An injury model myopathy mimicking dystrophy: implications regarding the function of dystrophin.
The pathogenesis of dystrophin deficient myopathies remains unknown. Rat and human muscles subjected to severe injury following repeated eccentric muscle actions demonstrate histopathological alterations which mimic a dystrophic process. Immunofluorescent histochemical examination of these injured muscles demonstrates a separation of proteoglycans of the basal lamina from the muscle plasma membrane, the identical histopathological alteration observed in Duchenne muscular dystrophy. These findings are consistent with the hypothesis that dystrophin is essential for maintenance of the structural integrity of the sarcolemma.
Stauber
W T
WT
Department of Physiology, West Virginia University, Morgantown 26506.
Fritz
V K
VK
Clarkson
P M
PM
Riggs
J E
JE
D000328:Adult; D000818:Animals; D004195:Disease Models, Animal; D016189:Dystrophin / Q000502:physiology*; D004558:Electric Stimulation; D006801:Humans; D009119:Muscle Contraction; D009132:Muscles / Q000293:injuries* / Q000473:pathology / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000209:etiology*; D009137:Muscular Dystrophy, Animal / Q000209:etiology / Q000503:physiopathology*; D051381:Rats; D011919:Rats, Inbred Strains
D016189:Dystrophin