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Neuromuscul Disord. 1995 Nov;5(6):445-56.

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

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  • 1Muscle Research Centre, Department of Medicine, University of Liverpool, UK.


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.

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