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Stem Cell Res. 2017 Dec;25:1-5. doi: 10.1016/j.scr.2017.08.021. Epub 2017 Sep 14.

Integration-free induced pluripotent stem cells derived from a patient with autosomal recessive Alport syndrome (ARAS).

Author information

1
Center of Regenerative Medicine in Barcelona (CMRB), Barcelona, Spain; National Stem Cell Bank-Barcelona Node, Biomolecular and Bioinformatics Resources Platform PRB2, ISCIII, CMRB, Barcelona, Spain.
2
Center of Regenerative Medicine in Barcelona (CMRB), Barcelona, Spain.
3
Molecular Biology Laboratory, Fundacio Puigvert, Instituto de Investigaciones Biomedicas Sant Pau (IIB-Sant Pau), Universitat Autonoma de Barcelona, REDinREN, Instituto de Investigacion Carlos III, Barcelona, Spain.
4
Inherited Kidney Disorders, Nephrology Department, Fundacio Puigvert, Instituto de Investigaciones Biomedicas Sant Pau (IIB-Sant Pau), Universitat Autonoma de Barcelona, REDinREN, Instituto de Investigacion Carlos III, Barcelona, Spain.
5
Center of Regenerative Medicine in Barcelona (CMRB), Barcelona, Spain; National Stem Cell Bank-Barcelona Node, Biomolecular and Bioinformatics Resources Platform PRB2, ISCIII, CMRB, Barcelona, Spain; Center for Networked Biomedical Research on Bioengineering, Biomaterials and Nanomedicine (CIBER-BBN), Madrid, Spain.
6
Center of Regenerative Medicine in Barcelona (CMRB), Barcelona, Spain; Center for Networked Biomedical Research on Bioengineering, Biomaterials and Nanomedicine (CIBER-BBN), Madrid, Spain; Institució Catalana de Recerca I Estudis Avançats (ICREA), Barcelona, Spain. Electronic address: araya@cmrb.eu.

Abstract

A skin biopsy was obtained from a 25-year-old female patient with autosomal recessive Alport syndrome (ARAS) with the homozygous COL4A3 mutation c.345delG, p.(P166Lfs*37). Dermal fibroblasts were derived and reprogrammed by nucleofection with episomal plasmids carrying OCT3/4, SOX2, KLF4 LIN28, L-MYC and p53shRNA. The generated induced Pluripotent Stem Cell (iPSC) clone AS FiPS1 Ep6F-2 was free of genomically integrated reprogramming genes, had the specific homozygous mutation, a stable karyotype, expressed pluripotency markers and generated embryoid bodies which were differentiated towards the three germ layers in vitro. This iPSC line offers a useful resource to study Alport syndrome pathomechanisms and drug testing.

PMID:
29246570
DOI:
10.1016/j.scr.2017.08.021
[Indexed for MEDLINE]
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