Generation of EB and teratoma from iPSC clones in the PS patient. (A) EB formation is derived from BiPS1 without mtDNA deletion. Scale bar: 100 μm. Undetectable means that mtDNA deletion was not identified. (B) Quantitative RT-PCR analysis of the expression of markers of the three germ layers, PAX6, TBXT, and SOX17, relative to GAPDH in BiPSC clones (BiPS1, 3, 8). Three germ layer markers were significantly higher expressed in EBs than in BiPSC clones. **, and *** mean P < 0.01, and 0.001, respectively. Mean ± SEM. (C) Teratoma formation derived from BiPS1. The black arrow indicates teratoma formation in the femoral region. (D) Three germ layer formations of BiPS1 by teratoma assay. Scale bars: 100 μm. (E) EB formation is derived from a SiPS10 clone with 27% heteroplasmy of mtDNA deletion. Scale bar: 100 μm. 25% deletion means the heteroplasmy of mtDNA deletion in EB. (F) Quantitative RT-PCR analysis of the expression of markers of the three germ layers in SiPS10. Three germ layer markers were significantly higher expressed in EBs than SiPS10. *** mean P < 0.001. Mean ± SEM. (G) Teratoma formation derived from SiPS10. The black arrow indicates teratoma formation. (H) Three germ layer formations of SiPS10 by teratoma assay. Scale bars: 100 μm. SiPSC: skin-derived iPSCs; BiPSCs: blood-derived iPSCs; EB: embryonic body.