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

Stem Cell Res. 2018 May;29:1-5. doi: 10.1016/j.scr.2018.03.004. Epub 2018 Mar 11.


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.

MeSH terms

  • Animals
  • Cell Line
  • Cystic Fibrosis / genetics*
  • Female
  • Humans
  • Induced Pluripotent Stem Cells / metabolism*
  • Kruppel-Like Factor 4
  • Male
  • Mutation