To date, seven genes have been implicated in the dominant and recessive ARVD, including transforming growth factor- β3 (TGFβ3) in ARVD1 [20], cardiac ryanodine receptor (RYR2) in ARVD2 [21] and five desmosomal proteins: desmoplakin (DSP) in ARVD8 [14] as well as Carvajal syndrome [16], plakophilin-2 (PKP-2) in ARVD9 [6], desmoglein-2 (DSG2) in ARVD10 [15], desmocollin-2 (DSC2) in ARVD11 [15] and desmocollin-2 (DSC2) [22] and junctional plakoglobin (JUP) in Naxos disease [17] and also in an autosomal dominant form of ARVD in a German family [23] (Table 2). The gene discussed is PKP2; the disease is arrhythmogenic cardiomyopathy with wooly hair and keratoderma.