Notably, the D179N mutation has been linked with CPVT and the equivalent RyR1 residue (D166G and D166N) has been linked with malignant hyperthermia (Supplementary material online, Figure S3), further highlighting the pivotal involvement of the negatively charged side chain of this residue in N-terminus self-association. Here, RYR1 is linked to catecholaminergic polymorphic ventricular tachycardia.