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 …

Inflammation and cardiac dysfunction during sepsis, muscular dystrophy, and myocarditis

Y Li, S Ge, Y Peng, X Chen - Burns & trauma, 2013 - academic.oup.com
Inflammation plays an important role in cardiac dysfunction under different situations. Acute
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 …

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 …

[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 …

[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 …

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 …

Nanotopography-responsive myotube alignment and orientation as a sensitive phenotypic biomarker for Duchenne Muscular Dystrophy

B Xu, A Magli, Y Anugrah, SJ Koester, RCR Perlingeiro… - Biomaterials, 2018 - Elsevier
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 …

[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 …

[HTML][HTML] Lifespan analysis of dystrophic mdx fast-twitch muscle morphology and its impact on contractile function

L Kiriaev, S Kueh, JW Morley, KN North… - Frontiers in …, 2021 - frontiersin.org
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 …