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Dystrophin–glycoprotein complex: Its role in the molecular pathogenesis of muscular dystrophies
Author(s) -
Matsumura Kiichiro,
Campbell Kevin P.
Publication year - 1994
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880170103
Subject(s) - dystroglycan , dystrophin , duchenne muscular dystrophy , muscular dystrophy , sarcolemma , pathogenesis , extracellular matrix , laminin , biology , glycoprotein , utrophin , microbiology and biotechnology , genetics , medicine , immunology , myocyte
Abstract Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, is associated with a large oligomeric complex of sarcolemmal glycoproteins, including dystroglycan which provides a linkage to the extracellular matrix component, laminin. In patients with DMD, the absence of dystrophin leads to the loss in all of the dystrophin‐associated proteins, causing the disruption of the linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. This may render the sarcolemma vulnerable to physical stress. These recent developments in the research concerning the function of the dystrophin–glycoprotein complex pave a way for the better understanding of the pathogenesis of muscular dystrophies. © 1994 John Wiley & Sons, Inc.