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

从牙周膜成纤维细胞中生成进行性骨化性纤维发育不良和控制整合自由 iPSC 系

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作者:G Sanchez-Duffhues, H Mikkers, D de Jong, K Szuhai, T J de Vries, C Freund, N Bravenboer, R J J van Es, J C Netelenbos, M-J Goumans, E M W Eekhoff, P Ten Dijke

Abstract

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.

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