Generation of a Bartter syndrome type 3 patient-derived induced pluripotent stem cell line ISRM-BS3-UM18-iPSC (HHUUKDi014-A)
Summary
SIX2-positive urine-derived renal progenitor cells (UdRPCs) were isolated from an 18-year-old Bartter syndrome type 3 (BS3) patient within a homozygous CLCNKB gene deletion. Two episomal-based plasmids expressing OCT4, SOX2, NANOG, KLF4, c-MYC and LIN28 we were able to generate an integration-free induced pluripotent stem cell line (iPSC). Pluripotency was confirmed by fluorescence-activated cell sorting analysis and immunocytochemistry for the markers-OCT4, SOX2, NANOG, TRA-1-60, TRA-1-81 and SSEA4. Embryoid body-based differentiation into the three germ layers was the conducted and confirmed by immunocytochemistry. Pluritest analysis revealed a Pearson correlation of 0,93. Short tandem repeat DNA fingerprinting and karyotype analyses were performed. Copyright © 2025 The Authors. Published by Elsevier B.V. All rights reserved.
| Authors | Thimm C, Zhong C, Wruck W, Grillo A, Mack R, Bohndorf M, Graffmann N, Tang A, D'Ambrosio V, Wan ER, Siew K, Evans RD, Walsh SB, Adjaye J |
|---|---|
| Journal | Stem cell research |
| Publication Date | 2025 Jun 28;87:103760 |
| PubMed | 40609324 |
| DOI | 10.1016/j.scr.2025.103760 |