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Fig. 6 | Stem Cell Research & Therapy

Fig. 6

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. 6

Summary of using human induced pluripotent stem cell (hiPSCs) to understand fibrodysplasia ossificans progressiva (FOP) R206H ACVR1 effects on human endothelial cell (EC) lineages. FOP hiPSCs are able to differentiate into ECs in a non-permissive condition and show increased osteogenic potential [14], which may reflect the increased number of ECs found in FOP lesions. FOP hiPSC-derived ECs (iECs) show increased expression of mesenchymal, extracellular matrix, chondrogenic, and osteogenic markers, which may play a major role in the tissue fibrosis found in patients with FOP during early stages of heterotopic ossification. FOP iECs show increased SMAD6 gene expression and respond differently to different ligands such as BMP, which could be proposed as a mechanism for their loss of cellular commitment and their proficiency in undergoing endothelial-to-mesenchymal transition

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Stem Cell Research & Therapy

ISSN: 1757-6512