An early demonstration of the utility of CRISPR-Cas9 genome editing in cardiomyopathy modeling involved the correction of a pathogenic SCN5A (OMIM: 600163) mutation in patient-derived iPSC-CMs, thereby establishing the causative role of SCN5A in arrhythmogenic right ventricular cardiomyopathy (ARVC) [137]. Here, SCN5A is linked to arrhythmogenic right ventricular cardiomyopathy.