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)

Audrey Ncube; Lisa Bewersdorf; Lucas-Sebastian Spitzhorn; Christiane Loerch; Martina Bohndorf; Nina Graffmann; Lea May; Samira Amzou; Malin Fromme; Wasco Wruck; Pavel Strnad; James Adjaye

Stem Cell Research (Sep 2023)

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)

Audrey Ncube,
Lisa Bewersdorf,
Lucas-Sebastian Spitzhorn,
Christiane Loerch,
Martina Bohndorf,
Nina Graffmann,
Lea May,
Samira Amzou,
Malin Fromme,
Wasco Wruck,
Pavel Strnad,
James Adjaye

Affiliations

Audrey Ncube: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Lisa Bewersdorf: Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany
Lucas-Sebastian Spitzhorn: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Christiane Loerch: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Martina Bohndorf: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Nina Graffmann: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Lea May: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Samira Amzou: Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany
Malin Fromme: Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany
Wasco Wruck: Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany
Pavel Strnad: Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany
James Adjaye: 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; Corresponding author.

Journal volume & issue: Vol. 71
p. 103171

Abstract

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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.

Published in Stem Cell Research

ISSN: 1873-5061 (Print); 1876-7753 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Science: Biology (General)
Website: https://www.journals.elsevier.com/stem-cell-research

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