TY - JOUR
T1 - Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Type 1: A Light on Molecular Mechanisms
AU - Vanderschuren, Koen L. A.
AU - Sieverink, Tom
AU - Wilders, Ronald
PY - 2013
Y1 - 2013
N2 - 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 β 3). Several mechanisms underlying the molecular basis of ARVD/C type 1 have been proposed. Evaluating previously described mechanisms might elucidate how TGF β 3 contributes to disease progression in ARVD/C type 1. Here we review how TGF β 3 can induce fibrogenesis through Smad and/or β -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
AB - 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 β 3). Several mechanisms underlying the molecular basis of ARVD/C type 1 have been proposed. Evaluating previously described mechanisms might elucidate how TGF β 3 contributes to disease progression in ARVD/C type 1. Here we review how TGF β 3 can induce fibrogenesis through Smad and/or β -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
U2 - https://doi.org/10.1155/2013/460805
DO - https://doi.org/10.1155/2013/460805
M3 - Article
C2 - 24416594
SN - 2090-3154
VL - 2013
SP - 460805
JO - Genetics research international
JF - Genetics research international
ER -