Generation of a Crigler-Najjar Syndrome Type I patient-derived induced pluripotent stem cell line CNS705 (HHUUKDi005-A)


Human fibroblasts cells from a Crigler-Najjar Syndrome (CNS) patient were used to generate integration-free induced pluripotent stem cells (iPSCs) by over-expressing episomal-based plasmids expressing OCT4, SOX2, NANOG, KLF4, c-MYC and LIN28. The derived CNS705-iPSC line is homozygous for the UGT1A1 c.877_890delTACATTAATGCTTCinsA mutation. Pluripotency was confirmed by the expression of associated markers and embryoid body-based differentiation into cell types from all three germ layers. Comparative transcriptome analysis of the iPSC and the human embryonic stem cell line H9 revealed a Pearson's correlation of 0.9468. Copyright © 2021 The Author(s). Published by Elsevier B.V. All rights reserved.

Authors Graffmann N, Martins S, Ljubikj T, Matte JC, Bohndorf M, Wruck W, Adjaye J
Journal Stem cell research
Publication Date 2021 Mar;51:102167
PubMed 33485181
DOI 10.1016/j.scr.2021.102167

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