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

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

Author information

1
Consejo Superior de Investigaciones Científicas (CSIC/IMEDEA), Miguel Marqués 21, 07190 Esporles, Spain.
2
Institute de Recerca, Hospital Vall d'Hebron (VHIR), Molecular Pathology Department, Passeig Vall d'Hebron, 119-129, 08035 Barcelona, Spain.
3
Letterkenny University Hospital, Kilmacrennan Road, Ballyboe Glencar, Letterkenny, Co. Donegal, F92 AE81, Ireland.
4
Consultas Médicas Messalut, Paseo del Ferrocarril 1, Manacor, Spain.
5
Fundación Caubet-CIMERA, Development and Regeneration Program, Ctra. Sóller km 12, 07110 Bunyola, Spain.
6
Gene Expression Laboratory, Salk Institute for Biological Studies, La Jolla, CA 92037, USA.
7
Consejo Superior de Investigaciones Científicas (CSIC/IMEDEA), Miguel Marqués 21, 07190 Esporles, Spain. Electronic address: d.b@csic.es.

Abstract

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.

PMID:
29554588
DOI:
10.1016/j.scr.2018.03.004
[Indexed for MEDLINE]
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