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Human Molecular Genetics Advance Access originally published online on May 11, 2005
Human Molecular Genetics 2005 14(13):1863-1876; doi:10.1093/hmg/ddi192
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

Gene expression in Huntington's disease skeletal muscle: a potential biomarker

Andrew D. Strand1,*, Aaron K. Aragaki2, Dennis Shaw4, Thomas Bird5, Janice Holton6, Christopher Turner7, Stephen J. Tapscott3, Sarah J. Tabrizi8, Anthony H. Schapira5,6, Charles Kooperberg2 and James M. Olson1

1Clinical Research Division, 2Public Health Sciences Division, 3Human Biology Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA, 4Department of Radiology, 5Department of Neurology, University of Washington, Seattle, WA 98195, USA, 6Department of Neuroscience, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK, 7University Department of Clinical Neurosciences, Royal Free and University College Medical School, UCL, London NW3 2PF, UK and 8Department of Neurodegenerative Disease/MRC Prion Unit Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK

* To whom correspondence should be addressed. Tel: +1 2066675681; Fax: +1 2066672917; Email: astrand{at}fhcrc.org

Received March 29, 2005; Revised April 29, 2005; Accepted May 6, 2005

Huntington's disease (HD) is an incurable and fatal neurodegenerative disorder. Improvements in the objective measurement of HD will lead to more efficient clinical trials and earlier therapeutic intervention. We hypothesized that abnormalities seen in the R6/2 mouse, a greatly accelerated HD model, might highlight subtle phenotypes in other mouse models and human HD. In this paper, we identify common gene expression changes in skeletal muscle from R6/2 mice, HdhCAG(150) homozygous knock-in mice and HD patients. This HD-triggered gene expression phenotype is consistent with the beginnings of a transition from fast-twitch to slow-twitch muscle fiber types. Metabolic adaptations similar to those induced by diabetes or fasting are also present but neither metabolic disorder can explain the full phenotype of HD muscle. The HD-induced gene expression changes reflect disease progression. This raises the possibility that muscle gene expression may be used as an objective biomarker to complement clinical HD-rating systems. Furthermore, an understanding of the molecular basis of muscle dysfunction in HD should provide insight into mechanisms involved in neuronal abnormalities and neurodegeneration.


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