Abstract
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by severe ventricular arrhythmias and progressive replacement of healthy myocardium with fibrous and adipose tissue. ARVC associates with mutations in genes coding for desmosomal proteins, most commonly plakophilin-2 (PKP2). It is unclear how disruption of desmosomes in cardiac myocytes leads to fibrosis and fat accumulation. We propose that cardiac-resident, desmosome-expressing, non-myocyte cells are a major source of fibroblasts and adipocytes in ARVC.
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