SCN5A and arrhythmogenic right ventricular cardiomyopathy: In this study, we generate a PKP2 genetic knock-in mouse model to demonstrate the sufficiency of a prevalent PKP2 RNA splicing mutation (human PKP2 IVS10-1G>C) to phenocopy key human ARVC features in mouse, including sudden death, ventricular arrhythmias (which include ventricular depolarization abnormalities serving as a primer for life-threatening arrhythmias, as well as electrophysiological alterations in sodium channel function and Nav1.5 protein localization), biventricular dysfunction, inflammation, desmosomal ultrastructural deficits and fibrofatty replacement of myocardium.