Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8425118 | Stem Cell Research | 2018 | 4 Pages |
Abstract
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.
Related Topics
Life Sciences
Biochemistry, Genetics and Molecular Biology
Biotechnology
Authors
Alisa Ritthaphai, Methichit Wattanapanitch, Manop Pithukpakorn, Worapa Heepchantree, Rungtip Soi-ampornkul, Panchalee Mahaisavariya, Daranporn Triwongwaranat, Kovit Pattanapanyasat, Chinnavuth Vatanashevanopakorn,