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https://www.readbyqxmd.com/read/27854211/n-terminal-%C3%AE-dystroglycan-%C3%AE-dg-n-a%C3%A2-potential-serum-biomarker-for-duchenne-muscular-dystrophy
#1
Kelly E Crowe, Guohong Shao, Kevin M Flanigan, Paul T Martin
BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a severe, progressive, neuromuscular disorder of childhood. While a number of serum factors have been identified as potential biomarkers of DMD, none, as yet, are proteins within the dystrophin-associated glycoprotein (DAG) complex. OBJECTIVE: We have developed an immobilized serum ELISA assay to measure the expression of a constitutively cleaved and secreted component of the DAG complex, the N-terminal domain of α dystroglycan (αDG-N), and assayed relative expression in serum from muscular dystrophy patients and normal controls...
May 27, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27854202/current-translational-research-and-murine-models-for-duchenne-muscular-dystrophy
#2
Merryl Rodrigues, Yusuke Echigoya, So-Ichiro Fukada, Toshifumi Yokota
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscle degeneration. Mutations in the DMD gene result in the absence of dystrophin, a protein required for muscle strength and stability. Currently, there is no cure for DMD. Since murine models are relatively easy to genetically manipulate, cost effective, and easily reproducible due to their short generation time, they have helped to elucidate the pathobiology of dystrophin deficiency and to assess therapies for treating DMD...
March 3, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27836895/lifelong-quercetin-enrichment-and-cardioprotection-in-mdx-utrn-ice
#3
Christopher Ballmann, Thomas Denney, Ronald J Beyers, Tiffany S Quindry, Matthew Romero, Rajesh H Amin, Joshua T Selsby, John C Quindry
Duchenne Muscular Dystrophy (DMD) is associated with progressive cardiac pathology, however, the SIRT1/PGC1-α activator quercetin may cardioprotect dystrophic hearts. We tested the extent to which long term 0.2% dietary quercetin enrichment attenuates dystrophic cardiopathology in Mdx/Utrn(+/-) mice. At 2 months, Mdx/Utrn(+/-) mice were fed quercetin enriched (Mdx/Utrn(+/-)-Q) or control diet (Mdx/Utrn(+/-)) for 8 months. Control C57BL/10 (C57) animals were fed a control diet for 10 months. Cardiac function was quantified by MRI at 2 and 10 months...
November 11, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27822449/similar-efficacy-from-specific-and-non-specific-mineralocorticoid-receptor-antagonist-treatment-of-muscular-dystrophy-mice
#4
Jeovanna Lowe, Kyle T Floyd, Neha Rastogi, Eric J Schultz, Jessica A Chadwick, Sarah A Swager, Jonathan G Zins, Feni K Kadakia, Suzanne Smart, Elise P Gomez-Sanchez, Celso E Gomez-Sanchez, Subha V Raman, Paul M L Janssen, Jill A Rafael-Fortney
BACKGROUND: Combined treatment with an angiotensin-converting enzyme inhibitor and a mineralocorticoid receptor (MR) antagonist improved cardiac and skeletal muscle function and pathology in a mouse model of Duchenne muscular dystrophy. MR is present in limb and respiratory skeletal muscles and functions as a steroid hormone receptor. OBJECTIVE: The goals of the current study were to compare the efficacy of the specific MR antagonist eplerenone with the non-specific MR antagonist spironolactone, both in combination with the angiotensin-converting enzyme inhibitor lisinopril...
2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27763556/structural-interface-forms-and-their-involvement-in-stabilization-of-multidomain-proteins-or-protein-complexes
#5
Jacek Dygut, Barbara Kalinowska, Mateusz Banach, Monika Piwowar, Leszek Konieczny, Irena Roterman
The presented analysis concerns the inter-domain and inter-protein interface in protein complexes. We propose extending the traditional understanding of the protein domain as a function of local compactness with an additional criterion which refers to the presence of a well-defined hydrophobic core. Interface areas in selected homodimers vary with respect to their contribution to share as well as individual (domain-specific) hydrophobic cores. The basic definition of a protein domain, i.e., a structural unit characterized by tighter packing than its immediate environment, is extended in order to acknowledge the role of a structured hydrophobic core, which includes the interface area...
October 18, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27750047/bgp-15-improves-aspects-of-the-dystrophic-pathology-in-mdx-and-dko-mice-with-differing-efficacies-in-heart-and-skeletal-muscle
#6
Tahnee L Kennedy, Kristy Swiderski, Kate T Murphy, Stefan M Gehrig, Claire L Curl, Chanchal Chandramouli, Mark A Febbraio, Lea M D Delbridge, René Koopman, Gordon S Lynch
Duchenne muscular dystrophy is a severe and progressive striated muscle wasting disorder that leads to premature death from respiratory and/or cardiac failure. We have previously shown that treatment of young dystrophic mdx and dystrophin/utrophin null (dko) mice with BGP-15, a coinducer of heat shock protein 72, ameliorated the dystrophic pathology. We therefore tested the hypothesis that later-stage BGP-15 treatment would similarly benefit older mdx and dko mice when the dystrophic pathology was already well established...
October 14, 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27734557/small-fractions-of-muscular-dystrophy-embryonic-stem-cells-yield-severe-cardiac-and-skeletal-muscle-defects-in-adult-mouse-chimeras
#7
J Patrick Gonzalez, Sergii Kyrychenko, Viktoriia Kyrychenko, Joel S Schneider, Celine J Granier, Eric Himelman, Kevin C Lahey, Qingshi Zhao, Ghassan Yehia, Yuan-Xiang Tao, Mantu Bhaumik, Natalia Shirokova, Diego Fraidenraich
Duchenne muscular dystrophy (DMD) is characterized by the loss of the protein dystrophin, leading to muscle fragility, progressive weakening, and susceptibility to mechanical stress. Although dystrophin-negative mdx mouse models have classically been used to study DMD, phenotypes appear mild compared to patients. As a result, characterization of muscle pathology, especially in the heart, has proven difficult. We report that injection of mdx embryonic stem cells (ESCs) into Wild Type blastocysts produces adult mouse chimeras with severe DMD phenotypes in the heart and skeletal muscle...
October 13, 2016: Stem Cells
https://www.readbyqxmd.com/read/27651778/mirna-targeted-signaling-pathway-in-the-early-stage-of-denervated-fast-and-slow-muscle-atrophy
#8
Gang Li, Qing-Shan Li, Wen-Bin Li, Jian Wei, Wen-Kai Chang, Zhi Chen, Hu-Yun Qiao, Ying-Wei Jia, Jiang-Hua Tian, Bing-Sheng Liang
Denervation often results in skeletal muscle atrophy. Different mechanisms seem to be involved in the determination between denervated slow and fast skeletal muscle atrophy. At the epigenetic level, miRNAs are thought to be highly involved in the pathophysiological progress of denervated muscles. We used miRNA microarrays to determine miRNA expression profiles from a typical slow muscle (soleus muscle) and a typical fast muscle (tibialis anterior muscle) at an early denervation stage in a rat model. Results showed that miR-206, miR-195, miR-23a, and miR-30e might be key factors in the transformation process from slow to fast muscle in denervated slow muscles...
August 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27638889/independent-variability-of-microtubule-perturbations-associated-with-dystrophinopathy
#9
Joseph J Belanto, John T Olthoff, Tara L Mader, Christopher M Chamberlain, D'anna M Nelson, Preston M McCourt, Dana M Talsness, Gregg G Gunderson, Dawn A Lowe, James M Ervasti
Absence of the protein dystrophin causes Duchenne muscular dystrophy. Dystrophin directly binds to microtubules in vitro, and its absence in vivo correlates with disorganization of the subsarcolemmal microtubule lattice, increased detyrosination of α-tubulin, and altered redox signaling. We previously demonstrated that the dystrophin homologue utrophin neither binds microtubules in vitro nor rescues microtubule lattice organization when overexpressed in muscles of dystrophin-deficient mdx mice. Here, we fine-mapped the dystrophin domain necessary for microtubule binding to spectrin-like repeats 20-22...
September 16, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27506543/tadalafil-treatment-delays-the-onset-of-cardiomyopathy-in-dystrophin-deficient-hearts
#10
David W Hammers, Margaret M Sleeper, Sean C Forbes, Ai Shima, Glenn A Walter, H Lee Sweeney
BACKGROUND: Cardiomyopathy is a leading cause of mortality among Duchenne muscular dystrophy patients and lacks effective therapies. Phosphodiesterase type 5 is implicated in dystrophic pathology, and the phosphodiesterase type 5 inhibitor tadalafil has recently been studied in a clinical trial for Duchenne muscular dystrophy. METHODS AND RESULTS: Tadalafil was evaluated for the prevention of cardiomyopathy in the mdx mouse and golden retriever muscular dystrophy dog models of Duchenne muscular dystrophy...
2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27485975/protein-anchoring-therapy-of-biglycan-for-mdx-mouse-model-of-duchenne-muscular-dystrophy
#11
Mikako Ito, Yuka Ehara, Shin Li, Kosuke Inada, Kinji Ohno
Duchenne muscular dystrophy (DMD) is a devastating muscle disease caused by loss-of-function mutations in <i>DMD</i> encoding dystrophin. No rational therapy is currently available. Utrophin is a paralog of dystrophin and is highly expressed at the neuromuscular junction. In <i>mdx</i> mice, utrophin is naturally upregulated throughout the muscle fibers, which mitigates muscular dystrophy. We previously reported the protein-anchoring therapy, in which a recombinant extracellular matrix protein is delivered to and anchored to a specific target using its proprietary binding domains...
August 2, 2016: Human Gene Therapy
https://www.readbyqxmd.com/read/27236198/differentiation-related-glycan-epitopes-identify-discrete-domains-of-the-muscle-glycocalyx
#12
Brian J McMorran, Francis E McCarthy, Elizabeth M Gibbs, Mabel Pang, Jamie L Marshall, Alison V Nairn, Kelley W Moremen, Rachelle H Crosbie-Watson, Linda G Baum
The neuromuscular junction (NMJ) is enriched with glycoproteins modified with N-acetylgalactosamine (GalNAc) residues, and four nominally GalNAc-specific plant lectins have historically been used to identify the NMJ and the utrophin-glycoprotein complex (UGC). However, little is known about the specific glycan epitopes on skeletal muscle that are bound by these lectins, the glycoproteins that bear these epitopes, or how creation of these glycan epitopes is regulated. Here we profile changes in cell surface glycosylation during muscle cell differentiation, and identify distinct differences in the binding preferences of GalNAc specific lectins, Wisteria floribunda agglutinin (WFA), Vicia villosa agglutinin (VVA), soybean agglutinin (SBA), and Dolichos biflorus agglutinin (DBA)...
May 28, 2016: Glycobiology
https://www.readbyqxmd.com/read/27183436/inhibition-of-human-glioma-cell-proliferation-caused-by-knockdown-of-utrophin-using-a-lentivirus-mediated-system
#13
Shang-Hang Shen, Ning Yu, Hao Xu, Xi-Yao Liu, Guo-Wei Tan, Zhan-Xiang Wang
BACKGROUND: Glioma is the most devastating brain tumor worldwide. Previous studies showed that UTRN (utrophin) was related to cancers, but its role in glioma cells remains uncovered. MATERIALS AND METHODS: RNAi was used to knockdown UTRN in U251 cells using lentivirus system. The knockdown efficiency was validated by real-time quantitative PCR. Cell proliferation, cell cycle, and apoptosis progression were determined by MTT, colony formation analysis, and flow cytometry analysis...
May 2016: Cancer Biotherapy & Radiopharmaceuticals
https://www.readbyqxmd.com/read/27069119/functional-characterization-of-orbicularis-oculi-and-extraocular-muscles
#14
Marijana Sekulic-Jablanovic, Nina D Ullrich, David Goldblum, Anja Palmowski-Wolfe, Francesco Zorzato, Susan Treves
The orbicularis oculi are the sphincter muscles of the eyelids and are involved in modulating facial expression. They differ from both limb and extraocular muscles (EOMs) in their histology and biochemistry. Weakness of the orbicularis oculi muscles is a feature of neuromuscular disorders affecting the neuromuscular junction, and weakness of facial muscles and ptosis have also been described in patients with mutations in the ryanodine receptor gene. Here, we investigate human orbicularis oculi muscles and find that they are functionally more similar to quadriceps than to EOMs in terms of excitation-contraction coupling components...
May 2016: Journal of General Physiology
https://www.readbyqxmd.com/read/27055247/safety-tolerability-and-pharmacokinetics-of-smt-c1100-a-2-arylbenzoxazole-utrophin-modulator-following-single-and-multiple-dose-administration-to-pediatric-patients-with-duchenne-muscular-dystrophy
#15
MULTICENTER STUDY
Valeria Ricotti, Stefan Spinty, Helen Roper, Imelda Hughes, Bina Tejura, Neil Robinson, Gary Layton, Kay Davies, Francesco Muntoni, Jonathon Tinsley
PURPOSE: SMT C1100 is a utrophin modulator being evaluated as a treatment for Duchenne muscular dystrophy (DMD). This study, the first in pediatric DMD patients, reports the safety, tolerability and PK parameters of single and multiple doses of SMT C1100, as well as analyze potential biomarkers of muscle damage. METHODS: This multicenter, Phase 1 study enrolled 12 patients, divided equally into three groups (A-C). Group A were given 50 mg/kg on Days 1 and 11, and 50 mg/kg bid on Days 2 to 10...
2016: PloS One
https://www.readbyqxmd.com/read/27037492/characterization-of-neuromuscular-synapse-function-abnormalities-in-multiple-duchenne-muscular-dystrophy-mouse-models
#16
Elizabeth M van der Pijl, Maaike van Putten, Erik H Niks, Jan J G M Verschuuren, Annemieke Aartsma-Rus, Jaap J Plomp
Duchenne muscular dystrophy (DMD) is an X-linked myopathy caused by dystrophin deficiency. Dystrophin is present intracellularly at the sarcolemma, connecting actin to the dystrophin-associated glycoprotein complex. Interestingly, it is enriched postsynaptically at the neuromuscular junction (NMJ), but its synaptic function is largely unknown. Utrophin, a dystrophin homologue, is also concentrated at the NMJ, and upregulated in DMD. It is possible that the absence of dystrophin at NMJs in DMD causes neuromuscular transmission defects that aggravate muscle weakness...
June 2016: European Journal of Neuroscience
https://www.readbyqxmd.com/read/26974331/correlation-of-utrophin-levels-with-the-dystrophin-protein-complex-and-muscle-fibre-regeneration-in-duchenne-and-becker-muscular-dystrophy-muscle-biopsies
#17
Narinder Janghra, Jennifer E Morgan, Caroline A Sewry, Francis X Wilson, Kay E Davies, Francesco Muntoni, Jonathon Tinsley
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...
2016: PloS One
https://www.readbyqxmd.com/read/26966179/cathepsin-s-contributes-to-the-pathogenesis-of-muscular-dystrophy-in-mice
#18
Andoria Tjondrokoesoemo, Tobias G Schips, Michelle A Sargent, Davy Vanhoutte, Onur Kanisicak, Vikram Prasad, Suh-Chin J Lin, Marjorie Maillet, Jeffery D Molkentin
Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the gene encoding dystrophin. Loss of dystrophin protein compromises the stability of the sarcolemma membrane surrounding each muscle cell fiber, leading to membrane ruptures and leakiness that induces myofiber necrosis, a subsequent inflammatory response, and progressive tissue fibrosis with loss of functional capacity. Cathepsin S (Ctss) is a cysteine protease that is actively secreted in areas of tissue injury and ongoing inflammation, where it participates in extracellular matrix remodeling and healing...
May 6, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/26851826/concordant-utrophin-upregulation-in-phenotypically-discordant-dmd-bmd-brothers
#19
Mariz Vainzof, Leticia Feitosa, Marta Canovas, Danielle Ayub-Guerrieri, Rita de Cássia M Pavanello, Mayana Zatz
Utrophin expression was investigated in two phenotypically discordant Duchenne muscular dystrophy half-brothers. The youngest was wheelchair-bound at age 9, while his mildly affected older brother was able to walk without difficulties at age 15. DNA analysis revealed an out-of-frame exon 2 duplication in the DMD gene, associated with muscle dystrophin protein deficiency. Utrophin localization and quantity was analyzed and compared in both sibs to verify whether this could explain the milder phenotype of the older brother...
March 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/26744329/genetic-overexpression-of-serpina3n-attenuates-muscular-dystrophy-in-mice
#20
Andoria Tjondrokoesoemo, Tobias Schips, Onur Kanisicak, Michelle A Sargent, Jeffery D Molkentin
Muscular dystrophy (MD) is associated with mutations in genes that stabilize the myofiber plasma membrane, such as through the dystrophin-glycoprotein complex (DGC). Instability of this complex or defects in membrane repair/integrity leads to calcium influx and myofiber necrosis leading to progressive dystrophic disease. MD pathogenesis is also associated with increased skeletal muscle protease levels and activity that could augment weakening of the sarcolemma through greater degradation of cellular attachment complexes...
March 15, 2016: Human Molecular Genetics
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