Dystrophin: from non-ischemic cardiomyopathy to ischemic cardiomyopathy.
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.
Department of Cardiology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China. tdxnsys@fmmu.edu.cn
Qiang Sun
QS
Zheng
Wan Gang
WG
Guo
Zi Fan
ZF
Lu
Xiao Qin
XQ
Shi
Fei Fei
FF
Su
Hua
H
Li
D009202:Cardiomyopathies / Q000235:genetics*; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism*; D006023:Glycoproteins / Q000378:metabolism; D006801:Humans; D017202:Myocardial Ischemia / Q000235:genetics*; D020257:Ventricular Remodeling / Q000502:physiology*
D016189:Dystrophin; D006023:Glycoproteins