A. Alkaline phosphatase (AP) staining and immunofluorescence of selected iPSC lines (1) show the expression of the human ESC-specific markers: AP (2), NANOG (3), SSEA4 (4) and TRA-1-60 (5). B. The endogenous expression of the pluripotency genes (NANOG, OCT3/4 and SOX2) was quantified by qRT-PCR. Gene expression was normalized to the H9 ESCs, which was arbitrarily set to 1 (n=3). C. Phase contrast photographs show the formation of EBs (1) on Day 8 and rosettes (2) on Day 16 from iPSCs, and qPCR detected the genes PAX6 (ectoderm), AFP (endoderm) and BRACHYURY (mesoderm) reflecting the 3-germ layer differentiation of EBs (3) (n=3). D. The sections of teratomas were stained with hematoxylin-eosin: endoderm (1), mesoderm (2) and ectoderm (3). E. The karyotypes of selected iPSC colonies are normal. F-H. Quantitative PCR (F) and western blotting (G, H) analysis revealed a significantly decreased expression of SMN in SMA III and SMA I iPSC lines compared to the control iPSCs (n=5, one way ANOVA, *P < 0.05, **P < 0.01, ***P < 0.001). All data presented as the mean ± SEM. Scale bar, 100 μm.