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Figure 5 | Orphanet Journal of Rare Diseases

Figure 5

From: Induced pluripotent stem cells from patients with human fibrodysplasia ossificans progressiva show increased mineralization and cartilage formation

Figure 5

Integration-free FOP iPS cells. (A) Representative images of FOP3 dermal fibroblasts subsequently converted to human iPS cell colonies in feeder-free conditions. Black scale bar = 50 μm. (B) Human integration-free iPS cells from donors with FOP are heterozygous for the ACVR1 R206H (617G > A) mutation but control lines are not. (C) Representative teratomas from one eFOP iPS cell line showing tissues representing all three germ layers (n, neuronal/primitive neural tube like structure; c, cartilage; g, primitive gut). Additional cell lines are shown in Additional file 6: Figure S4. Black scale bar = 50 μm. (D) Western blot showing total SMAD1/5/8 and phospho-SMAD1/5/8 (pSMAD) levels in FOP iPS cell lines relative to control lines with (+) and without (−) 45 minutes of BMP4 exposure. GAPDH, loading control. Representative blot of triplicate experiments.

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