Just say NO to muscle degeneration?
The devastating muscle degeneration characteristic of Duchenne muscular dystrophy is caused by mutations in the gene encoding dystrophin. The dystrophin complex has two functions: a structural role in maintaining sarcolemmal integrity during contraction and a scaffolding function that recruits signaling proteins such as neuronal nitric oxide synthase to the membrane. New studies indicate that transgenic restoration of nitric oxide (NO) production in the mdx dystrophic mouse improves muscle pathology. Although NO-mediated killing of inflammatory cells might be involved, other mechanisms are also possible. These results point to the therapeutic potential of manipulating the signaling activity of the dystophin complex as a way to ameliorate the progression of muscle degeneration.
Froehner
Stanley C
SC
D000818:Animals; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism*; D015316:Genetic Therapy; D006801:Humans; D051379:Mice; D008954:Models, Biological; D009132:Muscles / Q000378:metabolism; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D009569:Nitric Oxide / Q000378:metabolism*; D019001:Nitric Oxide Synthase / Q000235:genetics / Q000378:metabolism / Q000627:therapeutic use
D016189:Dystrophin; D009569:Nitric Oxide; D019001:Nitric Oxide Synthase