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Series GSE9038 Query DataSets for GSE9038
Status Public on Aug 23, 2011
Title Gene expression profiles of striatum and cerebellum from knock-in mouse model of Huntington's disease
Organism Mus musculus
Experiment type Expression profiling by array
Summary Huntington’s disease (HD) involves marked early neurodegeneration in the striatum whereas the cerebellum is relatively spared despite the ubiquitous expression of full-length mutant huntingtin, implying that inherent tissue-specific differences determine susceptibility to the HD CAG mutation. To understand this tissue specificity, we compared early mutant huntingtin-induced gene expression changes in striatum to those in cerebellum in young Hdh CAG knock-in mice, prior to onset of evident pathological alterations. Endogenous levels of full-length mutant huntingtin caused qualitatively similar, but quantitatively different gene expression changes in the two brain regions. Importantly, the quantitatively different responses in striatum and cerebellum in mutant mice were well accounted for by the intrinsic molecular differences in gene expression between striatum and cerebellum in wild-type animals. Tissue-specific gene expression changes in response to the HD mutation, therefore, appear to reflect the different inherent capacities of these tissues to buffer qualitatively similar effects of mutant huntingtin. These findings highlight a role for intrinsic quantitative tissue differences in contributing to HD pathogenesis, and likely to other neurodegenerative disorders exhibiting tissue-specificity, thereby guiding the search for effective therapeutic interventions.
Overall design Total RNA was isolated from striatum and cerebellum of Hdh CAG knock-in mice (HdhQ111/HdhQ111) and wild-type mice (Hdh+/Hdh+, 3~10 weeks) for gene expression profiling using Affymetrix MG430 2.0 arrays.
Contributor(s) Lee J
Citation(s) 21840924
Submission date Sep 13, 2007
Last update date Feb 11, 2019
Contact name Jong-Min Lee
Phone 617-726-5726
Organization name Massachusetts General Hospital
Department Center for Human Genetic Reseaarch
Street address 185 Cambridge Street
City Boston
State/province MA
ZIP/Postal code 02114
Country USA
Platforms (1)
GPL1261 [Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array
Samples (24)
GSM229429 Hdh+/Hdh+ striatum at 3~10 weeks biological rep 1
GSM229430 Hdh+/Hdh+ striatum at 3~10 weeks biological rep 2
GSM229431 Hdh+/Hdh+ striatum at 3~10 weeks biological rep 3
BioProject PRJNA102543

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SOFT formatted family file(s) SOFTHelp
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Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE9038_RAW.tar 89.4 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file
Processed data included within Sample table

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