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

Nina Graffmann; Soraia Martins; Tijana Ljubikj; Julie Carole Matte; Martina Bohndorf; Wasco Wruck; James Adjaye

doi:10.1016/j.scr.2021.102167

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

Stem Cell Res. 2021 Mar:51:102167. doi: 10.1016/j.scr.2021.102167. Epub 2021 Jan 12.

Authors

Nina Graffmann¹, Soraia Martins¹, Tijana Ljubikj¹, Julie Carole Matte¹, Martina Bohndorf¹, Wasco Wruck¹, James Adjaye²

Affiliations

¹ Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225 Düsseldorf, Germany.
² Institute for Stem Cell Research and Regenerative Medicine, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225 Düsseldorf, Germany. Electronic address: james.adjaye@med.uni-duesseldorf.de.

PMID: 33485181
DOI: 10.1016/j.scr.2021.102167

Abstract

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Cell Differentiation
Cell Line
Cellular Reprogramming
Crigler-Najjar Syndrome*
Embryoid Bodies
Fibroblasts
Humans
Induced Pluripotent Stem Cells*
Kruppel-Like Factor 4