Dystroglycan: important player in skeletal muscle and beyond - Review

被引：65

作者：

Cohn, RD ^{[1
]}

机构：

[1] Johns Hopkins Univ Hosp, Childrens Ctr, McKusick Nathans Inst Genet Med, Baltimore, MD 21287 USA

来源：

NEUROMUSCULAR DISORDERS | 2005年 / 15卷 / 03期

关键词：

dystroglycan; skeletal muscle; muscular dystrophy; neuronal migration disorders;

D O I：

10.1016/j.nmd.2004.11.005

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Dystroglycan is a transmembrane protein that connects the extracellular matrix to the cytoskeleton. Given the ubiquitous tissue expression of dystroglycan, different functional roles in various organ systems have been characterized during the-past decade. More recently, aberrant glycosylation of dystroglycan has been identified as a novel pathogenetic mechanism in several forms of congenital and late onset muscular dystrophy syndromes. The current review summarizes the recent scientific achievements as they relate to the function of dystroglycan under normal and pathophysiological conditions. (c) 2004 Elsevier B.V. All rights reserved.

引用

页码：207 / 217

页数：11

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