PKP2 and arrhythmogenic right ventricular cardiomyopathy: On the other hand, arrhythmogenic right ventricular cardiomyopathy, a disease mostly caused by mutations in genes encoding desmosome proteins, including plakophilin-2, plakoglobin, desmoglein-2 and desmoplakin [329], has been shown to be associated with secondary effects in Cx43 expression.