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An integration-free iPSC line ZZUNEUi008-A derived from dermal fibroblasts of a child with cardiac valvular dysplasia carrying a mutation in FLNA gene.
Stem Cell Research ( IF 0.8 ) Pub Date : 2020-06-22 , DOI: 10.1016/j.scr.2020.101882
Xiaowei Li 1 , Yanyu Lu 2 , Jiaxiang Wang 3 , Mengduan Liu 1 , Mengyu Wang 1 , Liang Hu 1 , Wenting Du 1 , Lu Wang 1 , Zhengming Jiang 2 , Xiangmei Gu 2 , Zhongren Ding 1 , Deguang Feng 3 , Ling Li 2 , Jianzeng Dong 4 , Xiaoyan Zhao 1
Affiliation  

FLNA gene encodes an actin-binding protein filamin A and mutations in FLNA can causes X-Linked cardiac valvular dysplasia. In this study, we report the generation of ZZUNEUi008-A, a human induced pluripotent stem cell line from a 10-year-old male patient with c. 84G → A in FLNA gene using non-integrative Sendai viral reprogramming technology. The ZZUNEUi008-A iPSC line expresses pluripotency markers, exhibits a normal male karyotype (46, XY) and can differentiate into three germ layers in vivo.



中文翻译:


无整合 iPSC 系 ZZUNEUi008-A,源自携带 FLNA 基因突变的心脏瓣膜发育不良儿童的真皮成纤维细胞。



FLNA 基因编码肌动蛋白结合蛋白 filamin A,FLNA 突变可导致 X 连锁心脏瓣膜发育不良。在这项研究中,我们报告了 ZZUNEUi008-A 的产生,这是一种来自一名 10 岁男性 c 患者的诱导多能干细胞系。 FLNA基因中的84G→A采用非整合仙台病毒重编程技术。 ZZUNEUi008-A iPSC 系表达多能性标记,表现出正常的雄性核型(46,XY),并且可以在体内分化成三个胚层。

更新日期:2020-06-22
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