Stretch-activated channels in muscular dystrophy

Duchenne muscular dystrophy is a devastating genetic disease caused by the absence of the protein dystrophin. The pathogenic mechanisms are complex and there is currently no cure for the disease. Dystrophic muscles are susceptible to damage induced by muscle contractions, and excessive calcium influx is thought to trigger a pathological cascade of events that ultimately results in muscle fiber degeneration. Stretch-activated (or mechanosensitive) ion channels have been proposed to be implicated in the regulation of calcium entry in dystrophic muscle. Although the precise molecular nature of the channels is unclear, emerging evidence suggests that these channels are mediators of altered calcium homeostasis, causing muscle damage and progressive muscle weakness in muscular dystrophy.