Derivation of an induced pluripotent stem cell line (MUSIi004-A) from dermal fibroblasts of a 48-year-old spinocerebellar ataxia type 3 patient

Alisa Ritthaphai; Methichit Wattanapanitch; Manop Pithukpakorn; Worapa Heepchantree; Rungtip Soi-ampornkul; Panchalee Mahaisavariya; Daranporn Triwongwaranat; Kovit Pattanapanyasat; Chinnavuth Vatanashevanopakorn

Stem Cell Research (Jul 2018)

Derivation of an induced pluripotent stem cell line (MUSIi004-A) from dermal fibroblasts of a 48-year-old spinocerebellar ataxia type 3 patient

Alisa Ritthaphai,
Methichit Wattanapanitch,
Manop Pithukpakorn,
Worapa Heepchantree,
Rungtip Soi-ampornkul,
Panchalee Mahaisavariya,
Daranporn Triwongwaranat,
Kovit Pattanapanyasat,
Chinnavuth Vatanashevanopakorn

Affiliations

Alisa Ritthaphai: Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Siriraj Center for Regenerative Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Methichit Wattanapanitch: Siriraj Center for Regenerative Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Manop Pithukpakorn: Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Siriraj Center of Research Excellence in Precision Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Worapa Heepchantree: Research Department, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Rungtip Soi-ampornkul: Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Siriraj Center for Regenerative Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Panchalee Mahaisavariya: Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Daranporn Triwongwaranat: Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Kovit Pattanapanyasat: Siriraj Center for Regenerative Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Siriraj Center of Research Excellence for Microparticle and Exosome in Diseases, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Chinnavuth Vatanashevanopakorn: Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Siriraj Center for Regenerative Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Corresponding author at: Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

Journal volume & issue: Vol. 30
pp. 113 – 116

Abstract

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Dermal fibroblasts were obtained from a 48-year-old female patient with spinocerebellar ataxia type 3 (SCA3). Fibroblasts were reprogrammed by nucleofection with episomal plasmids, carrying L-MYC, LIN28, OCT4, SOX2, KLF4, EBNA-1 and shRNA against p53. The SCA3 patient-specific iPSC line, MUSIi004-A, was characterized by immunofluorescence staining to verify the expression of pluripotent markers. The iPSC line exhibited an ability to differentiate into three germ layers by embryoid body (EB) formation. Karyotypic analysis of the MUSIi004-A line was normal. The mutant allele was still present in the iPSC line. This iPSC line represents a useful tool for studying neurodegeneration in SCA3.

Published in Stem Cell Research

ISSN: 1873-5061 (Print); 1876-7753 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Science: Biology (General)
Website: https://www.journals.elsevier.com/stem-cell-research

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