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Generation of 3 spinocerebellar ataxia type 1 (SCA1) patient-derived induced pluripotent stem cell lines LUMCi002-A, B, and C and 2 unaffected sibling control induced pluripotent stem cell lines LUMCi003-A and B
- Source :
- Stem Cell Research, 29, 125-128. Elsevier Inc., Stem Cell Research, Vol 29, Iss, Pp 125-128 (2018), Stem Cell Research, 29, 125-128
- Publication Year :
- 2018
-
Abstract
- Spinocerebellar ataxia type 1 (SCA1) is a hereditary neurodegenerative disease caused by a CAG repeat expansion in exon 8 of the ATXN1 gene. We generated induced pluripotent stem cells (hiPSCs) from a SCA1 patient and his non-affected sister by using non-integrating Sendai Viruses (SeV). The resulting hiPSCs are SeVfree, express pluripotency markers, display a normal karyotype, retain the mutation (length of the CAG repeat expansion in the ATXN1 gene) and are able to differentiate into the three germ layers in vitro.
- Subjects :
- 0301 basic medicine
Adult
Male
Spinocerebellar Ataxia Type 1
congenital, hereditary, and neonatal diseases and abnormalities
Cellular differentiation
Induced Pluripotent Stem Cells
Biology
medicine.disease_cause
Cell Line
03 medical and health sciences
Exon
0302 clinical medicine
medicine
Humans
Spinocerebellar Ataxias
Induced pluripotent stem cell
lcsh:QH301-705.5
Mutation
Siblings
Cell Differentiation
Cell Biology
General Medicine
medicine.disease
Molecular biology
030104 developmental biology
lcsh:Biology (General)
Cell culture
Spinocerebellar ataxia
Female
Trinucleotide repeat expansion
030217 neurology & neurosurgery
Developmental Biology
Subjects
Details
- Language :
- English
- ISSN :
- 18735061
- Database :
- OpenAIRE
- Journal :
- Stem Cell Research, 29, 125-128. Elsevier Inc., Stem Cell Research, Vol 29, Iss, Pp 125-128 (2018), Stem Cell Research, 29, 125-128
- Accession number :
- edsair.doi.dedup.....2365faad3d2df3a340e53f6cc59c04f0