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Figure 3 | Orphanet Journal of Rare Diseases

Figure 3

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

Figure 3

Increased mineralization in a second cohort of retroviral FOP iPS cells. (A, B) Representative photos of control and vFOP4 iPS cell colonies on feeders after retroviral iPS cell generation. White scale bar = 200 μm. (C) Retroviral iPS cell lines vFOP4-1, vFOP4-3, and vFOP5-22 show the ACVR1 R206H (617G > A) mutation. (D) Retroviral transgene expression in vFOP4 and vFOP5 iPS cell lines by RT-PCR. (E) vFOP4, vFOP5, and control iPS cells grown in mineralizing culture conditions. Mineral is detected as black staining with von Kossa. Representative images from triplicate assays with similar results. (F) Quantitation of mineralization intensity. Error bars represent the average of the three control cell lines (H9, vWT-TIG120-4f1, and vWT-201B7) or three vFOP iPS lines (vFOP4-1, vFOP4-3, and vFOP5-22), +/− 1 SD. n ≥ 6 measurements from per condition at each timepoint. **, p < 0.01; ***, p < 0.001 by Student’s t-test. (G) Increased mineralization by vFOP4 and vFOP5 iPS cells after 15 days of culture can be blocked by treatment with the BMP inhibitor DMH1. Representative experiment from triplicate assays with similar results. (H) Quantitation of mineralization intensity after DMH1 treatment of FOP iPS cell lines. n = 3 measurements for each DMH1 concentration and timepoint. *, p < 0.05; **, p < 0.01 by Dunnett’s multiple comparisons t-test compared to the no DMH1 treatment group.

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