Moreover, the fact that the PKP2 pathogenic variants, c.2146-1G>C and c.148_151delACAG, p.Thr50Serfs*61, were present in both ARVC subgroups (those with and without a rare PLEC variant) studied with immunohistochemistry suggests that abnormal plectin localization is also not specifically related to desmosomal variants. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.