Generation of two Alpha-I antitrypsin deficiency patient-derived induced pluripotent stem cell lines ISRM-AATD-iPSC-1 (HHUUKDi011-A) and ISRM-AATD-iPSC-2 (HHUUKDi012-A).


Journal

Stem cell research
ISSN: 1876-7753
Titre abrégé: Stem Cell Res
Pays: England
ID NLM: 101316957

Informations de publication

Date de publication:
09 2023
Historique:
received: 28 04 2023
revised: 17 07 2023
accepted: 21 07 2023
medline: 8 9 2023
pubmed: 29 7 2023
entrez: 28 7 2023
Statut: ppublish

Résumé

SIX2-positive urine derived renal progenitor cells were isolated from a male and female alpha1-antitrypsin deficiency (AATD) patients both harboring the homozygous PiZZ genotype. The cells were reprogrammed to generate two integration-free induced pluripotent stem cell (iPSC) lines by transfecting episomal-based plasmids expressing OCT4, SOX2, NANOG, c-MYC, KLF4 and LIN28. Pluripotency was confirmed by immunocytochemistry for associated markers and embryoid body-based differentiation into the three germ layers. The iPSC lines carried the parental PiZZ genotype. Comparative transcriptome analyses with human embryonic stem cell line H9 revealed a Pearson correlation of 0.945 for ISRM-AATD-iPSC-1 and 0.939 for ISRM-AATD-iPSC-2 respectively.

Identifiants

pubmed: 37506509
pii: S1873-5061(23)00157-5
doi: 10.1016/j.scr.2023.103171
pii:
doi:

Substances chimiques

Kruppel-Like Factor 4 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

103171

Informations de copyright

Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Auteurs

Audrey Ncube (A)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Lisa Bewersdorf (L)

Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany.

Lucas-Sebastian Spitzhorn (LS)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Christiane Loerch (C)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Martina Bohndorf (M)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Nina Graffmann (N)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Lea May (L)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Samira Amzou (S)

Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany.

Malin Fromme (M)

Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany.

Wasco Wruck (W)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany.

Pavel Strnad (P)

Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany.

James Adjaye (J)

Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany; University College London (UCL), EGA Institute for Women's Health, Zayed Centre for Research into Rare Diseases in Children (ZCR), 20 Guilford Street, London WC1N 1DZ, UK. Electronic address: james.adjaye@med.uni-duesseldorf.de.

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Classifications MeSH