Most of the previously identified gene mutations that underlie ARVC (ARVC 8, 9, 10 and 12 and Naxos disease) are mutations of genes encoding desmosomal proteins: Plakophilin2 (PKP2), Desmoplakin (DSP), Desmoglein2 (DSG2), Desmocollin2 (DSC2) and Junction Plakoglobin (JUP) [16]–[23]. Here, JUP is linked to Naxos disease.