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.
Zheng
Qiang Sun
QS
Department of Cardiology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China. tdxnsys@fmmu.edu.cn
Guo
Wan Gang
WG
Lu
Zi Fan
ZF
Shi
Xiao Qin
XQ
Su
Fei Fei
FF
Li
Hua
H
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