Although we acknowledge that more research is needed to fully understand the extent to which the observed changes in PKP2 Hom mice are present in humans, we believe our study represents an important step in unraveling complex mechanisms underlying PKP2-related ARVC, especially related to splicing mutations, providing a foundation for future investigations in both animal models and clinical settings. This evidence concerns the gene PKP2 and Arrhythmogenic right ventricular dysplasia.