Given that mutations in desmosomal proteins, including desmin, desmoplakin, desmoglein, desmocollin, plakoglobin and plakophilin-2 (PKP-2), are important in the pathogenesis of ARVC, this condition has been termed a desmosomal disease (5–7). Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.