COPS6 and Arrhythmogenic right ventricular dysplasia: The relevance of the cardiomyocyte CSN6-desmosomal complex in human cardiac disease was further shown as (i) cardiomyocyte-specific CSN6 knockout mice could recapitulate disease features reminiscent of a biventricular form of human ARVC, (ii) myocardial tissue from human ARVC patients harboring DSP and PKP2 mutations displayed a reduction in CSN6 cell-cell junction localization, and (iii) genetic mouse models harboring DSP and PKP2 mutations showcased molecular and disease alterations disruptive of the DSP structural-CSN signaling axis (Liang et al. 2021).