Striatal specificity of gene expression dysregulation in Huntington's disease.

Striatal specificity of gene expression dysregulation in Huntington's disease. Research Abstract Details 

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Striatal specificity of gene expression dysregulation in Huntington's disease. Abstract Text:

Elizabeth A Thomas,

Huntington's disease (HD) is a progressive neurodegenerative disorder caused by an expanded CAG repeat region in exon 1 of the HD gene. This mutation results in the presence of an abnormally long polyglutamine tract in the encoded protein, huntingtin (htt). A major question in this field is how the mutant htt protein, which is expressed ubiquitously throughout the brain and body, causes severe neuropathologic changes predominantly in the striatum. The mechanisms accounting for this specificity are unknown. The role of transcriptional dysregulation in the pathophysiology of HD has gained much attention in recent years, however, this theory has been unable to explain the specificity of dysfunction and degeneration in HD. Microarray studies have showed hundreds of gene expression changes in mouse models of HD and in post-mortem brain samples from HD subjects. Among the genes whose expression levels are preferentially altered are those that exhibit enriched expression in the striatum, which we have argued are the most relevant to disease pathology. These "striatal-enriched" genes are associated with several systems previously implicated in HD pathology, especially disturbances in transcriptional processes and calcium homeostasis. Large-scale changes in striatal gene expression in this manner would likely have particularly devastating effects to normal striatal function and could explain the specificity of striatal dysfunction and ultimate neurodegeneration observed in HD.

Striatal specificity of gene expression dysregulation in Huntington's disease. Publishing Authors By Initials

EA Thomas,

For similar genetic processes: gene expression: transcription, genetic research abstracts see: genetic processes: gene expression: transcription, genetic research

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Striatal specificity of gene expression dysregulation in Huntington's disease. Journal Published:

PUBLICATION TYPE: Review

Journal: Journal of neuroscience research

VOLUME: 84

Page Numbers: 1151-64

Journal Abbreviation: J. Neurosci. Res.

ISSN: 0360-4012

DAY: 1

MONTH: Nov

YEAR: 2006

Striatal specificity of gene expression dysregulation in Huntington's disease. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 7600111

Striatal specificity of gene expression dysregulation in Huntington's disease. Keywords Mesh Terms:

KEYWORDS: Transcription, Genetic

MESH TERMS: genetics

Chemical & Substance for Abstract: Striatal specificity of gene expression dysregulation in Huntington's disease. Information

Substance Name: GTP-Binding Proteins

Registry Number: EC 3.6.1.-

Grant and Affiliation Information for Striatal specificity of gene expression dysregulation in Huntington's disease.

AFFILIATION: Department of Molecular Biology, The Scripps Research Institute, La Jolla, California 92037, USA. bthomas@scripps.edu

Country: United States

AGENCY: United States NINDS

GRANT: NS44169

ACRONYM: NS

MEDLINETA: J Neurosci Res

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