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Generation of induced pluripotent stem cell(iPSC)line CJUHi001-A derived peripheral blood mononuclear cells of spinocerebellar ataxia type 1(SCA1) the CAG repeat mutation in ATXN1 gene.

Authors :
Jin Y
Nan G
Source :
Stem cell research [Stem Cell Res] 2023 Feb; Vol. 66, pp. 102975. Date of Electronic Publication: 2022 Nov 14.
Publication Year :
2023

Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disorder caused by CAG repeat mutations in the ATXN1 gene. In this study, we generated an induced pluripotent stem cell line (iPSC) by using non-integrating Sendai virus (SeV) from peripheral blood mononuclear cells(PBMCs)of SCA1 patient harboring a CAG repeat mutation in the ATXN1 gene. The induced patient-specific iPSC line with a normal karyotype and expresses pluripotent markers, it also shows differentiation totipotency and tridermogenesis in vitro. It may be an excellent model for studying spinocerebellar ataxia type 1 (SCA1) in vitro and will be beneficial for studying SCA1 pathogenesis and therapeutic intervention strategies.<br />Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.<br /> (Copyright © 2022. Published by Elsevier B.V.)

Details

Language :
English
ISSN :
1876-7753
Volume :
66
Database :
MEDLINE
Journal :
Stem cell research
Publication Type :
Academic Journal
Accession number :
36459834
Full Text :
https://doi.org/10.1016/j.scr.2022.102975