Key result
Combined alpha and epsilon sarcoglycan knockout in mice causes severe cardiomyopathy, revealing epsilon-sarcoglycan's compensatory role.
Population
Sgce-null mice and Sgca-;Sgce-null mice (lacking both α- and ε-sarcoglycans)
Comparison
Genetic knockout of Sgce and Sgca vs Wild-type phenotype / single knockout
Design
Preclinical
Authors
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Provides a mechanistic target for preventing cardiomyopathy in muscular dystrophy; leaves open whether upregulating epsilon.
Epsilon-sarcoglycan plays an important role in preventing cardiomyopathy in the setting of alpha-sarcoglycan deficiency.
Lancioni et al. (2011) studied Cardiomyopathy and muscular dystrophy. Combined deficiency of alpha and epsilon sarcoglycan (Sgca-;Sgce-null) vs. Sgce-null mice was evaluated on Development of muscular dystrophy and cardiomyopathy. Combined deficiency of alpha and epsilon sarcoglycan in mice resulted in progressive muscular dystrophy and severe cardiomyopathy, indicating epsilon-sarcoglycan compensates for alpha-sarcoglycan.
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