Generation of a Bartter syndrome type 3 patient-derived induced pluripotent stem cell line ISRM-BS3-UM18-iPSC (HHUUKDi014-A)

IF 0.8 4区医学 Q4 BIOTECHNOLOGY & APPLIED MICROBIOLOGY

Stem cell research Pub Date : 2025-06-28 DOI:10.1016/j.scr.2025.103760

Chantelle Thimm , Chutong Zhong , Wasco Wruck , Alessandra Grillo , Rosanne Mack , Martina Bohndorf , Nina Graffmann , Anson Tang , Viola D’Ambrosio , Elizabeth R Wan , Keith Siew , Rhys D Evans , Stephan B. Walsh , James Adjaye

引用次数: 0

Abstract

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.

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Bartter综合征3型诱导多能干细胞系ISRM-BS3-UM18-iPSC （HHUUKDi014-A）的生成

从一名18岁的Bartter综合征3型（BS3）患者中分离到six2阳性尿源性肾祖细胞（UdRPCs），该患者具有纯合子CLCNKB基因缺失。两个episomal质粒表达OCT4， SOX2， NANOG, KLF4， c-MYC和LIN28，我们能够产生无整合诱导多能干细胞系（iPSC）。标记物oct4、SOX2、NANOG、TRA-1-60、TRA-1-81和SSEA4的多能性通过荧光活化细胞分选分析和免疫细胞化学证实。通过免疫细胞化学方法证实了胚状体向三种胚层的分化。多元分析显示Pearson相关性为0.93。进行短串联重复DNA指纹图谱和核型分析。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Stem cell research 生物-生物工程与应用微生物

CiteScore

2.20

自引率

8.30%

发文量

338

审稿时长

55 days

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