Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but severe cardiac condition frequently associated with mutations of proteins involved in desmosomes (Marcus et al., 2013) (Plakophilin-2, Desmoplakin, Desmoglein-2, and Desmocollin-2), structures involved in the cell–cell interactions. Here, DSC2 is linked to Arrhythmogenic right ventricular dysplasia.