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Series GSE37517 Query DataSets for GSE37517
Status Public on Apr 25, 2012
Title Expression data from human induced pluripotent stem cell derived NSCs and striatal-like cells
Organism Homo sapiens
Experiment type Expression profiling by array
Summary Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an expanded stretch of CAG trinucleotide repeats that results in neuronal dysfunction and death. We made induced pluripotent stem cell (iPSC) lines from HD patients and controls. Though no obvious effects of the CAG expansion on reprogramming or subsequent neural stem cell (NSC) production were seen, HD-NSCs showed CAG expansion-associated gene expression patterns and, upon differentiation, changes in electrophysiology, metabolism, cell adhesion, and ultimately an increased risk of cell death for both medium and longer CAG repeat expansions, with some deficits greater in cells from longer repeat HD NSCs. The HD180 lines were more vulnerable than control lines to cellular stressors and BDNF withdrawal using a range of assays across consortium laboratories. This HD iPSC collection represents a unique and well-characterized resource to elucidate disease mechanisms in HD and provides a novel human stem cell platform for screening new candidate therapeutics.
Overall design 8 NSC samples (5 HD, 3 control), of which 3 were run as replicates (2HD, 1 control), and 5 striatal-like samples (3 HD, 2 control)
Contributor: The HD iPS consortium
Contributor(s) Thompson LM
Citation(s) 22748968
Submission date Apr 23, 2012
Last update date Jan 25, 2019
Contact name Leslie Thompson
Organization name University of California, Irvine
Street address Biological Sciences III
City Irvine
State/province CA
ZIP/Postal code 92697
Country USA
Platforms (1)
GPL10739 [HuGene-1_0-st] Affymetrix Human Gene 1.0 ST Array [probe set (exon) version]
Samples (13)
GSM920919 HD NSC 109Q
GSM920920 control NSC 21Q
GSM920921 HD NSC 180Q rep1
BioProject PRJNA161903

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE37517_RAW.tar 54.7 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file
Processed data included within Sample table

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