Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome
Summary
Prune belly syndrome (PBS) is a rare, multi-system congenital myopathy mainly affecting males, whose genetic basis is still being explored. Phenotypically, its morbidity spans from mild to lethal, and PBS cases manifest three cardinal pathological features: wrinkled, flaccid ventral abdominal wall with skeletal muscle deficiency, urinary tract dilation with poorly contractile smooth muscle, and intra-abdominal undescended testes. NCHi028-A is an iPSC line derived from skin fibroblasts of a 1-month-old male with PBS using Sendai Virus reprogramming factors. This iPSC line shows typical iPSC morphology, has normal karyotype, expresses undifferentiated hPSC state markers, and can be differentiated into three germ layers. Copyright © 2025 The Authors. Published by Elsevier B.V. All rights reserved.
| Authors | Gomez Limia CE, Albert K, Basnet K, Atre C, Malik H, Streeter DL, Amado NG, Baker LA |
|---|---|
| Journal | Stem cell research |
| Publication Date | 2025 Apr;84:103681 |
| PubMed | 40022902 |
| DOI | 10.1016/j.scr.2025.103681 |