This review highlights the molecular mechanisms underlying ARVD/C type 1, particularly the roles of TGF β 3, fibrogenesis, apoptosis, and immune system modulation in disease progression.
Review of TGF β 3 mechanisms in ARVD/C type 1 leaves open the dominant pathway; no practice change without functional validation.
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.
No takes yet. Share an insight, caveat, or question.
Vanderschuren et al. (2013) conducted a review in Arrhythmogenic right ventricular dysplasia/cardiomyopathy type 1. Transforming growth factor beta 3 (TGF β 3) was evaluated.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: