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Generation of Fibrodysplasia ossificans progressiva and control integration free iPSC lines from periodontal ligament fibroblasts.
Stem Cell Research ( IF 0.8 ) Pub Date : 2019-11-05 , DOI: 10.1016/j.scr.2019.101639
G Sanchez-Duffhues 1 , H Mikkers 2 , D de Jong 1 , K Szuhai 1 , T J de Vries 3 , C Freund 4 , N Bravenboer 5 , R J J van Es 6 , J C Netelenbos 7 , M-J Goumans 1 , E M W Eekhoff 7 , P Ten Dijke 1
Affiliation  

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.



中文翻译:

牙骨膜增生性纤维增生的产生和来自牙周膜成纤维细胞的无整合iPSC系的控制。

骨增生性纤维增生症(FOP)是一种非常罕见的毁灭性异位骨化疾病,通常由ACVR1基因中的杂合单点突变(c.617G> A)引起,编码A型骨形态发生蛋白(BMP)受体,也称为激活素受体样激酶(ALK)2。FOP患者会因炎症反应而发作异位骨化,从而损害组织采样和FOP体外替代模型的开发。在这里,我们描述了使用仙台病毒载体从牙周膜成纤维细胞衍生的对照和经典FOP诱导的多能干细胞(iPSC)系的生成和表征。

更新日期:2019-11-05
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