Polyglutamine and transcription: Gene expression changes shared by DRPLA and Huntington's disease mouse models reveals context-independent effects

Ruth Luthi-Carter, Andrew D. Strand, Sarah A. Hanson, Charles Kooperberg, Gabriele Schilling, Albert R. La Spada, Diane E. Merry, Anne B. Young, Christopher A. Ross, David R. Borchelt, James M. Olson

Research output: Contribution to journalArticlepeer-review

Abstract

Recent evidence indicates that transcriptional abnormalities may play an important role in the pathophysiology of polyglutamine diseases. In the present study, we have explored the extent to which polyglutamine-related changes in gene expression may be independent of protein context by comparing mouse models of dentatorubral-pallidoluysian atrophy (DRPLA) and Huntington's disease (HD). Microarray gene expression profiling was conducted in mice of the same background strain in which the same promoter was employed to direct the expression of full-length atrophin-1 or partial huntingtin transproteins (At-65Q or N171-82Q mice). A large number of overlapping gene expression changes were observed in the cerebella of At-65Q and N171-820 mice. Six of the gene expression changes common to both huntingtin and atrophin-1 transgenic mice were also observed in the cerebella of mouse models expressing full-length mutant ataxin-7 or the androgen receptor. These results demonstrate that some of the gene expression effects of expanded polyglutamine proteins occur independently of protein context.

Original languageEnglish (US)
Pages (from-to)1927-1937
Number of pages11
JournalHuman molecular genetics
Volume11
Issue number17
StatePublished - Aug 15 2002

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Genetics(clinical)

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