Fig. 1

Information on LQT 7 patient and establishment of induced pluripotent stem cells. Images and quantifications are from at least three (n ≥ 3) independent differentiations for each hiPSC lines. A Twelve-lead electrocardiography examination of a patient with LQT7. B Pedigree of the LQT7 patient in this study. Squares indicate males; circles, females; filled-in symbols, clinically affected individuals; blank symbols, clinically unaffected individuals. C Schematic diagram showing the location of the KCNJ2 mutation, which was verified by Sanger sequencing. D iPSC lines showed RNA expression of the pluripotency-related genes POU5F1 and NODAL by RT‒PCR analyzed (MSC n = 3, hESC n = 3, CRISPR n = 6, Mutation n = 6). Data are shown as mean ± SD.*p < 0.05; data were compared by two-sample t-test. E Morphology of patient PBMCs and established LQT7 patient-derived iPSCs. F iPSCs from the mutation and CRISPR groups had a normal karyotype (Mutation n = 3, CRISPR n = 3). G iPSCs were stained for pluripotency markers (SSEA-4 and TRA-1-60) by flow cytometry analyzed (Mutation n = 6, CRISPR n = 6). H All iPSCs of the two groups gave rise to typical teratomas, which contained differentiated structures representing the three germ layers (nervous tissue [ectoderm], cartilage tissue [mesoderm] and intestinal epithelium [endoderm]) (Mutation n = 3, CRISPR n = 3)