Abstract
Dilated cardiomyopathy is a serious and almost inevitable complication of Duchenne Muscular Dystrophy, a devastating and fatal disease of skeletal muscle resulting from the lack of functional dystrophin, a protein linking the cytoskeleton to the extracellular matrix. Ultimately, it leads to congestive heart failure and arrhythmias resulting from both cardiac muscle fibrosis and impaired function of the remaining cardiomyocytes. Here we summarize findings obtained in several laboratories, focusing on cellular mechanisms that result in degradation of cardiac functions in dystrophy.
Copyright © 2012 Elsevier Ltd. All rights reserved.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Arrhythmias, Cardiac / complications
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Arrhythmias, Cardiac / metabolism
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Arrhythmias, Cardiac / pathology
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Cardiomyopathy, Dilated / complications
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Cardiomyopathy, Dilated / genetics*
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Cardiomyopathy, Dilated / physiopathology
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Dystrophin / deficiency
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Dystrophin / genetics*
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Dystrophin / metabolism
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Heart Failure / complications
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Heart Failure / metabolism
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Heart Failure / pathology
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Humans
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Muscle, Skeletal / metabolism
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Muscle, Skeletal / physiopathology
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Muscular Dystrophy, Duchenne / complications
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Muscular Dystrophy, Duchenne / genetics*
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Muscular Dystrophy, Duchenne / physiopathology
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Myocytes, Cardiac / metabolism
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Myocytes, Cardiac / pathology*