title: Free radicals: a potential pathogenic mechanism in inherited muscular dystrophy.
abstract: Despite years of intensive work, the biochemical defect responsible for the pathogenesis of inherited muscular dystrophy has not been identified either in humans or animal models. This review examines evidence in support of the hypothesis that free radicals may be responsible for muscle degeneration in this disorder. A variety of cellular abnormalities noted in dystrophic muscles can be accounted for by free radical mediated damage. In addition, chemical by-products associated with free radical damage are found in dystrophic muscle tissue from humans and animals with this disease. Various enzymatic antioxidant systems can be enhanced as a normal cellular response to oxidative stress, and such changes are seen both in dystrophic muscle cells and certain other tissues of dystrophic animals. An increased level of free radical damage would follow from either: enhanced production of free radical species, or a deficient component of the cellular antioxidant system, such as vitamin E. The free radical hypothesis of muscular dystrophy can account for data supporting several alternative theories of the pathogenesis of this disease, as well as other observations which have not previously been explained.
mesh_terms: D002118:Calcium / Q000378:metabolism; D003094:Collagen / Q000378:metabolism; D005609:Free Radicals; D005997:Glycerylphosphorylcholine / Q000378:metabolism; D008566:Membranes / Q000502:physiology; D008833:Microcirculation; D009132:Muscles / Q000098:blood supply / Q000166:cytology; D009136:Muscular Dystrophies / Q000150:complications / Q000503:physiopathology*; D012701:Serotonin / Q000378:metabolism; D014811:Vitamin E Deficiency / Q000150:complications
chemical_list: D005609:Free Radicals; D012701:Serotonin; D005997:Glycerylphosphorylcholine; D003094:Collagen; D002118:Calcium