Our data suggest that the molecular consequences of the PKP2 IVS10-1G>C splice acceptor site mutation on PKP2 protein levels during early cardiac development may confer more pronounced ARVC-related cardiac phenotypes (that may not be fully compensated by the mutant PKP2 protein), as we observed pronounced baseline arrhythmias and fatty replacement in conjunction with cardiac mechanical and pathological dysfunction in the PKP2 Hom model compared to other models that ablate or express mutations in PKP2 in adult heart settings35,36,39. The gene discussed is PKP2; the disease is Arrhythmia.