From a genetic point of view, the majority of mutations have been observed in genes encoding for proteins of the cardiac desmosomes, i.e., desmoplakin (DSP), plakophilin-2 (PKP2), desmoglein-2 (DSG2), PG and desmocollin-2 (DSC2), which are collectively responsible for about 50% of ARVC cases. Here, DSG2 is linked to arrhythmogenic right ventricular cardiomyopathy.