Generation of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome.

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Tác giả: Karunya Albert, Nathalia G Amado, Chaitrali Atre, Linda A Baker, Kusum Basnet, Cintia E Gomez Limia, Hudaa Malik, Darria L Streeter

Ngôn ngữ: eng

Ký hiệu phân loại:

Thông tin xuất bản: England : Stem cell research , 2025

Mô tả vật lý:

Bộ sưu tập: NCBI

ID: 710681

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.
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