Menstuff® has information on Musculaar Degeneration.
Muscular degeneration in the absence of dystrophin is a
Duchenne muscular dystrophy (DMD) is a progressive degenerative
muscular disease that is due to mutations in the dystrophin gene.
Neither the function of dystrophin nor the physiopathology of the
disease have been clearly established yet. Several groups have
reported elevated calcium concentrations in the mdx mouse model of
DMD, but the effect of calcium levels on the progression of the
disease continues to be a matter of debate. Here, we show that, in
Caenorhabditis elegans, a gain-of-function mutation in the egl-19
calcium channel gene dramatically increases muscle degeneration in
dystrophin mutants. Conversely, RNAi-mediated inhibition of egl-19
function reduces muscle degeneration by half. Therefore, our results
demonstrate that calcium channel activity is a critical factor in the
progression of dystrophin-dependent muscle degeneration.
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