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English Wikipedia
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Arrhythmogenic right ventricular dysplasia/cardiomyopathy type 1: a light on molecular mechanisms Vanderschuren KL; Sieverink T; Wilders RGenet Res Int 2013[]; 2013 (ä): 460805Arrhythmogenic 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.ä |
