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Fig. 4 | Stem Cell Research & Therapy

Fig. 4

From: The ACVR1 R206H mutation found in fibrodysplasia ossificans progressiva increases human induced pluripotent stem cell-derived endothelial cell formation and collagen production through BMP-mediated SMAD1/5/8 signaling

Fig. 4

Fibrodysplasia ossificans progressiva (FOP) human induced pluripotent stem cell (hiPSC)-derived endothelial cells (iECs) form in conditions not permissive for controls and respond differently upon stimulation. a Wild type (WT) and FOP hiPSCs were differentiated as illustrated in Fig. 1d using different concentration of BMP4 (0, 1, 4, and 12 ng/ml). Shown is the mean percentage of PECAM+/KDR+ cells analyzed by fluorescent-activated cell sorting (FACS) at day 6 from three WT and four FOP hiPSC lines in at least three separate experiments for each group. Mean values were statistically different for 0, 1, and 4 ng/ml of BMP4. b WT and FOP hiPSCs were differentiated with or without supplemental Activin A. FACS analysis of PECAM+/KDR+ cells at day 6 did not show significant differences. c Representative western blot showing activation of SMAD pathways upon BMP4, Activin A, or TGFβ1 stimulation in WT and FOP iECs. d Western blot quantification of p-SMAD2 and p-SMAD1/5/8. Phosphorylation of SMAD2 was significantly increased in WT and FOP iECs treated with TGFβ1 compared to untreated cells. p-SMAD2 and p-SMAD1/5/8 protein expression were normalized to GAPDH protein expression. At least three separate experiments were run for WT and FOP iECs for each group. Error bars represent the mean ± one standard deviation. Mean values were statistically different by Student’s t test: * p < 0.05, ** p < 0.01, *** p<0.005

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