Stem Cell Research (May 2018)

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

  • Aarne Fleischer,
  • Iván M. Lorenzo,
  • Esther Palomino,
  • Trond Aasen,
  • Fernando Gómez,
  • Miguel Servera,
  • Víctor J. Asensio,
  • Víctor Gálvez,
  • Juan Carlos Izpisúa-Belmonte,
  • Daniel Bachiller

Journal volume & issue
Vol. 29
pp. 1 – 5

Abstract

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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.