Fig. 2.

Schematic illustration of RyR2, a large 4967-amino acid sarcoplasmic reticulum protein.

∗: Highlighted is the c.1195A > G (p.Met399Val) variant of RyR2, a novel disease-causing RyR2 variant through its role as a binding site for FKBP12.6/calstabin-2. Known CPVT mutation clusters are shown as dotted lines, with amino acid numbers indicating their boundaries (residues 1–466, 2246–2534, 3778–4201, 4497–4959).

Figure adapted from: Blayney LM, Lai FA. Ryanodine receptor-mediated arrhythmias and sudden cardiac death. Pharmacol Ther. 2009; 123(2):151–177. https://doi.org/10.1016/j.pharmthera.2009.03.006.