Figure 1.

Introduction of R420Q and Q4201R mutations into the ryanodine receptor 2 (RYR2) gene of hiPSCs. A and B: Sequencing of polymerase chain reaction (PCR) products show R420Q (CGG to CAG) and Q4201R (CAG to CGG) mutations. C: PCR analysis of the mutations. PCR products from homozygous mutants were completely digested into 2 fragments: 323 and 126 bp for R420Q and 340 and 141 bp for Q4201R. D: Quantification of RyR2 transcription levels in wild-type (WT) and mutant hiPSC-CMs. **P < .01 vs WT. E and F: Western blot image and quantification of RyR2 protein levels in WT and 3 homozygous mutants. The F2483I homozygous clone was established in the previous study.¹⁷