Human Molecular Genetics, 2002, Vol. 11, No. 17 1927-1937
© 2002 Oxford University Press
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
1Center for Aging, Genetics and Neurodegeneration, Massachusetts General Hospital, Charlestown, MA 02129-4404, USA, 2Fred Hutchinson Cancer Research Center, Seattle WA 98109, USA, 3Department of Neuropathology and 4Department of Psychiatry and Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA, 5Department of Laboratory Medicine and Division of Medical Genetics (Medicine), University of Washington, Seattle, WA 98195-7110, USA and 6Thomas Jefferson University, Philadelphia, PA 19107, USA
Received March 12, 2002; Accepted June 7, 2002
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 N171-82Q 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.
* To whom correspondence should be addressed at: Fred Hutchinson Cancer Research Center, D4-100, 1100 Fairview Ave N, Seattle, WA 98109, USA. Tel: +1 2066677955; Fax: +1 2066672917; Email: jolson{at}fhcrc.org
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