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Figure 3 | Orphanet Journal of Rare Diseases

Figure 3

From: Niemann-Pick type C1 patient-specific induced pluripotent stem cells display disease specific hallmarks

Figure 3

Embryoid body formation and teratoma induction. Free floating embryoid bodies (EBs) of mutNPC1 and wtNPC1 hiPSCs were generated (A,B), which subsequently spontaneously differentiated (C-H). EBs of both cell lines were positive for markers of the three germ layers, namely Nestin (C,D) for ectoderm, smooth muscle actin (SMA) for mesoderm (E,F), and alpha fetoprotein (alpha FP) for endoderm (G,H). Nuclei were stained by DAPI, shown in blue, staining of Nestin, SMA and alpha FP is shown in green (scale bars A-H = 100 μm). HiPSCs of the mutNPC1 and wtNPC1 cell lines were injected in immunodeficient mice and tumors were extracted after 8–12 weeks. MutNPC1 and wtNPC1 hiPSCs induced teratomas contained structures typical for the ectoderm (I,J), mesoderm (K,L), and endoderm (M,N, scale bars I-N = 50 μm).

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