Key result
Overexpressing wild-type myotilin worsens muscle degeneration and myofibrillar aggregation versus single-transgenic mutants in LGMD1A mice.
Why the study?
The effect of overexpression of wild-type myotilin on myopathology in an LGMD1A mouse model was not previously examined.
Does overexpression of wild-type myotilin worsen myopathology in an LGMD1A mouse model?
Population
LGMD1A mutant transgenic mice
Comparison
Double-transgenic mice overexpressing wild-type myotilin vs single-transgenic mutant mice
Design
Preclinical study in mouse model
Authors
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Supports myotilin reduction as potential LGMD1A strategy; leaves open translation beyond this mouse model.
Does overexpression of wild-type myotilin worsen myopathology in an LGMD1A mouse model?
Overexpression of wild-type myotilin exacerbates myopathology in an LGMD1A mouse model, suggesting that lowering total myotilin levels may be a therapeutic strategy.
Garvey et al. (2008) studied Limb-girdle muscular dystrophy type 1A (LGMD1A). Overexpression of wild-type myotilin vs. Single-transgenic mutant mice was evaluated on Muscle degeneration, myofibrillar aggregation, and onset of aggregation. Overexpression of wild-type myotilin in an LGMD1A mouse model resulted in more severe muscle degeneration and enhanced myofibrillar aggregation compared to single-transgenic mutant mice.
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