Generation of human iPS cell line IBCHi001-A from dentatorubral–pallidoluysian atrophy patient's fibroblasts
[ 1 ] Instytut Chemii Bioorganicznej Polskiej Akademii Nauk, Polska Akademia Nauk | [ P ] pracownik
2019
artykuł naukowy
angielski
EN
Dentatorubral–pallidoluysian atrophy (DRPLA) is an incurable autosomal dominant disease caused by an expansion of a CAG repeats in ATN1 gene encoding atrophin 1 protein. Here we report the generation of IBCHi001-A, an induced pluripotent stem cell (iPSC) line derived from DRPLA patient fibroblasts using non-integrative reprogramming technology with OCT4, SOX2, cMYC and KLF4 reprogramming factors. The pluripotency of iPSC was confirmed by immunocytochemistry and PCR for pluripotency markers and by the ability to form three germ layers in vitro. The established iPSC line offers a useful resource to study the pathogenesis of DPRLA.
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CC BY-ND (uznanie autorstwa - bez utworów zależnych)
ostateczna wersja opublikowana
07.2019
przed opublikowaniem
70
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