Circulation Research. 2004;94:1023-1031
doi: 10.1161/01.RES.0000126574.61061.25
doi: 10.1161/01.RES.0000126574.61061.25
© 2004 American Heart Association, Inc.
Reviews |
The Dystrophin Glycoprotein Complex
Signaling Strength and Integrity for the Sarcolemma
From the Department of Molecular Genetics and Cell Biology (K.A.L.), Department of Medicine (R.K., E.M.M.), and Department of Human Genetics (E.M.M.), University of Chicago, Ill.
Correspondence to E.M. McNally, MD, PhD, University of Chicago, 5841 S Maryland, MC6088, Room G611, Chicago, IL 60637. E-mail emcnally{at}medicine.bsd.uchicago.edu
This Review is part of a thematic series on Myocyte Intra- and Extrasarcomeric Structural Proteins, which includes the following articles:
The Giant Protein Titin: A Major Player in Myocardial Mechanics, Signaling, and Disease
The Dystrophin Glycoprotein Complex: Signaling Strength and Integrity for the Sarcolemma
Cardiac Myosin Binding Protein C: Its Role in Physiology and Disease
David Kass Editor
The dystrophin glycoprotein complex (DGC) is a specialization of cardiac and skeletal muscle membrane. This large multicomponent complex has both mechanical stabilizing and signaling roles in mediating interactions between the cytoskeleton, membrane, and extracellular matrix. Dystrophin, the protein product of the Duchenne and X-linked dilated cardiomyopathy locus, links cytoskeletal and membrane elements. Mutations in additional DGC genes, the sarcoglycans, also lead to cardiomyopathy and muscular dystrophy. Animal models of DGC mutants have shown that destabilization of the DGC leads to membrane fragility and loss of membrane integrity, resulting in degeneration of skeletal muscle and cardiomyocytes. Vascular reactivity is altered in response to primary degeneration in striated myocytes and arises from a vascular smooth muscle cell–extrinsic mechanism.
Key Words: dystrophin • sarcoglycan • membrane • cardiomyocyte • skeletal muscle
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|
|
|
|
|
|
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|
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|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
|
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|
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|
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|
|
|
|
|
|
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|
|
|
|
|
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|
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|
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