Generation of induced pluripotent stem cell line RCPCMi008-A derived from patient with spinocerebellar ataxia 17


IPSC line RCPCMi004-8 was generated from skin fibroblasts collected from a male patient with spinocerebellar ataxia 17. The patient has expanded trinucleotide CAG repeats in the TBP (TATA-binding protein) gene on chromosome 6q27. The reprogramming of fibroblasts was performed with Sendai viruses containing Oct-4, Sox-2, Klf-4, and c-Myc. Pluripotency was confirmed by immunofluorescence, RT-PCR, and the formation of embryoid bodies. The RCPCMi008-A cell line carries the same trinucleotide CAG repeats in the TBP gene. The RCPCMi008-A cell line can be used to model Spinocerebellar ataxia in vitro. Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.

Authors Shuvalova LD, Davidenko AV, Eremeev AV, Khomyakova EA, Zerkalenkova EA, Lebedeva OS, Bogomazova AN, Klyushnikov SA, Illarioshkin SN, Lagarkova MA
Journal Stem cell research
Publication Date 2021 Jul;54:102431
PubMed 34171784
DOI 10.1016/j.scr.2021.102431

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