Key result
Dystrophin-deficient mice showed poor passive myocyte compliance and reduced survival during stress testing, whereas sarcoglycan-deficient mice had normal compliance but increased cardiac fibrosis.
Population
Mouse models of Duchenne muscular dystrophy, LGMD-2C, and LGMD-2F
Comparison
Genetic deficiency of dystrophin, γ-sarcoglycan… vs Control mice
Design
Preclinical
Authors
Loading...
Should not yet alter clinical management of dystrophic cardiomyopathy; leaves open distinct dystrophin versus sarcoglycan mechanisms for targeted research.
Dystrophin, but not sarcoglycans, protects cardiac myocytes against mechanical damage, highlighting distinct pathophysiological mechanisms underlying different models of inherited dystrophic cardiomyopathy.
Townsend et al. (2011) studied Duchenne muscular dystrophy and limb girdle muscular dystrophy. Dystrophin or sarcoglycan deficiency vs. Control mice was evaluated on Cardiac myocyte compliance, survival during dobutamine stress testing, and cardiac function. Dystrophin-deficient mice showed poor passive myocyte compliance and reduced survival during stress testing, whereas sarcoglycan-deficient mice had normal compliance but increased cardiac fibrosis.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: