Reduced Na+ current in Purkinje fibers explains cardiac conduction defects and arrhythmias in Duchenne muscular dystrophy

J Ebner, P Uhrin, PL Szabo, A Kiss… - American Journal …, 2020 - journals.physiology.org
Cardiac arrhythmias significantly contribute to mortality in Duchenne muscular dystrophy
(DMD), a degenerative muscle disease triggered by mutations in the gene encoding for the …

Full-length dystrophin expression in half of the heart cells ameliorates β-isoproterenol-induced cardiomyopathy in mdx mice

Y Yue, JW Skimming, M Liu, T Strawn… - Human molecular …, 2004 - academic.oup.com
Gene therapy holds great promise for curing Duchenne muscular dystrophy (DMD), the most
common fatal inherited childhood muscle disease. Success of DMD gene therapy depends …

Corticortophin releasing factor 2 receptor agonist treatment significantly slows disease progression in mdx mice

RT Hinkle, FR Lefever, ET Dolan, DL Reichart… - BMC medicine, 2007 - Springer
Background Duchenne muscular dystrophy results from mutation of the dystrophin gene,
causing skeletal and cardiac muscle loss of function. The mdx mouse model of Duchenne …

Some dystrophy phenotypes of dystrophin‐deficient mdx mice are exacerbated by mild, repetitive daily stress

A Lindsay, J Holm, M Razzoli… - The FASEB …, 2021 - Wiley Online Library
Psychosocial stressors can cause physical inactivity, cardiac damage, and hypotension‐
induced death in the mdx mouse model of Duchenne muscular dystrophy (DMD). Because …

[HTML][HTML] Experimental models of duchenne muscular dystrophy: relationship with cardiovascular disease

V Ameen, LG Robson - The open cardiovascular medicine journal, 2010 - ncbi.nlm.nih.gov
Almost every boy that has Duchenne Muscular Dystrophy (DMD) will develop cardiac
problems. Whereas, it used to be respiratory problems that was the main cause of death in …

Ryanodine receptor dysfunction causes senescence and fibrosis in Duchenne dilated cardiomyopathy

M Souidi, J Resta, H Dridi, Y Sleiman… - Journal of Cachexia …, 2024 - Wiley Online Library
Background Duchenne muscular dystrophy (DMD) is an X‐linked disorder characterized by
progressive muscle weakness due to the absence of functional dystrophin. DMD patients …

Dystrophic cardiomyopathy: role of the cardiac myofilaments

TG George, LM Hanft, M Krenz, TL Domeier… - Frontiers in …, 2023 - frontiersin.org
Dystrophic cardiomyopathy arises from mutations in the dystrophin gene. Dystrophin forms
part of the dystrophin glycoprotein complex and is postulated to act as a membrane …

Contraction-induced loss of plasmalemmal electrophysiological function is dependent on the dystrophin glycoprotein complex

CW Baumann, A Lindsay, SR Sidky, JM Ervasti… - Frontiers in …, 2021 - frontiersin.org
Weakness and atrophy are key features of Duchenne muscular dystrophy (DMD).
Dystrophin is one of the many proteins within the dystrophin glycoprotein complex (DGC) …

Cardiac-specific expression of ΔH2-R15 mini-dystrophin normalized all electrocardiogram abnormalities and the end-diastolic volume in a 23-month-old mouse model …

NB Wasala, JH Shin, Y Lai, Y Yue, F Montanaro… - Human gene …, 2018 - liebertpub.com
Heart disease is a major health threat for Duchenne/Becker muscular dystrophy patients and
carriers. Expression of a 6–8 kb mini-dystrophin gene in the heart holds promise to change …

Gender influences cardiac function in the mdx model of duchenne cardiomyopathy

B Bostick, Y Yue, D Duan - Muscle & nerve, 2010 - Wiley Online Library
Aged mdx mice represent an important model for studying Duchenne cardiomyopathy.
Herein we compared the cardiac phenotypes of 22‐month‐old male and female mdx mice …