Mouse models of CPVT (CASQ2 KO [20] and several other lines [54,55,56]) have been reported to recapitulate the human disease and have been extensively used to facilitate mechanistic studies of CPVT and RyR2 dysfunction [37,57,58,59,60]. Here, RYR2 is linked to catecholaminergic polymorphic ventricular tachycardia.