Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Type 1: A Light on Molecular Mechanisms

被引：4
作者：
Vanderschuren, Koen L. A. [1 ]
Sieverink, Tom [1 ]
Wilders, Ronald [1 ]
机构：
[1] Univ Amsterdam, Acad Med Ctr, Heart Failure Res Ctr, Meibergdreef 15,POB 22700, NL-1100 DE Amsterdam, Netherlands
来源：
GENETICS RESEARCH INTERNATIONAL | 2013年 / 2013卷
关键词：
D O I：
10.1155/2013/460805
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy associated with cardiac arrhythmias originating in the right ventricle, heart failure, and sudden cardiac death. Development of ARVD/C type 1 has been attributed to differential expression of transforming growth factor beta 3 (TGF beta 3). Several mechanisms underlying the molecular basis of ARVD/C type 1 have been proposed. Evaluating previously described mechanisms might elucidate how TGF beta 3 contributes to disease progression in ARVD/C type 1. Here we review how TGF beta 3 can induce fibrogenesis through Smad and/or beta-catenin signaling. Moreover, the role of apoptosis is addressed. Finally the extent to which the immune system has been demonstrated to be a modulating and amplifying agent in the onset and progression of ARVD/C in general is discussed.
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