肌营养不良蛋白及肌营养不良蛋白相关蛋白的功能。
Functions of dystrophin and dystrophin associated proteins.
作者信息
Michalak M, Opas M
机构信息
Department of Biochemistry, University of Alberta, Edmonton, Canada.
出版信息
Curr Opin Neurol. 1997 Oct;10(5):436-42. doi: 10.1097/00019052-199710000-00014.
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.
摘要
肌营养不良蛋白是X连锁基因突变的蛋白质产物，该基因突变会导致杜氏和贝克型肌营养不良症。这种蛋白质与肌动蛋白结合，并与肌营养不良蛋白-糖蛋白复合物相关联，从而将细胞骨架与细胞外基质连接起来。肌营养不良蛋白-糖蛋白复合物成分的缺陷是导致几种肌营养不良症表型的原因。
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