Generation of two induced pluripotent stem cell (iPSC) lines from p.F508del Cystic Fibrosis patients


Cystic Fibrosis (CF) is a monogenic, lethal disease caused by mutations in the cystic fibrosis transmembrane conductance (CFTR) gene. Here we report the production of CF-iPS cell lines from two different p.F508del homozygous female patients (Table 1). Two different primary cell types, skin fibroblasts and keratinocytes, were transfected with retroviral cocktails containing four: c-MYC, KLF4, OCT4 and SOX2 (MKOS) or three: KLF4, OCT4 and SOX2 (KOS) reprogramming factors. Two fibroblast-derived MKOS lines are described in the main text. The lines carry the p.F508del mutation, have a normal karyotype, express pluripotency markers and are able to differentiate into the three germ layers. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

Authors Fleischer A, Lorenzo IM, Palomino E, Aasen T, Gómez F, Servera M, Asensio VJ, Gálvez V, Izpisúa-Belmonte JC, Bachiller D
Journal Stem cell research
Publication Date 2018 May;29:1-5
PubMed 29554588
DOI 10.1016/j.scr.2018.03.004

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