Early genetic studies defined ARVC as a disease of the cardiac desmosome with the identification of mutations in desmosomal genes plakoglobin (JUP), desmoplakin (DSP), plakophilin (PKP2), desmoglein 2 (DSG2), and desmocollin 2 (DSC2), and the non-desmosomal transmembrane protein 43 (TMEM43) gene [1]. Here, DSP is linked to arrhythmogenic right ventricular cardiomyopathy.