How does dystrophin deficiency lead to muscle degeneration?--evidence from the mdx mouse

Neuromuscul Disord. 1995 Nov;5(6):445-56. doi: 10.1016/0960-8966(95)00001-4.


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.

Publication types

  • Review

MeSH terms

  • Animals
  • Dystrophin / deficiency*
  • Mice
  • Mice, Inbred mdx / metabolism*
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / physiopathology*
  • Muscular Dystrophy, Animal / genetics
  • Muscular Dystrophy, Animal / metabolism*
  • Muscular Dystrophy, Animal / physiopathology*


  • Dystrophin