On the other hand, six genes (PKP2 = plakophillin-2, DSP = desmoplakin, DSG2 = desmoglein-2, JUP = junctional plakoglobin, TGFβ 3 = Transforming Growth Factor-β3, RyR2 = cardiac Ryanodin Receptor-2) and 12 loci have been identified in ARVD/C so far, accounting for 30–70% of cases (Basso et al., 2006; Corrado and Thiene, 2006; Pilichou et al., 2006; Van Tintelen et al., 2006; Sen-Chowdhry et al., 2007a,b), with an autosomal dominant inheritance in half of cases (Hamid et al., 2002) and with a tropism for desmosomal proteins. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.