Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.

doi:10.1093/hmg/ddm133

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Article

Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.

Human molecular genetics - 1 Aug 2007

Kuhn Alexandre, Goldstein Darlene R, Hodges Angela, Strand Andrew D, Sengstag Thierry, Kooperberg Charles, Becanovic Kristina, Pouladi Mahmoud A, Sathasivam Kirupa, Cha Jang-Ho J, Hannan Anthony J, Hayden Michael R, Leavitt Blair R, Dunnett Stephen B, Ferrante Robert J, Albin Roger, Shelbourne Peggy, Delorenzi Mauro, Augood Sarah J, Faull Richard L M, Olson James M, Bates Gillian P, Jones Lesley, Luthi-Carter Ruth

Abstract excerpt

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...

Topics

Animals
Brain
Corpus Striatum
Disease Models, Animal
Gene Dosage
Gene Expression
Humans
Huntingtin Protein

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Huntington Disease

Mice

Mice, Mutant Strains

Mutation

Nerve Tissue Proteins

Neurons

Nuclear Proteins

Phenotype

RNA, Messenger

Source

Open in PubMed

Identifiers

PMID: 17519223
DOI: 10.1093/hmg/ddm133

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026.

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