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. 2019 May 16;12(6):1242–1249. doi: 10.1016/j.stemcr.2019.04.016

Figure 4.

Figure 4

Functional Correction of FVIII Deficiency in Endothelial Cells Differentiated from Corrected iPSC Clones

(A) Phase image of cobblestone-like morphologies at the time of differentiation on day 4 in the indicated clones. Scale bars, 200 μm.

(B) Expression of marker proteins (CD31 and vWF) representing endothelial cells (ECs) derived from parental patient and corrected clones. The DAPI signal indicates the total cell content in the image. Scale bars, 100 μm.

(C) Expression of FVIII mRNA including EC marker genes were verified in cells differentiated from patients and corrected clones using RT-PCR (upper panel) and qPCR (lower panel). GAPDH expression was used for normalization. Data are means ± SEM of three independent experiments. ND, not detected.

(D) The FVIII activity was determined after 20-fold concentration in supernatants obtained from patient or corrected clones after differentiation into ECs. Data indicate activity detected per 1 × 106 ECs. Data are means ± SEM of three independent experiments. ∗p < 0.001 compared with the parental cells (Student's t test).