How does dystrophin deficiency lead to muscle degeneration?--evidence from the mdx mouse.
The mdx mouse has a defect in the same gene as boys with Duchenne muscular dystrophy, which results in the absence of the protein product, dystrophin. A large number of recent studies have used the mdx mouse model to examine the potential role of dystrophy in normal muscle and the mechanisms by which dystrophin-deficiency leads to myopathy. This review discusses critically the results of these studies and their relevance to understanding the mechanisms by which dystrophin-deficiency leads to muscle necrosis.
McArdle
A
A
Muscle Research Centre, Department of Medicine, University of Liverpool, UK.
Edwards
R H
RH
Jackson
M J
MJ
D000818:Animals; D016189:Dystrophin / Q000172:deficiency*; D051379:Mice; D018101:Mice, Inbred mdx / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000503:physiopathology*; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology*
D016189:Dystrophin