One arm is enough, two might actually be worse - When dystrophic muscle cells grow old

摘要

The quest for pathophysiological mechanisms of Duchenne muscular dystrophy (DMD), a common inherited wasting muscle disorder, continues. Even more than 25 years after introducing mdx mouse models, we don't have unifying hypotheses at hand to explain all pathophysiological findings arising from the absence of dystrophin, e.g. mechanical instability, disrupted Ca~(2+) homeostasis, altered ion channel function, activated proteolysis, deranged metabolic control, decreased force and increased fatigability. Disease haEmarks are continuous degenerative-regenerative cycles in muscle that might explain progressive force loss through tissue remodelling (Pastoret & Sebille, 1995).