ARVC is mainly caused by pathogenic mutations in genes encoding desmosomal proteins: plakophilin-2 (PKP2), desmoplakin (DSP), desmocolin-2 (DSC2), desmoglein-2 (DSG2) and plakoglobin (PG), encoded by the JUP gene. The gene discussed is PKP2; the disease is arrhythmogenic right ventricular cardiomyopathy.