Oxidative stress and the pathogenesis of muscular dystrophies.
The muscular dystrophies represent a diverse group of diseases differing in underlying genetic basis, age of onset, mode of inheritance, and severity of progression, but they share certain common pathologic features. Most prominent among these features is the necrotic degeneration of muscle fibers. Although the genetic basis of many of the dystrophies has been known for over a decade and new disease genes continue to be discovered, the pathogenetic mechanisms leading to muscle cell death in the dystrophies remain a mystery. This review focuses on the oxidative stress theory, which states that the final common pathway of muscle cell death in these diseases involves oxidative damage.
Rando
Thomas A
TA
Neurology Service and GRECC, VA Palo Alto Health Care System, Palo Alto, California, USA.
D017209:Apoptosis; D016189:Dystrophin / Q000172:deficiency; D006801:Humans; D008562:Membrane Glycoproteins / Q000378:metabolism; D018485:Muscle Fibers, Skeletal / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism*; D009336:Necrosis; D018384:Oxidative Stress
D016189:Dystrophin; D008562:Membrane Glycoproteins