Functions of dystrophin and dystrophin associated proteins
- PMID: 9330892
- DOI: 10.1097/00019052-199710000-00014
Functions of dystrophin and dystrophin associated proteins
Abstract
Dystrophin is a protein product of the X-linked gene mutation that is responsible for Duchenne and Becker muscular dystrophies. The protein binds actin and associates with dystrophin-glycoprotein complex to link the cytoskeleton to the extracellular matrix. Defects in the components of the dystrophin-glycoprotein complex are responsible for several phenotypes of muscular dystrophy.
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