| Interestingly, all defects of desmosomal components causing heart defects such as desmoplakin, plakoglobin, plakophilin 2, Dsg 2 and Dsc 2 lead to the phenotype of arrhythmogenic right ventricular cardiomyopathy (ARVC) which is clinically characterized by right bundle block and arrhythmia and histologically by fibrofatty replacement of cardiomyocytes, possibly due to impaired cell adhesion caused by loss and alterations of desmosomes (Asimaki et al. 2007; Gerull et al. 2004; McKoy et al. 2000; Pilichou et al. 2006; Rampazzo et al. 2002; Syrris et al. 2006; Heuser et al. 2006).