title: Arrhythmogenic right ventricular dysplasia/cardiomyopathy type 1: a light on molecular mechanisms.
abstract: 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.
authors.affiliation: Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Meibergdreef 15, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.
authors.forename: Koen L A
authors.initials: KL
authors.lastname: Vanderschuren
authors.affiliation: Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Meibergdreef 15, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.
authors.forename: Tom
authors.initials: T
authors.lastname: Sieverink
authors.affiliation: Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Meibergdreef 15, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.
authors.forename: Ronald
authors.initials: R
authors.lastname: Wilders