| Literature DB >> 18562127 |
Qiang Sun Zheng1, Wan Gang Guo, Zi Fan Lu, Xiao Qin Shi, Fei Fei Su, Hua Li.
Abstract
Dystrophin and its associated proteins form a scaffold underneath the cardiomyocyte membrane and connect the intracellular cytoskeleton to the extracellular matrix. Dystrophin localizes at the X chromosome, whose mutations might result in Duchenne muscular dystrophy, Becker muscular dystrophy and X-linked dilated cardiomyopathy. In addition to these genetic dilated cardiomyopathies, some acquired dilated cardiomyopathy like viral dilated cardiomyopathy is also related to dystrophin disruption or aberrant cleavage. In this review, we summarize the structure and distribution of dystrophin and researches of dystrophin in genetic and viral dilated cardiomyopathy. Moreover, we hypothesize that dystrophin play a critical role in ventricular remodeling in ischemic myocardium and treatment targeting restoration of dystrophin onto membrane could benefit for ischemic cardiomyopathy.Entities:
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Year: 2008 PMID: 18562127 DOI: 10.1016/j.mehy.2008.04.004
Source DB: PubMed Journal: Med Hypotheses ISSN: 0306-9877 Impact factor: 1.538