The pathological damage in Duchenne muscular dystrophy may be due to increased intracellular OXY-radical generation caused by the absence of dystrophin and subsequent alterations in Ca2+ metabolism.
Recent advances in the genetic and molecular pathogenesis of Duchenne muscular dystrophy and the evidence suggesting a role for oxygen free radicals (oxy-radicals) in the development of this disease are reviewed. In addition, we outline a working of hypothesis as to how disruptions in intracellular Ca2+ homeostasis within the dystrophic cell may initiate cycles of increased oxy-radical fluxes within these cells, leading to intracellular oxidative damage.
Baker
M S
MS
Department of Biochemistry, Monash University, Clayton, Australia.
Austin
L
L
D002118:Calcium / Q000378:metabolism; D016189:Dystrophin; D005609:Free Radicals; D006801:Humans; D008954:Models, Biological; D009124:Muscle Proteins / Q000378:metabolism; D009136:Muscular Dystrophies / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D010100:Oxygen / Q000378:metabolism
D016189:Dystrophin; D005609:Free Radicals; D009124:Muscle Proteins; D010100:Oxygen; D002118:Calcium