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Stem Cell Res. 2019 Nov 5;41:101639. doi: 10.1016/j.scr.2019.101639. [Epub ahead of print]

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

Author information

1
Department of Cell and Chemical Biology, Oncode Institute, Leiden University Medical Center, Leiden, the Netherlands. Electronic address: g.sanchez_duffhues@lumc.nl.
2
Department of Cell and Chemical Biology, Oncode Institute, Leiden University Medical Center, Leiden, the Netherlands; Leiden University Medical Center hiPSC Hotel, Leiden, the Netherlands.
3
Department of Cell and Chemical Biology, Oncode Institute, Leiden University Medical Center, Leiden, the Netherlands.
4
Department of Periodontology, Academic Centre for Dentistry Amsterdam, University of Amsterdam and VU University, Amsterdam, the Netherlands.
5
Leiden University Medical Center hiPSC Hotel, Leiden, the Netherlands; Department of Anatomy and Embryology, Leiden University Medical Center, Leiden, the Netherlands.
6
Department of Clinical Chemistry, Amsterdam UMC, VU University, Amsterdam, the Netherlands.
7
Department of oral and maxillofacial surgery, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
8
Internal Medicine, Endocrinology section, Amsterdam UMC, VU University, Amsterdam, the Netherlands.

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.

PMID:
31733439
DOI:
10.1016/j.scr.2019.101639
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