Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome
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 w...
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| Format: | Article |
| Language: | English |
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Elsevier
2025-04-01
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| Series: | Stem Cell Research |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1873506125000315 |
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| author | Cintia E. Gomez Limia Karunya Albert Kusum Basnet Chaitrali Atre Hudaa Malik Darria L. Streeter Nathalia G. Amado Linda A. Baker |
| author_facet | Cintia E. Gomez Limia Karunya Albert Kusum Basnet Chaitrali Atre Hudaa Malik Darria L. Streeter Nathalia G. Amado Linda A. Baker |
| author_sort | Cintia E. Gomez Limia |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-ee211a4af7f9447a9dee31d675f83778 |
| institution | DOAJ |
| issn | 1873-5061 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Stem Cell Research |
| spelling | doaj-art-ee211a4af7f9447a9dee31d675f837782025-08-20T02:52:21ZengElsevierStem Cell Research1873-50612025-04-018410368110.1016/j.scr.2025.103681Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly SyndromeCintia E. Gomez Limia0Karunya Albert1Kusum Basnet2Chaitrali Atre3Hudaa Malik4Darria L. Streeter5Nathalia G. Amado6Linda A. Baker7The Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAThe Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAThe Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAThe Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAThe Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAThe Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USACorresponding authors at: The Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USA (N.G. Amado).; The Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USACorresponding authors at: The Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USA (N.G. Amado).; The Kidney and Urinary Tract Center, The Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, USAPrune 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.http://www.sciencedirect.com/science/article/pii/S1873506125000315 |
| spellingShingle | Cintia E. Gomez Limia Karunya Albert Kusum Basnet Chaitrali Atre Hudaa Malik Darria L. Streeter Nathalia G. Amado Linda A. Baker Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome Stem Cell Research |
| title | Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome |
| title_full | Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome |
| title_fullStr | Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome |
| title_full_unstemmed | Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome |
| title_short | Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome |
| title_sort | generation of induced pluripotent stem cell line nchi028 a from a male child with prune belly syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S1873506125000315 |
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