Back to Search Start Over

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

Authors :
Bert Eussen
Christian Freund
Marga J. Bouma
Sarah L. Gardiner
Ronald A.M. Buijsen
Merel W. Boogaard
Barry A. Pepers
Linda M. van der Graaf
Willeke M. C. van Roon-Mom
Annelies de Klein
Clinical Genetics
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.

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