Generation of an induced pluripotent stem cell line HHUUKDi013-A (ISRM-AATD-iPSC-3) from a pediatric patient of Alpha-I Antitrypsin Deficiency (AATD)
Summary
Renal progenitor cells were isolated from urine from a male, pediatric AATD-patient, harboring the homologous Pi*ZZ genotype. SIX2-positive urine derived renal progenitor cells (UdRPCs) were reprogrammed to iPSCs by integration-free nucleofection of episomal-based plasmids expressing OCT4, KLF4, c-MYC, SOX2, NANOG and LIN28. ISRM-AATD-iPSC-3 show pluripotent gene and protein expression, are capable of forming embryoid bodies and carry the parental Pi*ZZ genotype. Global transcriptome analyses revealed a correlation coefficient 0.9091 between the human embryonic stem cell line H9 and ISRM-AATD-iPSC-3. Copyright © 2025 The Authors. Published by Elsevier B.V. All rights reserved.
| Authors | Loerch C, Hokamp R, Wruck W, Katzer D, Weigert A, Machui A, Graffmann N, Ganschow R, Adjaye J |
|---|---|
| Journal | Stem cell research |
| Publication Date | 2025 Jun 28;87:103762 |
| PubMed | 40602288 |
| DOI | 10.1016/j.scr.2025.103762 |