To model the fringing field of the main solenoid, a bucking coil and a cylindrical shim is provided. The current of the bucking coil and the position and shape of the shim are optimized with INTMAG for minimum fringing field to allow expansion of the electron beam by its space charge. The magnetic field data output from INTMAG is directly used as input for EGUN to calculate the electron and ion trajectories. The initial conditions for the trajectories were computed from the paraxial ray equation. Different operation modes of the collector are investigated including the behaviour of secondary electrons.

Awareness programs and change in taste-based caste prejudice.

PubMed

Banerjee, Ritwik; Datta Gupta, Nabanita

2015-01-01

Becker's theory of taste-based discrimination predicts that relative employment of the discriminated social group will improve if there is a decrease in the level of prejudice for the marginally discriminating employer. In this paper we experimentally test this prediction offered by Garry Becker in his seminal work on taste based discrimination, in the context of caste in India, with management students (potential employers in the near future) as subjects. First, we measure caste prejudice and show that awareness through a TV social program reduces implicit prejudice against the lower caste and the reduction is sustained over time. Second, we find that the treatment reduces the prejudice levels of those in the left tail of the prejudice distribution--the group which can potentially affect real outcomes as predicted by the theory. And finally, a larger share of the treatment group subjects exhibit favorable opinion about reservation in jobs for the lower caste.

KSC-02pd0119

NASA Image and Video Library

2002-02-11

KENNEDY SPACE CENTER, FLA. -- Center Director Roy Bridges Jr. (center) cuts the ribbon for the opening of KSC Direct, the new Web-Broadcast Studio at KSC. Joining him are (left to right) Dennis Armstrong, Web Multimedia manager; JoAnn H. Morgan, director of External Relations and Business Development; Bridges; Vanessa Stromer, Information Technology Division, Spaceport Services; and Brian Chase, district director for Congressman Dave Weldon, who was unable to attend the ceremony. Located in the News Center on the Press Mound at KSC, the Web Broadcast Studio provides video clips of launches, landings and other KSC events in a real-time environment, called KSC Direct, through KSC's Web pages

GRACE under fire.

PubMed

Stewart, Sheena

2016-01-01

On May 1, 2016, a wildfire broke out south of Fort McMurray, Alberta. Although fires aren't uncommon at this time of year in northern Alberta, a dry winter followed by an even drier spring had turned the countryside around the city into tinder. By May 3, whipped on by high winds and 32° heat, the wildfire grew out of control, forcing a mandatory evacuation of almost 90,000 people in the city and surrounding communities. It also necessitated an emergency evacuation of the patients at the Northern Lights Regional Health Centre, where registered nurse JoAnn Cluney was on shift in the emergency department.

Haberman Star Teacher Interview as a Predictor of Success in Urban Teacher Preparation

ERIC Educational Resources Information Center

Waddell, Jennifer H.; Marszalek, Jacob M.

2018-01-01

In urban schools, along with skills for effective teaching, successful teachers must also possess values and belief systems conducive to teaching effectively in diverse settings (Becker, Kennedy, & Hundersmarck, 2003; Haberman, 2008; Metzgar & Wu, 2008). As demonstrated in CAEP standard 3, there is a critical need for EPPs to admit…

Factors of the Earning Functions and Their Influence on the Intellectual Capital of an Organization

ERIC Educational Resources Information Center

Ileanu, Bogdan Vasile; Tanasoiu, Ovidiu Emil

2008-01-01

This paper tries to consider some earning function as "start point" for the construction of indicators for intellectual capital measure. The analyze combines concepts from Mincer's and Becker theories and intellectual capital definitions currently in use. The correlation, significance and relation between elements are shown using three econometric…

Sperm-mediated transgenesis in chicken using a PiggyBac transposon system

USDA-ARS?s Scientific Manuscript database

Sperm-mediated transgenesis in chicken using a PiggyBac transposon system Emmanuel Quansah1,2, Julie Long2, David Donovan2, Stephen Becker2, Bhanu Telugu2, Juli Frey2, Nigel Urwin1 1,Charles Sturt University, Graham Center of Agricultural Innovation, Wagga Wagga. Australia and 2Beltsville Agricultu...

The AMATYC Review. Volume 13, 1991-1992.

ERIC Educational Resources Information Center

Cohen, Don, Ed.

1992-01-01

This document consists of the two numbers of "The AMATYC Review" issued during publication year 1991-1992. The following articles are featured: (1) "Educational Reflections" (D. A. Crocker); (2) "Mathematics: An International View" (I. Malyshev, J. R. Becker, editors); (3) "Pandora's Rectangular Parallelepiped" (L. R. Tanner, editor); (4) "The…

Approaches to Cyber Intrusion Response. A Legal Foundations Study. Report 12 of 12

DTIC Science & Technology

1997-01-01

Computers: Controlling Behavior in Cyberspace through a Contract Law Paradigm, 35 Jurimetrics J. 1, 8-9 (1994). 5 Ingrid Becker, Cybercrime: Cops Can’t...Computers: Controlling Behavior in Cyberspace through a Contract Law Paradigm, 35 JURIMETRICS J. 1-15 (1994). 9 • Pro: This approach allows the victim

Is Young Children's Passive Syntax Semantically Constrained? Evidence from Syntactic Priming

ERIC Educational Resources Information Center

Messenger, Katherine; Branigan, Holly P.; McLean, Janet F.; Sorace, Antonella

2012-01-01

Previous research suggests that English-speaking children comprehend agent-patient verb passives earlier than experiencer-theme verb passives (Maratsos, Fox, Becker, & Chalkley, 1985). We report three experiments examining whether such effects reflect delayed acquisition of the passive syntax or instead are an artifact of the experimental task,…

Role of PY Motif Containing Protein, WBP-2 in ER, PR Signaling and Breast Tumorigenesis

DTIC Science & Technology

2009-09-01

WW-domains and PPXY motif have been implicated in many diseases, including muscular dystrophy , Liddle’s syndrome, Alzheimer’s, Huntington disease...ED, Becker M, Qiu Y, Johnson TA, Elbi C, Fletcher TM, John S, Hager GL 2004 Subnuclear trafficking and gene targeting by steroid re- ceptors. Ann NY

Meaning of Muscular Dystrophy

MedlinePlus

... is very similar to Duchenne, except kids with Becker MD may not have problems until much later, when they're teenagers or adults. It takes a long time for their muscles to become weak. How Does a Kid Get Muscular Dystrophy? MD is not contagious (say: con-TAY-juss), ...

Role of the PY Motif Containing Protein, WBP-2 in ER, PR Signaling and Breast Tumorigenesis

DTIC Science & Technology

2007-03-01

implicated in many diseases, including muscular dystrophy , Liddle’s syndrome, Alzheimer’s, Huntington disease and Cancers [16, 31, 35, 75]. Many...of nuclear receptor movement and tran- scription. Biochim Biophys Acta 1677:46–51 5. Nagaich AK, Rayasam GV, Martinez ED, Becker M, Qiu Y, Johnson TA

ARE MACROPHAGES ACTIVATED AND INDUCE PULMONARY INJURY BY INTRACELLULARLY BIOAVAILABLE IRON?

EPA Science Inventory

ARE MACROPHAGES ACTIVATED AND INDUCE PULMONARY INJURY BY INTRACELLULARLY BIOAVAILABLE IRON? UP Kodavanti1, MCJ Schladweiler1, S Becker2, DL Costa1, P Mayer3, A Ziesenis3, WG Kreyling3, 1ETD, 2HSDivision, NHEERL, USEPA, Research Triangle Park, NC, USA, and 3GSF, Inhalation Biology...

Attraction of Mosquitoes to Diethyl Methylbenzamide and Ethyl Hexanediol

DTIC Science & Technology

1990-09-01

tant to the biting midges Culicoides pulicaris that 8% ethyl acetate was attractant to the Linn. and Culicoides puncticoUis Becker (Cera- vinegar fly...Drosoph- finding by mosquitoes (Diptera: Culcidae): a review. ila melanogaster Meigen to the products of ferment - Bull. Entomol. Res. 70:525-532. ing banana

The Perceptions of Elementary STEM Schools in Missouri

ERIC Educational Resources Information Center

Alumbaugh, Kelli Michelle

2015-01-01

Science, technology, engineering, and mathematics education, or STEM, is an area that is currently growing in popularity with educators (Becker & Park, 2011). A qualitative study consisting of interviews was conducted and data were gathered from three leaders in professional STEM organizations, four principals from elementary STEM schools, and…

Prompt Follow Up of Flaring/Transient Fermi LAT Galactic Plane Sources

DTIC Science & Technology

2016-08-20

resolution imaging. Students supported through this effort UNM graduate students Ken Obenberger and Karishma Bansal were partially supported by this...Karishma Bansal to the 2016 AAS meeting in Orlando, Florida where she presented a poster. References Becker, W . et al. 1994, ApJS, 91, 347 Hartman, R.C

Impact of Technology Policy in the Higher Education Classroom: Emerging Trends

ERIC Educational Resources Information Center

Roberge, Ginette D.; Gagnon, Lissa L.

2014-01-01

With the constant evolution of technology, there has been a growing use of electronic devices in postsecondary classrooms, by students and educators, over the last several decades. A recent publication by Becker (2013) highlights potential issues with students utilizing a variety of electronic devices in higher education and the implications for…

An Evaluation of the Effectiveness of Integrated Learning Systems on Urban Middle School Student Achievement

ERIC Educational Resources Information Center

Williams, Yamilette

2012-01-01

Many school districts have chosen to invest their federal funds in computer-based integrated learning systems that focus on literacy to increase high-stakes test scores and academic gains (Becker, 1994). Buly and Velencia (2002) supported the belief that a student's reading ability can improve substantially when instruction is integrated with…

Anti- and Pro-Immigrant Entrepreneurs Labeling Theory Revisited

ERIC Educational Resources Information Center

Wilson, Tamar Diana

2009-01-01

Almost forty years ago, noted immigration scholar Jorge A. Bustamante published an article in the "American Journal of Sociology" applying Howard Becker's labeling theory to the phenomenon of deviantizing and stigmatizing the undocumented. While immigration laws and some of the players involved have changed since his article was published,…

The Effects of Split-Attention and Redundancy on Cognitive Load When Learning Cognitive and Psychomotor Tasks

ERIC Educational Resources Information Center

Pociask, Fredrick D.; Morrison, Gary

2004-01-01

Human working memory can be defined as a component system responsible for the temporary storage and manipulation of information related to higher level cognitive behaviors, such as understanding and reasoning (Baddeley, 1992; Becker & Morris, 1999). Working memory, while able to manage a complex array of cognitive activities, presents with an…

Side-Bet Theory and the Three-Component Model of Organizational Commitment

ERIC Educational Resources Information Center

Powell, Deborah M.; Meyer, John P.

2004-01-01

We tested Becker's (1960) side-bet conceptualization of commitment within the context of Meyer and Allen's (1991) three-component model of organizational commitment. Employees (N=202) from various organizations completed a survey including measures of (a) seven categories of side bets (b) affective, normative, and continuance commitment, and (c)…

America's Opinion Leader Historians on Behalf of Success.

ERIC Educational Resources Information Center

Carpenter, Ronald H.

1983-01-01

Examines the rhetorical role of several twentieth century historians who were opinion leaders on behalf of the American way of achieving success: by emulating the earlier qualities of our frontier and founding fathers. Discusses the role of Frederick Turner Jackson, Charles A. Beard, Carl Becker, Allan Nevins, and others. (PD)

Qualitative Methods Reclaimed.

DTIC Science & Technology

1983-07-01

Contradictions in Technical Careers," Technology Review, Nov./Dec., 1982 March, 1982. Working Paper 1281-82. TR-5 Van Maanen, John, & Deborah Kolb. "The...process." Quality and Quantity, 11:97-117. Becker, Allen L. 1982 "On Emerson on Language." in David Tannen (ed.), Analyzing Discourse: 39-62

Visualizing Concordance of Sets

DTIC Science & Technology

2006-01-01

Elements Filtering with Human Muscular Dystrophy Dataset of 21 sets and 163 elements. 4.1.4 Diagram Ordering using the Rank-by-Feature Framework...Proceedings of Advanced Visual Interfaces, pp. 110-119, 2000. [4] R. A. Becker and W. S. Cleveland, "Brushing Scatterplots," Technometrics, vol. 29, pp. 127

Poor Facial Affect Recognition among Boys with Duchenne Muscular Dystrophy

ERIC Educational Resources Information Center

Hinton, V. J.; Fee, R. J.; De Vivo, D. C.; Goldstein, E.

2007-01-01

Children with Duchenne or Becker muscular dystrophy (MD) have delayed language and poor social skills and some meet criteria for Pervasive Developmental Disorder, yet they are identified by molecular, rather than behavioral, characteristics. To determine whether comprehension of facial affect is compromised in boys with MD, children were given a…

Symposium on the Paragraph.

ERIC Educational Resources Information Center

Christensen, Francis; And Others

1966-01-01

Three authors comment upon each other's views as expressed in previous articles on the paragraph. Francis Christensen discusses the "sentence-based" theory and compares it to other descriptions of the paragraph. A.L. Becker compares Christensen's and Paul Rodgers' views and discusses their major differences in (1) identity and variation in…

Training for Inner City Parents in Child Rearing: Why Fried Chicken Franchises for Parenting Don't Work.

ERIC Educational Resources Information Center

James, Richard; Etheridge, George

In an effort to examine the effectiveness of commercially produced parent education programs, a child management and communication class given for Memphis, Tennessee, inner city parents is evaluated in this paper. The program, sponsored by the Mid-South Teacher Corps Project, utilized two models: (1) Becker's 1971 "Parents Are Teachers: A…

The cost of murder. Anti-violence groups, hospitals work to try and stop homicides, which have negative impacts on both the community and bottom lines.

PubMed

Carlson, Joe

2009-11-30

The brutal toll of homicide in their communities has some hospitals working with anti-violence groups to halt the slaughter by trying to stop retaliation. Talking to victims after an attack is "really this golden window of opportunity, when they're open to change", says Marla Becker, left, of Youth Alive.

Why is Southern African canine babesiosis so virulent? An evolutionary perspective.

PubMed

Penzhorn, Barend L

2011-04-13

Canine babesiosis is a common, highly virulent disease in Southern Africa with even pups and juveniles being severely affected. This contrasts with bovine babesiosis, for example, where host, parasite and vector co-evolved and young animals develop immunity after infection without showing clinical signs. Babesia rossi, the main causative organism of canine babesiosis in sub-Saharan Africa, was first described from a side-striped jackal (Canis adustus) in Kenya. Although data are meagre, there is evidence that indigenous African canids, such as jackals and wild dogs (Lycaon pictus), can harbour the parasite without showing untoward effects. Dogs are not indigenous to Africa. The vast majority of dogs presented at veterinary facilities in South Africa represent recently introduced European, Asian or American breeds. The contention is that B. rossi is a new challenge to which these dogs have not adapted. With intensive treatment of clinical cases, natural selection is effectively negated and the status quo will probably be maintained indefinitely. It is postulated that Babesia vogeli, which frequently results in unapparent infections or mild manifestations in dogs, represents or is closely related to the ancestral form of the canine parasite, possibly originating from wolves (Canis lupus).

Why is Southern African canine babesiosis so virulent? An evolutionary perspective

PubMed Central

2011-01-01

Canine babesiosis is a common, highly virulent disease in Southern Africa with even pups and juveniles being severely affected. This contrasts with bovine babesiosis, for example, where host, parasite and vector co-evolved and young animals develop immunity after infection without showing clinical signs. Babesia rossi, the main causative organism of canine babesiosis in sub-Saharan Africa, was first described from a side-striped jackal (Canis adustus) in Kenya. Although data are meagre, there is evidence that indigenous African canids, such as jackals and wild dogs (Lycaon pictus), can harbour the parasite without showing untoward effects. Dogs are not indigenous to Africa. The vast majority of dogs presented at veterinary facilities in South Africa represent recently introduced European, Asian or American breeds. The contention is that B. rossi is a new challenge to which these dogs have not adapted. With intensive treatment of clinical cases, natural selection is effectively negated and the status quo will probably be maintained indefinitely. It is postulated that Babesia vogeli, which frequently results in unapparent infections or mild manifestations in dogs, represents or is closely related to the ancestral form of the canine parasite, possibly originating from wolves (Canis lupus). PMID:21489239

Transforming Graph Data for Statistical Relational Learning

DTIC Science & Technology

2012-10-01

Jordan, 2003), PLSA (Hofmann, 1999), ? Classification via RMN (Taskar et al., 2003) or SVM (Hasan, Chaoji, Salem , & Zaki, 2006) ? Hierarchical...dimensionality reduction methods such as Principal 407 Rossi, McDowell, Aha, & Neville Component Analysis (PCA), Principal Factor Analysis ( PFA ), and...clustering algorithm. Journal of the Royal Statistical Society. Series C, Applied statistics, 28, 100–108. Hasan, M. A., Chaoji, V., Salem , S., & Zaki, M

Annual Assessment of Longitudinal Studies and Injury Surveillance for Gender Integration in the Army, 2016

DTIC Science & Technology

2017-03-01

REPORT DOCUMENTATION PAGE Form Approved OMB No. 0704-0188 The public reporting burden for this collection of information is estimated to average 1...AUTHOR(S) Keith Hauret and Dr. Bruce Jones (Army Public Health Center) Cindy Bush, Steve Rossi, and Melissa Richardson (contractors for the Armed Forces...ORGANIZATION NAME(S) AND ADDRESS(ES) Army Public Health Center, Clinical Public Health and Epidemiology Directorate, Injury Prevention Division; Aberdeen

Annual Surveillance Summary: Klebsiella Infections in the Military Health System (MHS), 2015

DTIC Science & Technology

2017-03-01

EDC-TR-190-2017 By Kristen Rossi and Uzo Chukwuma EpiData Center Department Prepared March 2017 Approved for public release. Distribution is...treatments to combat them, resulting in wide-ranging global public health implications.4 Furthermore, bacteria with carbapenem-resistant genes...Prepared March 2017 EpiData Center Department NMCPHC-EDC-TR-190-2017 In 2001, Jones et al. reported that nosocomial infections account for more than

Accurate Virus Quantitation Using a Scanning Transmission Electron Microscopy (STEM) Detector in a Scanning Electron Microscope

DTIC Science & Technology

2017-06-29

Accurate Virus Quantitation Using a Scanning Transmission Electron Microscopy (STEM) Detector in a Scanning Electron Microscope Candace D Blancett1...L Norris2, Cynthia A Rossi4 , Pamela J Glass3, Mei G Sun1,* 1 Pathology Division, United States Army Medical Research Institute of Infectious...Diseases (USAMRIID), 1425 Porter Street, Fort Detrick, Maryland, 21702 2Biostatistics Division, United States Army Medical Research Institute of

Aligning Instruction and Assessment with Game and Simulation Design. CRESST Report 780

ERIC Educational Resources Information Center

Wainess, Richard; Koenig, Alan; Kerr, Deirdre

2011-01-01

Effective design of training-related games (games for training and/or assessment) requires synergy between the mechanisms for delivering instructional content and the mechanisms for learning game play and game functionality (Becker, 2006). The learning domain must be embedded as a core game mechanic: that is, the game cannot be advanced or won…

Evaluating Twitter and Its Impact on Student Learning in Principles of Economics Courses

ERIC Educational Resources Information Center

Al-Bahrani, Abdullah; Patel, Darshak; Sheridan, Brandon J.

2017-01-01

Ever since Becker and Watts (1996) found that economic educators rely heavily on "chalk and talk" as a primary teaching method, economic educators have been seeking new ways to engage students and improve learning outcomes. Recently, the use of social media as a pedagogical tool in economics has received increasing interest. The authors…

Utilization and Perceived Impact of Smart Phone Apps Among Persons Pursuing Mental Health Services

DTIC Science & Technology

2015-12-01

impact of smart phone apps among persons pursuing mental health services Robin E. Becker, MA*, Daniel G. Cassidy, PhD, and William C. Isler, PhD...applications (apps) designed for use on phones and other ‘smart’ devices. The purpose of this study is to address 1) whether and by what means individuals

Comment on "Habitat split and the global decline of amphibians".

PubMed

Cannatella, David C

2008-05-16

Becker et al. (Reports, 14 December 2007, p. 1775) reported that forest amphibians with terrestrial development are less susceptible to the effects of habitat degradation than those with aquatic larvae. However, analysis with more appropriate statistical methods suggests there is no evidence for a difference between aquatic-reproducing and terrestrial-reproducing species.

Testing the Human Capital Development Model: The Case of Apprenticeships in Turkey

ERIC Educational Resources Information Center

Akpinar, Taner; Gün, Servet

2016-01-01

Human capital theory was developed to study how individual agents make rational choices or how they invest in human capital to maximize their welfare. One of the leading founders of this perspective, Becker, argues that schooling, on-the-job training, medical care, migration and searching for information about prices and incomes are different…

Is "Connected Teaching" in Mathematics a Gender-Equitable Pedagogy for Adults?

ERIC Educational Resources Information Center

Miller-Reilly, Barbara

2008-01-01

"Connected teaching" is a feminist theory first proposed by Belenky, Clinchy, Goldberger and Tarule (1996, p. 214) then related to the teaching of mathematics by Becker (1995, 1996), Buerk (1994b) and Morrow (1996). The theory of intellectual development elaborated by Belenky and her colleagues uncovered themes common to many women's…

EFFECT OF THREE DIFFERENT SIZED FRACTIONS OF OUTDOOR PM ON INFLAMMATORY AND OXIDATIVE MARKERS IN VIVO

EPA Science Inventory

EFFECT OF THREE DIFFERENT SIZED FRACTIONS OF OUTDOOR PM ON INFLAMMATORY MARKERS IN VIVO
C A J Dick', P Singh2, P. Evansky3, S Becker3 and M I Gilmour3.
'Center For Environmental Medicine and Lung Biology, UNC, Chapel Hill, NC 27599 2NCSU, Raleigh, NC 'Experimental Toxicolog...

Chemical Modifications of Antisense Morpholino Oligomers Enhance Their Efficacy against Ebola Virus Infection

DTIC Science & Technology

2009-05-01

RNA replication. J. Virol. 79:4599–4609. 6. Dolnik, O., L. Kolesnikova, and S. Becker . 2008. Filoviruses: interactions with the host cell. Cell. Mol... dystrophy ) and inhibit murine coronavirus rep- lication in vivo. Biochem. Soc. Trans. 35:826–828. 22. Moulton, H. M., M. H. Nelson, S. A. Hatlevig, M. T

The Bicycle Helmet Attitudes Scale: Using the Health Belief Model to Predict Helmet Use among Undergraduates

ERIC Educational Resources Information Center

Ross, Thomas P.; Ross, Lisa Thomson; Rahman, Annalise; Cataldo, Shayla

2010-01-01

Objective: This study examined bicycle helmet attitudes and practices of college undergraduates and developed the Bicycle Helmet Attitudes Scale, which was guided by the Health Belief Model (HBM; Rosenstock, 1974, in Becker MH, ed. "The Health Belief Model and Personal Health Behavior". Thorofare, NJ: Charles B. Slack; 1974:328-335) to predict…

Tick-borne haemoparasites and Anaplasmataceae in domestic dogs in Zambia.

PubMed

Qiu, Yongjin; Kaneko, Chiho; Kajihara, Masahiro; Ngonda, Saasa; Simulundu, Edgar; Muleya, Walter; Thu, May June; Hang'ombe, Mudenda Bernard; Katakura, Ken; Takada, Ayato; Sawa, Hirofumi; Simuunza, Martin; Nakao, Ryo

2018-05-01

Tick-borne diseases (TBDs), including emerging and re-emerging infectious diseases, are important threats to human and animal health worldwide. Indeed, the number of reported human and animal infectious cases of novel TBD agents has increased in recent decades. However, TBDs tend to be neglected, especially in resource-limited countries that often have limited diagnostic capacity. The aim of this molecular survey was to detect and characterise tick-borne pathogens (Babesia, Theileria, and Hepatozoon parasites and Anaplasmataceae bacteria) in domestic dogs in Zambia. In total, 247 canine peripheral blood samples were collected in Lusaka, Mazabuka, Monze, and Shangombo. Conventional PCR to detect the selected pathogens was performed using DNA extracted from canine blood. One hundred eleven samples were positive for protozoa and 5 were positive for Anaplasmataceae. Sequencing of thirty-five randomly selected protozoa-positive samples revealed the presence of Babesia rossi, Babesia vogeli, and Hepatozoon canis 18S rDNA. Based on these sequences, a multiplex PCR system was developed to yield PCR products with different amplicons, the size of which depended on the parasite species; thus, each species could be identified without the need for sequence analysis. Approximately 40% of dogs were positive for H. canis. In particular, the positive rate (75.2%) of H. canis infection was significantly higher in Shangombo than in other sampling sites. Multiplex PCR assay detected B. rossi and B. vogeli infections in five and seven dogs, respectively, indicating that this approach is useful for detecting parasites with low prevalence. Sequencing analysis of gltA and groEL genes of Anaplasmataceae revealed that two and one dogs in Lusaka were infected with Anaplasma platys and Ehrlichia canis, respectively. The data indicated that Zambian dogs were infected with multiple tick-borne pathogens such as H. canis, B. rossi, B. vogeli, A. platys, E. canis and uncharacterized Ehrlichia sp

Coalition readiness management system preliminary interoperability experiment (CReaMS PIE)

NASA Astrophysics Data System (ADS)

Clark, Peter; Ryan, Peter; Zalcman, Lucien; Robbie, Andrew

2003-09-01

The United States Navy (USN) has initiated the Coalition Readiness Management System (CReaMS) Initiative to enhance coalition warfighting readiness through advancing development of a team interoperability training and combined mission rehearsal capability. It integrates evolving cognitive team learning principles and processes with advanced technology innovations to produce an effective and efficient team learning environment. The JOint Air Navy Networking Environment (JOANNE) forms the Australian component of CReaMS. The ultimate goal is to link Australian Defence simulation systems with the USN Battle Force Tactical Training (BFTT) system to demonstrate and achieve coalition level warfare training in a synthetic battlespace. This paper discusses the initial Preliminary Interoperability Experiment (PIE) involving USN and Australian Defence establishments.

Women's History Month Event

NASA Image and Video Library

2018-03-27

JoAnn Morgan, former associate director of NASA Kennedy Space Center, speaks to workers during a Women's History Month event at the center. With the theme "Nevertheless She Persisted," Morgan described her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Women's History Month Event

NASA Image and Video Library

2018-03-27

JoAnn Morgan, former associate director of NASA Kennedy Space Center, was the keynote speaker during a Women's History Month event at the center. With the theme "Nevertheless She Persisted," Morgan described her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Women's History Month Event

NASA Image and Video Library

2018-03-27

JoAnn Morgan, former associate director of Kennedy Space Center, was the keynote speaker during a Women's History Month event at the center. With the theme "Nevertheless She Persisted," Morgan described her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Flavivirus RNA Replication: Essential Viral Functions as Targets for Antiviral Therapeutics

DTIC Science & Technology

1991-04-30

therapeutics 5 S2P• SONAI . aUTl4Of4S) Marc S. Collett and JoAnn A. Suzich 138. TYPE OF REPORr 𔃽m, TME COVERED 14 DATE OF REPoRT IYfm ,oW,.Oav,-iy PAGE COUNT...H O N E ( A w h p o A m &• iC g a 2 2 c. O WF C E S Y M B O L Mrs. Virginia M. Miller 301,-663-7325 ..,) ....•• Z,. OOFom 1473, JUI NSE oet,, are tv...supernatant fraction, a particulate fraction (P20) was isolated by centrifugation at 20,000 x g . The P20 fraction contained nearly all of the viral RDRP

77 FR 72387 - Jeffery J. Becker, D.D.S., and Jeffery J. Becker, D.D.S., Affordable Care Decision and Order

Federal Register 2010, 2011, 2012, 2013, 2014

2012-12-05

....' '' Order Regarding Respondent's Multiple Motions For Appropriate Relief (ALJ Ex. 25), at 10 (quoting Forest... silent on the requirements of a registrant under 21 CFR 1307.21.'' Gov. Resp. to Respondent's Motion to...

Remote Sensing Data Analytics for Planetary Science with PlanetServer/EarthServer

NASA Astrophysics Data System (ADS)

Rossi, Angelo Pio; Figuera, Ramiro Marco; Flahaut, Jessica; Martinot, Melissa; Misev, Dimitar; Baumann, Peter; Pham Huu, Bang; Besse, Sebastien

2016-04-01

Planetary Science datasets, beyond the change in the last two decades from physical volumes to internet-accessible archives, still face the problem of large-scale processing and analytics (e.g. Rossi et al., 2014, Gaddis and Hare, 2015). PlanetServer, the Planetary Science Data Service of the EC-funded EarthServer-2 project (#654367) tackles the planetary Big Data analytics problem with an array database approach (Baumann et al., 2014). It is developed to serve a large amount of calibrated, map-projected planetary data online, mainly through Open Geospatial Consortium (OGC) Web Coverage Processing Service (WCPS) (e.g. Rossi et al., 2014; Oosthoek et al., 2013; Cantini et al., 2014). The focus of the H2020 evolution of PlanetServer is still on complex multidimensional data, particularly hyperspectral imaging and topographic cubes and imagery. In addition to hyperspectral and topographic from Mars (Rossi et al., 2014), the use of WCPS is applied to diverse datasets on the Moon, as well as Mercury. Other Solar System Bodies are going to be progressively available. Derived parameters such as summary products and indices can be produced through WCPS queries, as well as derived imagery colour combination products, dynamically generated and accessed also through OGC Web Coverage Service (WCS). Scientific questions translated into queries can be posed to a large number of individual coverages (data products), locally, regionally or globally. The new PlanetServer system uses the the Open Source Nasa WorldWind (e.g. Hogan, 2011) virtual globe as visualisation engine, and the array database Rasdaman Community Edition as core server component. Analytical tools and client components of relevance for multiple communities and disciplines are shared across service such as the Earth Observation and Marine Data Services of EarthServer. The Planetary Science Data Service of EarthServer is accessible on http://planetserver.eu. All its code base is going to be available on GitHub, on

Local Anesthesia Part 2: Technical Considerations

PubMed Central

Reed, Kenneth L.; Malamed, Stanley F.; Fonner, Andrea M.

2012-01-01

An earlier paper by Becker and Reed provided an in-depth review of the pharmacology of local anesthetics. This continuing education article will discuss the importance to the safe and effective delivery of these drugs, including needle gauge, traditional and alternative injection techniques, and methods to make injections more comfortable to patients. PMID:23050753

Stimulation of vesicular-arbuscular mycorrhizal fungi by mycotrophic and nonmycotrophic plant root systems.

PubMed

Schreiner, R P; Koide, R T

1993-08-01

Transformed root cultures of three nonmycotrophic and one mycotrophic plant species stimulated germination and hyphal growth of the vesicular-arbuscular mycorrhizal fungus Glomus etunicatum (Becker & Gerd.) in a gel medium. However, only roots of the mycotrophic species (carrot) supported continued hyphal exploration after 3 to 4 weeks and promoted appressoria formation by G. etunicatum.

Towards Distributed Intelligence: A High Level Definition

DTIC Science & Technology

2004-12-01

Some of the first research in multi-robot systems came in the foraging /sorting area by Parker [77] and Beckers [9] and was likely fueled by the bio...chapter 24, pages 28–39. Artificial Intelligence at MIT. The MIT Press, 1989. 15. R.A. Brooks. Robotic Science, chapter 11, The Whole Iguana , pages 432

A Programmatic Approach to Determine Eligibility of Prehistoric Sites in the San Diego Subregion, Southern Coast Archeological Region, California, for the National Register of Historic Places

DTIC Science & Technology

2007-08-01

Inland Highland .......................................................................................22 3 Prehistoric Human Adaptations ...highlands on the base has provided valuable insight regarding late Holocene adaptations in these landscapes (Hale and Becker 2005; Reddy 1997a, 2000; York...interpretations of the prehistoric human adaptations in the four landscape categories. Major research issues relevant to the study and the study area are

War/Peace Report. What Should Kids Be Taught About Peace and War? Volume 11, Number 1.

ERIC Educational Resources Information Center

New York Friends Group, Inc., New York. Center for War/Peace Studies.

This article is reprinted from a monthly publication of fact and opinion on progress toward a world of peace with justice. The feature article presents a dialogue among educators who have been designing strategies for war/peace studies. Speaking on the topic in the title were: James Becker, director of Foreign Policy Association's School Service…

Proceedings of a Workshop on Cognitive Testing Methodology (11-12 June 1984)

DTIC Science & Technology

1986-01-01

Becker , and G.E. Handlemann. 1979. Hippocampus, space, and memory. Behav. Brain Sci. 2:313-365. Osbourne, D.P., E.R. Brown, and C.T. Randt. 1982...the B complex. J. Appl. Psychol. 30:359-379. Chernorutskii, M.V. 1943. Problem of alimentary dystrophy . Stud. Lenirg:ad Physicians 3:3-13. Cited in A

Stealth Evangelism and the Public Schools

ERIC Educational Resources Information Center

Boston, Bob

2010-01-01

United Methodist minister David Jenkins was meeting with fellow clergy last year in the small Kansas community of Sharon Springs when one of the pastors made what sounded like a routine request. He noted that the Todd Becker Foundation was coming to town, and the evangelical Christian organization, which purports to warn youngsters about the…

VizieR Online Data Catalog: The North 20cm Survey (White+ 1992)

NASA Astrophysics Data System (ADS)

White, R. L.; Becker, R. H.

1995-08-01

This catalog contains a list of 30239 radio sources at 1.4GHz over the declination range of -5 to +82 degrees. This catalog is based on the Green Bank 1.4GHz Northern Sky Survey (Condon and Broderick, 1986, Cat. VIII/6) which was generated using the Green Bank 300 foot (91m) telescope. The threshold for identifying a 1.4GHz radio source was set at 100mJy. The catalog data include the source name, a confusion flag, right ascension (1950), declination (1950), 1.4GHz flux, a flag to indicate if the source is extended at 20cm, 4.85GHz flux (from Becker et al., 1991, Cat. VIII/13), a flag to indicate if the source is extended at 6cm, 365MHz flux (from the Texas Survey, Douglas et al. 1980; see Cat. VIII/42)), spectral index between 6 and 20 cm, and spectral index between 20 and 80 cm. Where possible, the source name is derived from the 6cm catalog of Becker et al. 1991 (Cat. VIII/13). (1 data file).

Crime and punishment: Does it pay to punish?

NASA Astrophysics Data System (ADS)

Iglesias, J. R.; Semeshenko, V.; Schneider, E. M.; Gordon, M. B.

2012-08-01

Crime is the result of a rational distinctive balance between the benefits and costs of an illegal act. This idea was proposed by Becker more than forty years ago (Becker (1968) [1]). In this paper, we simulate a simple artificial society, in which agents earn fixed wages and can augment (or lose) wealth as a result of a successful (or not) act of crime. The probability of apprehension depends on the gravity of the crime, and the punishment takes the form of imprisonment and fines. We study the costs of the law enforcement system required for keeping crime within acceptable limits, and compare it with the harm produced by crime. A sharp phase transition is observed as a function of the probability of punishment, and this transition exhibits a clear hysteresis effect, suggesting that the cost of reversing a deteriorated situation might be much higher than that of maintaining a relatively low level of delinquency. Besides, we analyze economic consequences that arise from crimes under different scenarios of criminal activity and probabilities of apprehension.

Quantification of transuranic elements by time interval correlation spectroscopy of the detected neutrons

PubMed

Baeten; Bruggeman; Paepen; Carchon

2000-03-01

The non-destructive quantification of transuranic elements in nuclear waste management or in safeguards verifications is commonly performed by passive neutron assay techniques. To minimise the number of unknown sample-dependent parameters, Neutron Multiplicity Counting (NMC) is applied. We developed a new NMC-technique, called Time Interval Correlation Spectroscopy (TICS), which is based on the measurement of Rossi-alpha time interval distributions. Compared to other NMC-techniques, TICS offers several advantages.

Multi-Scale Computational Analyses of JP-8 Fuel Droplets and Vapors in Human Respiratory Airway Models

DTIC Science & Technology

2007-10-31

equation of ultrafine particles , or (JP-8) fuel vapor, whose dominant radial transfer mechanisms are Brownian motion and turbulent dispersion is given in...Deposition of ultrafine particles at carinal ridges of the upper bronchial airways. Aerosol Science and Technology 38, 991-1000. Comer, J.K...from studies of ultrafine particles . Environmental Health Perspectives 113, 823-839. Ritchie, G., Still, K., Rossi III, J., Bekkedal, M., Bobb, A. and

The Search for Quality by Management in Service-Related Industry Today

DTIC Science & Technology

1988-09-05

service delivery. Rossy, Gerard L. ’The Executive’s Role in Ethics: The View from Business and Industry.* Healthcare Executive 2 (September/October...to study and compare: h-spitality, pharmaceutical, and healthcare . The hospitality industry was identified due to the dependence upon the consumer. If...only regulatory requirements, but a social obligation. The healthcare industry was chosen due to the need for quality, the increased competition, and

Timing and Spectral Study of 4U 1538-52

NASA Technical Reports Server (NTRS)

Clark, George W.

2000-01-01

Improved orbital parameters of the high-mass binary X-ray pulsar 4U 1538-52 have been derived from high count rate data obtained from the Rossi X-Ray Timing Explorer. Pulse-timing analysis yields an eccentricity of 0.174 +/- 0.015, a periastron at 64 deg +/- 9 deg, and evidence of orbital decay with prime-P(sub orb)/P(sub orb) = (-2.9 +/- 2.1) x 10(exp -6)/yr.

Toward Meaningful Military Compensation Reform: Research in Support of DoD’s Review

DTIC Science & Technology

2014-01-01

CARE INFRASTRUCTURE AND TRANSPORTATION INTERNATIONAL AFFAIRS LAW AND BUSINESS NATIONAL SECURITY POPULATION AND AGING PUBLIC SAFETY SCIENCE AND...have highlighted the advantages of a hybrid plan for achieving these objectives. For example, the Defense Science Board Task Force on Human...Compensation, as well as the input of Patricia Mulcahy. We are grateful to Joel Sitrin, Chief Actuary , and Peter Rossi and Peter Zouras of the DoD Office of

A Randomized Phase 2 Trial of 177Lu Radiolabeled Anti-PSMA Monoclonal Antibody J591 in Patients With High-Risk Castrate, Biochemically Relapsed Prostate Cancer

DTIC Science & Technology

2017-09-01

MDS Myelodysplastic syndrome and acute myeloid leukemia has been reported in patients previously treated with anti-CD20 based RIT for non-hodgkin’s...RR, Rossi A, Jhaveri K, Feldman EJ, Leonard JP. Therapy-related myelodysplastic syndrome and acute myeloid leukemia following initial treatment with... syndrome and acute myelogenous leukemia in patients treated with ibritumomab tiuxetan radioimmunotherapy. J Clin Oncol. 2007 Sep 20;25(27):4285-92. 119

A Species Distribution Modeling Informed Conservation Assessment of Bog Spicebush

DTIC Science & Technology

2016-09-14

small populations, or establishing new popu- lations to increase the metapopulation structure within clusters of popula- tions. These outplanting data...fire suppression, and flooding (e.g., due to beaver activity) on vege- tation composition and structure . This effort used vegetation and disturb- ance...Suppression to Prescription. Tall Timbers Research Station, Tallahassee, FL, pp 70–81. Godefroid, S., C. Piazza, G. Rossi, S. Buord, A. D. Stevens, R

Assessing Post Conflict State Building Efforts

DTIC Science & Technology

2013-03-01

Develop a global partnership for development Target: Develop further an open, rule-based, predictable, non-discriminatory trading and finance system...Beginner’s Guide to Nation-Building (Santa Monica, CA: Rand Corporation ), 2007, xvii. 15 Samuel Berger, Brent Scowcroft, and William L. Nash, “In...The Beginner’s Guide, xxi. 25 Richard A. Berk and Peter H. Rossi, Thinking About Program Evaluation 2, (Thousand Oaks, CA: Sage Publications, 1999

Polynomial complexity despite the fermionic sign

NASA Astrophysics Data System (ADS)

Rossi, R.; Prokof'ev, N.; Svistunov, B.; Van Houcke, K.; Werner, F.

2017-04-01

It is commonly believed that in unbiased quantum Monte Carlo approaches to fermionic many-body problems, the infamous sign problem generically implies prohibitively large computational times for obtaining thermodynamic-limit quantities. We point out that for convergent Feynman diagrammatic series evaluated with a recently introduced Monte Carlo algorithm (see Rossi R., arXiv:1612.05184), the computational time increases only polynomially with the inverse error on thermodynamic-limit quantities.

The Effects of Parenting Styles on Children's Self-Esteem: A Developmental Perspective.

ERIC Educational Resources Information Center

Elings, Joette Renee

The effects of parenting styles on children's self-esteem were examined by dividing the empirical literature into three age groups: the preschool child; the elementary child; and the adolescent. Major literature reviews by W. C. Becker, B. Martin, and E. E. Maccoby and J. A. Martin are discussed and inconsistencies among the reviews are examined.…

Stimulation of Vesicular-Arbuscular Mycorrhizal Fungi by Mycotrophic and Nonmycotrophic Plant Root Systems

PubMed Central

Schreiner, R. Paul; Koide, Roger T.

1993-01-01

Transformed root cultures of three nonmycotrophic and one mycotrophic plant species stimulated germination and hyphal growth of the vesicular-arbuscular mycorrhizal fungus Glomus etunicatum (Becker & Gerd.) in a gel medium. However, only roots of the mycotrophic species (carrot) supported continued hyphal exploration after 3 to 4 weeks and promoted appressoria formation by G. etunicatum. Images PMID:16349030

Survey of Research into Electromagnetic and Other Wave Effects on Mechanical Behavior and Processing of Materials

DTIC Science & Technology

1992-01-01

Suisse des Electriciens, Vol. 64, No. 16, 1973, 991-997. 469. U. Becker, and G. Strese, "Welding of Plastics", Kunststoffe , Vol. 59, No. 10, 1969, 679-684...Magnetic Fields", Kautschuk und Gummi Kunststoffe , Vol. 39, No. 12, 1986, 1182-1185. 681. T. A. Manko, A. N. Kvasha, A. V. Solovev, and I. M. Ermolaev

Behavior Principles Structural Model of a Follow Through Program, Dayton, Ohio: Model Programs. Childhood Education.

ERIC Educational Resources Information Center

American Institutes for Research in the Behavioral Sciences, Palo Alto, CA.

Prepared for a White House Conference on Children (December 1970), this report describes a program in which first- through third-graders in three schools in Dayton, Ohio, participate in a model of a Follow Through program sponsored by Siegfried Engelmann and Wesley Becker of the University of Oregon at Eugene. All teachers chose to participate,…

Questioning quantum mechanics

NASA Astrophysics Data System (ADS)

Frappier, Mélanie

2018-03-01

A century after its inception, quantum mechanics continues to puzzle us with dead-and-alive cats, waves "collapsing" into particles, and "spooky action at a distance." In his first book, What Is Real?, science writer and astrophysicist Adam Becker sets out to explore why the physics community is still arguing today about quantum mechanics's true meaning.

Root traits contributing to plant productivity under drought

USDA-ARS?s Scientific Manuscript database

ROOT TRAITS CONTRIBUTING TO PLANT PRODUCTIVITY UNDER DROUGHT L.H. Comas1, S.R. Becker2, V.M.V. Cruz3,4, P.F. Byrne2, D.A. Dierig3 1USDA-ARS, Water Management Research Unit, Fort Collins, CO, USA 2Colorado State University, Soil and Crop Sciences, Fort Collins, CO, USA 3USDA-ARS, National Center fo...

Minority Language Researchers and Their Role in Policy Development

ERIC Educational Resources Information Center

Gorter, Durk

2012-01-01

This paper deals with the role of researchers in the development of language policies for European minority languages. This question is placed in the context of a long-standing debate in sociology to which several authors have contributed; among them are Max Weber, Howard Becker and Alvin Gouldner. This article also briefly refers to the European…

Family Decisionmaking Over the Life Cycle: Some Implications for Estimating the Effects of Income Maintenance Programs.

ERIC Educational Resources Information Center

Smith, James P.

The standard one-period labor supply model that economists have used is in some ways an inadequate tool to evaluate a Family Assistance Plan (FAP). The principal difficulty is that an FAP will have important interperiod or life cycle effects. The pure life cycle model, an extension of the work of Becker and Ghez, is derived here without reference…

Optimal Consumption When Consumption Takes Time

ERIC Educational Resources Information Center

Miller, Norman C.

2009-01-01

A classic article by Gary Becker (1965) showed that when it takes time to consume, the first order conditions for optimal consumption require the marginal rate of substitution between any two goods to equal their relative full costs. These include the direct money price and the money value of the time needed to consume each good. This important…

Temporal Variations of Strength and Location of the South Atlantic Anomaly as Measured by RXTE

NASA Technical Reports Server (NTRS)

Wilms, Jorn; Felix, Furst; Rothschild, Richard E.; Pottschmidt, Katja; Smith, David M.; Lingenfelter, Richard

2009-01-01

The evolution of the particle background at an altitude of approx.540km during the time interval between 1996 and 2007 is studied using the particle monitor of the High Energy X-ray Timing Experiment on board NASA's Rossi X-ray Timing Explorer. A special emphasis of this study is the location and strength of the South Atlantic Anomaly (SAA). The size and strength of the SAA are anti-correlated with the the 10.7 cm radio flux of the Sun, which leads the SAA strength by approx.1 year reflecting variations in solar heating of the upper atmosphere. The location of the SAA is also found to drift westwards with an average drift rate of about 0.3deg/yr following the drift of the geomagnetic field configuration. Superimposed to this drift rate are irregularities, where the SAA suddenly moves eastwards and where furthermore the speed of the drift changes. The most prominent of these irregularities is found in the second quarter of 2003 and another event took place in 1999. We suggest that these events are previously unrecognized manifestations of the geomagnetic jerks of the Earth's magnetic field. Key words: space radiation environment, South Atlantic Anomaly, radiation monitors, Rossi X-ray Timing Explorer

Normal and Differential SAR Interferometry

DTIC Science & Technology

2005-02-01

incorporating the use of a rough DEM. [ Adragna 1995]. The same technique is also used for flat Earth removal, and for differential interferometry (Cap.5...and F. Adragna , 1994. Radar Interferometric Mapping of Deformation in the Year After the Landers Earthquake, Nature, Vol. 369, pp. 227-230 Massonnet...D., M. Rossi, C. Carmona, F. Adragna , G. Peltzer, K. Feigi, and T. Rabaute, 1993. The Displacement Field of the Landers Earthquake Mapped by Radar

Women's History Month Event

NASA Image and Video Library

2018-03-27

NASA Kennedy Space Center's Deputy Director Janet Petro welcomes workers to the center's Women's History Month event, with the theme "Nevertheless She Persisted." Keynote speaker, JoAnn Morgan, former associate director of the center, spoke to the group about her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Women's History Month Event

NASA Image and Video Library

2018-03-27

JoAnn Morgan, former associate director of Kennedy Space Center, at left, accepts a special coin from Janet Petro, deputy director of Kennedy, during a Women's History Month event at the center. With the theme "Nevertheless She Persisted," Morgan described her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Women's History Month Event

NASA Image and Video Library

2018-03-27

JoAnn Morgan, far left at the podium, former associate director of Kennedy Space Center, was the keynote speaker during a Women's History Month event at the center. With the theme "Nevertheless She Persisted," Morgan described her experience as the first female engineer working in the space program in the 1960s. Morgan was the first female in the Launch Control Center firing room during the Apollo 11 launch. The event was hosted by the center's Kennedy Networking Opportunities for Women (KNOW) and Launching Leaders organizations. The purpose of KNOW is to provide focus on issues such as employment, retention, promotion, training, career and personal development, education, and identify and eliminate barriers that hinder the advancement of women in the workforce.

Hermetic Encapsulation of Nanoenergetic Porous Silicon Wafer by Parylene

DTIC Science & Technology

2014-08-01

Hermetic Encapsulation of Nanoenergetic Porous Silicon Wafer by Parylene by Eugene Zakar, Wayne Churaman, Collin Becker, Bernard Rod, Luke...Laboratory Adelphi, MD 20783-1138 ARL-TR-7025 August 2014 Hermetic Encapsulation of Nanoenergetic Porous Silicon Wafer by Parylene...Hermetic Encapsulation of Nanoenergetic Porous Silicon Wafer by Parylene 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6

Walking with purpose after an HPV-related head and neck cancer diagnosis | Center for Cancer Research

Cancer.gov

In November 2015, Michael Becker was diagnosed with Stage IV head and neck cancer caused by a strain of the human papillomavirus, or HPV. He now advocates for education and awareness of the HPV vaccine as cancer prevention for both boys and girls. “I want to make people aware that you can avoid my situation by getting the shot.” Read more…

Rational addiction to alcohol: panel data analysis of liquor consumption.

PubMed

Baltagi, Badi H; Griffin, James M

2002-09-01

Utilizing a panel data set of 42 states over the period 1959-1994, this paper estimates a rational addiction model for liquor consumption for the US. The empirical evidence is consistent with the rational addiction hypothesis proposed by Becker and Murphy. However, the results are sensitive to the assumption of homogeneity across states or over time. Copyright 2002 John Wiley & Sons, Ltd.

Endocrine Therapy of Breast Cancer

DTIC Science & Technology

2006-06-01

Becker muscular dystrophy (BMD, hypomorphic for dys- trophin, n = 5), Dysferlin deficiency (putative vesicle traf- fic defect, n = 9), and Calpain III...this study. (1) Limb-girdle muscular dystrophy (LGMD, provided by Children National Medical Center, Center for Genetic Medicine): 4 diagnostic...groups, Fukutin related protein de- ficiency (FKRP) (homozygous missense for glycosylation enzyme, limb-girdle muscular dystrophy sub-type, n = 7

Translational Research for Muscular Dystrophy

DTIC Science & Technology

2012-05-01

common in-frame deletions for which clinical information from human Becker muscular dystrophy patients (deletion of exons 44-45, 49-51, 48-53) is lacking... Dystrophy PRINCIPAL INVESTIGATOR: Gregory A. Cox, Ph.D. CONTRACTING ORGANIZATION: The Jackson Laboratory...REPORT TYPE Annual 3. DATES COVERED 1 MAR 2011 - 30 APR 2012 4. TITLE AND SUBTITLE Translational Research for Muscular Dystrophy 5a. CONTRACT

The Impacts of Family Size on Investment in Child Quality

ERIC Educational Resources Information Center

Caceres-Delpiano, Julio

2006-01-01

Using multiple births as an exogenous shift in family size, I investigate the impact of the number of children on child investment and child well-being. Using data from the 1980 US Census Five-Percent Public Use Micro Sample, 2SLS results demonstrate that parents facing a change in family size reallocate resources in a way consistent with Becker's…

Anti-gravity training improves walking capacity and postural balance in patients with muscular dystrophy.

PubMed

Berthelsen, Martin Peter; Husu, Edith; Christensen, Sofie Bouschinger; Prahm, Kira Philipsen; Vissing, John; Jensen, Bente Rona

2014-06-01

Recent studies in patients with muscular dystrophies suggest positive effects of aerobic and strength training. These studies focused training on using bicycle ergometers and conventional strength training, which precludes more severely affected patients from participating, because of their weakness. We investigated the functional effects of combined aerobic and strength training in patients with Becker and limb-girdle muscular dystrophies with knee muscle strength levels as low as 3% of normal strength. Eight patients performed 10 weeks of aerobic and strength training on an anti-gravity treadmill, which offered weight support up to 80% of their body weight. Six minute walking distance, dynamic postural balance, and plasma creatine kinase were assessed 10 weeks prior to training, immediately before training and after 10 weeks of training. Training elicited an improvement of walking distance by 8±2% and dynamic postural balance by 13±4%, indicating an improved physical function. Plasma creatine kinase remained unchanged. These results provide evidence that a combination of aerobic and strength training during anti-gravity has the potential to safely improve functional ability in severely affected patients with Becker and limb-girdle muscular dystrophies. Copyright © 2014 Elsevier B.V. All rights reserved.

Morpholino oligomer-mediated exon skipping averts the onset of dystrophic pathology in the mdx mouse.

PubMed

Fletcher, Sue; Honeyman, Kaite; Fall, Abbie M; Harding, Penny L; Johnsen, Russell D; Steinhaus, Joshua P; Moulton, Hong M; Iversen, Patrick L; Wilton, Stephen D

2007-09-01

Duchenne and Becker muscular dystrophies are allelic disorders arising from mutations in the dystrophin gene. Duchenne muscular dystrophy is characterized by an absence of functional protein, whereas Becker muscular dystrophy, commonly caused by in-frame deletions, shows synthesis of partially functional protein. Anti-sense oligonucleotides can induce specific exon removal during processing of the dystrophin primary transcript, while maintaining or restoring the reading frame, and thereby overcome protein-truncating mutations. The mdx mouse has a non-sense mutation in exon 23 of the dystrophin gene that precludes functional dystrophin production, and this model has been used in the development of treatment strategies for dystrophinopathies. A phosphorodiamidate morpholino oligomer (PMO) has previously been shown to exclude exon 23 from the dystrophin gene transcript and induce dystrophin expression in the mdxmouse, in vivo and in vitro. In this report, a cell-penetrating peptide (CPP)-conjugated oligomer targeted to the mouse dystrophin exon 23 donor splice site was administered to mdxmice by intraperitoneal injection. We demonstrate dystrophin expression and near-normal muscle architecture in all muscles examined, except for cardiac muscle. The CPP greatly enhanced uptake of the PMO, resulting in widespread dystrophin expression.

The Status of Exon Skipping as a Therapeutic Approach to Duchenne Muscular Dystrophy

PubMed Central

Lu, Qi-Long; Yokota, Toshifumi; Takeda, Shin'ichi; Garcia, Luis; Muntoni, Francesco; Partridge, Terence

2011-01-01

Duchenne muscular dystrophy (DMD) is associated with mutations in the dystrophin gene that disrupt the open reading frame whereas the milder Becker's form is associated with mutations which leave an in-frame mRNA transcript that can be translated into a protein that includes the N- and C- terminal functional domains. It has been shown that by excluding specific exons at, or adjacent to, frame-shifting mutations, open reading frame can be restored to an out-of-frame mRNA, leading to the production of a partially functional Becker-like dystrophin protein. Such targeted exclusion can be achieved by administration of oligonucleotides that are complementary to sequences that are crucial to normal splicing of the exon into the transcript. This principle has been validated in mouse and canine models of DMD with a number of variants of oligonucleotide analogue chemistries and by transduction with adeno-associated virus (AAV)-small nuclear RNA (snRNA) reagents encoding the antisense sequence. Two different oligonucleotide agents are now being investigated in human trials for splicing out of exon 51 with some early indications of success at the biochemical level. PMID:20978473

Stability of transition waves and positive entire solutions of Fisher-KPP equations with time and space dependence

NASA Astrophysics Data System (ADS)

Shen, Wenxian

2017-09-01

This paper is concerned with the stability of transition waves and strictly positive entire solutions of random and nonlocal dispersal evolution equations of Fisher-KPP type with general time and space dependence, including time and space periodic or almost periodic dependence as special cases. We first show the existence, uniqueness, and stability of strictly positive entire solutions of such equations. Next, we show the stability of uniformly continuous transition waves connecting the unique strictly positive entire solution and the trivial solution zero and satisfying certain decay property at the end close to the trivial solution zero (if it exists). The existence of transition waves has been studied in Liang and Zhao (2010 J. Funct. Anal. 259 857-903), Nadin (2009 J. Math. Pures Appl. 92 232-62), Nolen et al (2005 Dyn. PDE 2 1-24), Nolen and Xin (2005 Discrete Contin. Dyn. Syst. 13 1217-34) and Weinberger (2002 J. Math. Biol. 45 511-48) for random dispersal Fisher-KPP equations with time and space periodic dependence, in Nadin and Rossi (2012 J. Math. Pures Appl. 98 633-53), Nadin and Rossi (2015 Anal. PDE 8 1351-77), Nadin and Rossi (2017 Arch. Ration. Mech. Anal. 223 1239-67), Shen (2010 Trans. Am. Math. Soc. 362 5125-68), Shen (2011 J. Dynam. Differ. Equ. 23 1-44), Shen (2011 J. Appl. Anal. Comput. 1 69-93), Tao et al (2014 Nonlinearity 27 2409-16) and Zlatoš (2012 J. Math. Pures Appl. 98 89-102) for random dispersal Fisher-KPP equations with quite general time and/or space dependence, and in Coville et al (2013 Ann. Inst. Henri Poincare 30 179-223), Rawal et al (2015 Discrete Contin. Dyn. Syst. 35 1609-40) and Shen and Zhang (2012 Comm. Appl. Nonlinear Anal. 19 73-101) for nonlocal dispersal Fisher-KPP equations with time and/or space periodic dependence. The stability result established in this paper implies that the transition waves obtained in many of the above mentioned papers are asymptotically stable for well-fitted perturbation. Up to the author�

Drop of coherence of the lower kilo-Hz QPO in neutron stars: Is there a link with the innermost stable circular orbit?

NASA Astrophysics Data System (ADS)

Barret, D.; Olive, J.-F.; Miller, M. Coleman

2005-11-01

Using all available archival data from the Rossi X-ray Timing Explorer (RXTE), we follow the frequency of the kilo-Hz QPOs in three low luminosity neutron star low mass X-ray binaries; namely 4U 1636-536, 4U 1608-522, and 4U 1735-44. Following earlier work by Barret et al. (2005a,b), we focus our analysis on the lower kilo-Hz QPO, for which we study the dependency of its quality factor (Q=\

Benefit from NASA

NASA Image and Video Library

1995-01-01

In this photograph, Sandra Rossi user her NASA-developed prosthesis for the first time. Derived from foam insulation technology used to protect the Space Shuttle External Tank from excessive heat, FAB/CAD, a subsidiary of the Harshberger Prosthetic and Orthotic Center, utilized the technology to replace the heavy, fragile plaster they used to produce master molds for prosthetics. The new material was lighter, cheaper and easier to manufacture than plaster, resulting in lower costs to the customer.

Prosthesis Material

NASA Technical Reports Server (NTRS)

1995-01-01

In this photograph, Sandra Rossi user her NASA-developed prosthesis for the first time. Derived from foam insulation technology used to protect the Space Shuttle External Tank from excessive heat, FAB/CAD, a subsidiary of the Harshberger Prosthetic and Orthotic Center, utilized the technology to replace the heavy, fragile plaster they used to produce master molds for prosthetics. The new material was lighter, cheaper and easier to manufacture than plaster, resulting in lower costs to the customer.

Prostate Cancer Evaluation: Design, Synthesis and Evaluation of Novel Enzyme-Activated Proton MRI Contrast Agents

DTIC Science & Technology

2010-10-01

93. Becker E . M., Lovejoy D. B., Greer J. M., Watts R., Richardson D. R. (2003). Identification of the di-pyridyl ketone isonicotinoyl hydrazone (PKIH...6 D1$0(2)5(63216,%/(3(5621 D5(3257 E $%675$&7 F7+,63$*( /,0,7$7,212) $%675$&7 6WDQGDUG)RUP 5HY 3UHVFULEHG... E \\$16,6WG= 7KHSXEOLFUHSRUWLQJEXUGHQIRUWKLVFROOHFWLRQRI LQIRUPDWLRQLVHVWLPDWHGWRDYHUDJHKRXUSHUUHVSRQVH LQFOXGLQJWKHWLPH

Were Increased Closed Seclusions the Result of a Reinforcer-Abolishing Effect?

PubMed

Poling, Alan

2013-01-01

In recent years, the motivating operation (MO) concept and the terms associated with it, including "reinforcer-abolishing effect" (Laraway, Snycerski, Michael, & Poling, 2003), have been widely used in the behavior-analytic literature (Laraway, Snycerski, Olson, Becker, & Poling, in press) and elsewhere (Lotfizadeh, Edwards, & Poling, 2013). MOs are changes in the environment that alter the reinforcing effectiveness of designated classes of stimuli, such as food or water.

Leadership Principles Leveraged from Key Civil War Battles

DTIC Science & Technology

2017-05-15

primary research method utilized for this research paper. The purpose of the literature review was to find data and information regarding analysis of...review was the primary research method utilized for this research paper. The purpose of the literature review was to find data and information...data generation and analysis. Organizational Research Methods , 15 (2), 316-335. Becker, W.S. & Burke, M.J. (2014). Instructional staff

The Effect of Advanced Education on the Retention and Promotion of Army Officers

DTIC Science & Technology

2007-03-01

2 Ronal G.Ehrenberg, Robert S.Smith, Modern Labor Economics , Theory and Public Policy, (New York: Pearson Education, Inc, 2006...Ehrenberg and Robert S. Smith, Modern Labor Economics , 9th ed. (New York: Pearson Education, Inc, 2006). 30 G S Becker, Human Capital: A Theoretical...their jobs. As a 32 Ronald G. Ehrenberg and Robert S. Smith, Modern Labor Economics , 9th ed. (New

Key for European species of the Cheilosia proxima group (Diptera, Syrphidae) with a description of a new species

PubMed Central

Vujić, Ante; Radenković, Snežana; Trifunov, Sonja; Nikolić, Tijana

2013-01-01

Abstract A new hoverfly species, Cheilosia barbafacies Vujić & Radenković sp. n. (Diptera, Syrphidae), is described and distinguished from the closely related species Cheilosia pascuorum Becker, 1894, based on material collected from the mountains of the Balkan Peninsula. Diagnostic characteristics and an identification key for the members of the proxima group of Cheilosia s. str., including the new taxon, are provided. PMID:23653524

Calculation of the equilibrium distribution for a deleterious gene by the finite Fourier transform.

PubMed

Lange, K

1982-03-01

In a population of constant size every deleterious gene eventually attains a stochastic equilibrium between mutation and selection. The individual probabilities of this equilibrium distribution can be computed by an application of the finite Fourier transform to an appropriate branching process formula. Specific numerical examples are discussed for the autosomal dominants, Huntington's chorea and chondrodystrophy, and for the X-linked recessive, Becker's muscular dystrophy.

Does Aid to Families with Dependent Children Displace Familial Assistance?

DTIC Science & Technology

1996-07-01

brief discussion of theoretical models of familial transfers that predict displacement as well as previous empirical studies that have examined this...summarizes the findings. Models of Familial Transfers, and Previous Empirical Studies of Displacement Theoretical Models Several models of private...transfer behavior have been posed, including altruism, exchange, and "warm glow." The altruism model (Becker, 1974; Barro, 1974) states, in terms of

The Role of a Novel Myosin Isoform in Prostate Cancer Metastasis

DTIC Science & Technology

2013-10-01

of unconventional myosin function and targeting, Annu. Rev. Cell Dev. Biol. 27 (2011) 133–155. [42] W. Kliche, S. Fujita- Becker , M. Kollmar, D.J...tissue-specific diseases (laminopathies), including Emery–Dreifuss muscular dystrophy , Dunnigan-type familial partial lipodystrophy (FPLD), and...structure of the C-terminal domain of lamin A/C, mutated in muscular dystrophies , cardiomyopathy, and partial lipodystrophy. Structure 10, 811–823