Article
Myotilin overexpression enhances myopathology in the LGMD1A mouse model.
Muscle & nerve - 1 May 2008
Garvey Sean M, Liu Yutao, Miller Sara E, Hauser Michael A
Abstract excerpt
Missense mutations in the myotilin gene cause limb-girdle muscular dystrophy type 1A (LGMD1A). We set out to examine the effect of overexpression of wild-type myotilin in an LGMD1A mouse model by crossing wild-type and mutant transgenic mice. Compared to single-transgenic mutant mice, double-transgenic mice overexpressing myotilin showed more severe muscle degeneration, enhanced myofibrillar aggregation, and...
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