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Series GSE9803 Query DataSets for GSE9803
Status Public on Dec 17, 2007
Title Striatal gene expression data from 12 weeks-old R6/2 mice and control mice (set 1)
Organism Mus musculus
Experiment type Expression profiling by array
Summary To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in seven genetic mouse models of Huntington's disease (HD) and postmortem human HD caudate. Transgenic models expressing short N-terminal fragments of mutant huntingtin (R6/1 and R6/2 mice) exhibited the most rapid effects on gene expression, consistent with previous studies. Although changes in the brains of knock-in and full-length transgenic models of HD took longer to appear, 15- and 22-month CHL2(Q150/Q150), 18-month Hdh(Q92/Q92) and 2-year-old YAC128 animals also exhibited significant HD-like mRNA signatures. Whereas it was expected that the expression of full-length huntingtin transprotein might result in unique gene expression changes compared with those caused by the expression of an N-terminal huntingtin fragment, no discernable differences between full-length and fragment models were detected. In addition, very high correlations between the signatures of mice expressing normal levels of wild-type huntingtin and mice in which the wild-type protein is absent suggest a limited effect of the wild-type protein to change basal gene expression or to influence the qualitative disease-related effect of mutant huntingtin. The combined analysis of mouse and human HD transcriptomes provides important temporal and mechanistic insights into the process by which mutant huntingtin kills striatal neurons. In addition, the discovery that several available lines of HD mice faithfully recapitulate the gene expression signature of the human disorder provides a novel aspect of validation with respect to their use in preclinical therapeutic trials.
Keywords: genetic modification
Overall design Striatal samples from 4 R6/2 mutant mice (12 weeks-old) and 5 age-matched wild-type mice.
Contributor(s) Kuhn A, Sathasivam K, Bates GP, Luthi-Carter R
Citation(s) 17519223
Submission date Dec 06, 2007
Last update date Feb 11, 2019
Contact name Alexandre Kuhn
Organization name NIH
Department NIA
Lab Laboratory of Neurogenetics
Street address 35 Convent Dr
City Bethesda
State/province MD
ZIP/Postal code 20892-3707
Country USA
Platforms (1)
GPL1261 [Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array
Samples (9)
GSM247436 striatum_wildtype_48
GSM247437 striatum_wildtype_45
GSM247438 striatum_wildtype_42
This SubSeries is part of SuperSeries:
GSE9857 Striatal gene expression data from 12 weeks-old R6/2 mice and control mice
GSE10263 Mutant huntingtin's effects on striatal gene expression in mice
BioProject PRJNA105121

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
GSE9803_RAW.tar 53.2 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file
Processed data included within Sample table

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