Generation of Fibrodysplasia ossificans progressiva and control integration free iPSC lines from periodontal ligament fibroblasts


Fibrodysplasia ossificans progressiva (FOP) is a very rare devastating heterotopic ossification disorder, classically caused by a heterozygous single point mutation (c.617G>A) in the ACVR1gene, encoding the Bone morphogenetic protein (BMP) type I receptor, also termed activin receptor-like kinase (ALK)2. FOP patients develop heterotopic ossification episodically in response to inflammatory insults, thereby compromising tissue sampling and the development of in vitro surrogate models for FOP. Here we describe the generation and characterization of a control and a classical FOP induced pluripotent stem cell (iPSC) line derived from periodontal ligament fibroblast cells using Sendai virus vectors. Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.

Authors Sanchez-Duffhues G, Mikkers H, de Jong D, Szuhai K, de Vries TJ, Freund C, Bravenboer N, van Es RJJ, Netelenbos JC, Goumans MJ, Eekhoff EMW, Ten Dijke P
Journal Stem cell research
Publication Date 2019 Dec;41:101639
PubMed 31733439
DOI 10.1016/j.scr.2019.101639

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