Similarly, for CTNNA3, 2 variants were reported in 2 ARVC probands: one likely de novo missense variant absent from gnomAD (NM_013266.3(CTNNA3): c.281T>A; p.Val94Asp) and one in-frame deletion (NM_013266.3(CTNNA3): c.2296_2298del; p.Leu766del) with limited segregation.31 A germline knockout mouse showed altered PKP2 distribution without affecting other junctional components of the area composita. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.