Article
Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy.
The Journal of clinical investigation - 1 Jul 2006
Garcia-Gras Eduardo, Lombardi Raffaella, Giocondo Michael J, Willerson James T, Schneider Michael D, Khoury Dirar S, Marian Ali J
Abstract excerpt
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is a genetic disease caused by mutations in desmosomal proteins. The phenotypic hallmark of ARVC is fibroadipocytic replacement of cardiac myocytes, which is a unique phenotype with a yet-to-be-defined molecular mechanism. We established atrial myocyte cell lines expressing siRNA against desmoplakin (DP), responsible for human ARVC. We show...
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