PKP2 and arrhythmogenic right ventricular cardiomyopathy: To investigate the direct consequence of the PKP2 RNA splice site IVS10-1G>C mutation in the pathogenesis of ARVC, we created a knock-in mouse model within mouse intron 9 (mouse IVS9-1G>C equivalent to human IVS10-1G>C) through CRISPR–Cas9 genome editing (Fig. 1a).