Six induced pluripotent stem cell lines from fibroblasts of individuals with CLN3-related conditions.

从患有 CLN3 相关疾病的个体的成纤维细胞中诱导出六种多能干细胞系

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作者:Dwojak Ewelina, O'Mard Danielle, Zou Jizhong, Wassif Christopher A, Burkett Sandra, Eckhaus Michael, Rueda Faucz Fabio, Padilla Cameron, Villasmil Rafael, Zheng Wei, Dang Do An N
Primary fibroblasts from six individuals with CLN3-related conditions were used to generate induced pluripotent stem cell (iPSC) lines CHDTRi001-B, CHDTRi002-B, CHDTRi003-A, CHDTRi004-B, CHDTRi005-A, and CHDTRi006-E through the expression of four reprogramming factors: human OCT3/4, KLF4, SOX2, and c-MYC. The iPSC lines were characterized to confirm their pluripotency via immunocytochemistry, flow cytometry, and teratoma formation. Genomic stability, cell line identity, and CLN3 genotype were confirmed. These iPSC lines may be used as participant-derived experimental models for further investigation of CLN3, a rare, fatal, pediatric, blindness and neurodegenerative lysosomal disorder with no cure.

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