Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy
DG Allen, NP Whitehead… - Physiological …, 2016 - journals.physiology.org
Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a
complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further …
complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further …
Inflammation and cardiac dysfunction during sepsis, muscular dystrophy, and myocarditis
Inflammation plays an important role in cardiac dysfunction under different situations. Acute
systemic inflammation occurring in patients with severe burns, trauma, and inflammatory …
systemic inflammation occurring in patients with severe burns, trauma, and inflammatory …
[HTML][HTML] Forelimb treatment in a large cohort of dystrophic dogs supports delivery of a recombinant AAV for exon skipping in Duchenne patients
C Le Guiner, M Montus, L Servais, Y Cherel… - Molecular Therapy, 2014 - cell.com
Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disorder caused by
mutations in the dystrophin gene, without curative treatment yet available. Our study …
mutations in the dystrophin gene, without curative treatment yet available. Our study …
Affinity proteomics within rare diseases: a BIO‐NMD study for blood biomarkers of muscular dystrophies
B Ayoglu, A Chaouch, H Lochmüller… - EMBO molecular …, 2014 - embopress.org
Despite the recent progress in the broad‐scaled analysis of proteins in body fluids, there is
still a lack in protein profiling approaches for biomarkers of rare diseases. Scarcity of …
still a lack in protein profiling approaches for biomarkers of rare diseases. Scarcity of …
[HTML][HTML] Prevalence and distribution of late gadolinium enhancement in a large population of patients with Duchenne muscular dystrophy: effect of age and left …
KN Hor, MD Taylor, HR Al-Khalidi, LH Cripe… - Journal of …, 2013 - Elsevier
Background Duchenne muscular dystrophy (DMD), an X-linked disorder affects
approximately 1 in 5000 males, is universally associated with heart disease. We previously …
approximately 1 in 5000 males, is universally associated with heart disease. We previously …
[HTML][HTML] Skeletal muscle fibrosis in the mdx/utrn+/-mouse validates its suitability as a murine model of Duchenne muscular dystrophy
KM Gutpell, WT Hrinivich, LM Hoffman - PloS one, 2015 - journals.plos.org
Various therapeutic approaches have been studied for the treatment of Duchenne muscular
dystrophy (DMD), but none of these approaches have led to significant long-term effects in …
dystrophy (DMD), but none of these approaches have led to significant long-term effects in …
Treatment of dystrophin cardiomyopathies
J Finsterer, L Cripe - Nature Reviews Cardiology, 2014 - nature.com
Abstract Treatment of cardiac disease in patients with dystrophinopathies substantially
improves outcomes. In this Review, we summarize and discuss findings from the past 20 …
improves outcomes. In this Review, we summarize and discuss findings from the past 20 …
Nanotopography-responsive myotube alignment and orientation as a sensitive phenotypic biomarker for Duchenne Muscular Dystrophy
Abstract Duchenne Muscular Dystrophy (DMD) is a fatal genetic disorder currently having no
cure. Here we report that culture substrates patterned with nanogrooves and functionalized …
cure. Here we report that culture substrates patterned with nanogrooves and functionalized …
[HTML][HTML] Angiogenesis as a novel therapeutic strategy for Duchenne muscular dystrophy through decreased ischemia and increased satellite cells
Y Shimizu-Motohashi, A Asakura - Frontiers in physiology, 2014 - frontiersin.org
Duchenne muscular dystrophy (DMD) is the most common hereditary muscular dystrophy
caused by mutation in dystrophin, and there is no curative therapy. Dystrophin is a protein …
caused by mutation in dystrophin, and there is no curative therapy. Dystrophin is a protein …
[HTML][HTML] Lifespan analysis of dystrophic mdx fast-twitch muscle morphology and its impact on contractile function
Duchenne muscular dystrophy is caused by the absence of the protein dystrophin from
skeletal muscle and is characterized by progressive cycles of necrosis/regeneration. Using …
skeletal muscle and is characterized by progressive cycles of necrosis/regeneration. Using …