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Human Molecular Genetics, 2000, Vol. 9, No. 4 503-513
© 2000 Oxford University Press

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

Vanessa C. Wheeler1, Jacqueline K. White1,+, Claire-Anne Gutekunst2, Vladimir Vrbanac1, Meredith Weaver1, Xiao-Jiang Li3, Shi-Hua Li3, Hong Yi2, Jean-Paul Vonsattel4, James F. Gusella1, Steven Hersch2, Wojtek Auerbach5, Alexandra L. Joyner5,6 and Marcy E. MacDonald1,§

1Molecular Neurogenetics Unit, 4Laboratory for Molecular Neuropathology Massachusetts General Hospital, Charlestown, MA 02129, USA, 2Department of Neurology, 3Department of Genetics and Molecular Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA, 5Howard Hughes Medical Institute and Skirball Institute for Biomolecular Medicine and 6Department of Cell Biology, New York University School of Medicine, New York, NY 10016, USA

Huntington’s disease (HD) is caused by an expanded N-terminal glutamine tract that endows huntingtin with a striatal-selective structural property ultimately toxic to medium spiny neurons. In precise genetic models of juvenile HD, HdhQ92 and HdhQ111 knock-in mice, long polyglutamine segments change huntingtin’s physical properties, producing HD-like in vivo correlates in the striatum, including nuclear localization of a version of the full-length protein predominant in medium spiny neurons, and subsequent formation of N-terminal inclusions and insoluble aggregate. These changes show glutamine length dependence and dominant inheritance with recruitment of wild-type protein, critical features of the altered HD property that strongly implicate them in the HD disease process and that suggest alternative pathogenic scenarios: the effect of the glutamine tract may act by altering interaction with a critical cellular constituent or by depleting a form of huntingtin essential to medium spiny striatal neurons.

+ Present address: Department of Medicine, Wellcome Trust Addenbrooke’s Hospital Site, Cambridge CB2 2XY, UK

§ To whom correspondence should be addressed. Tel: +1 617 726 5089; Fax: +1 617 726 5735; Email: macdonam@helix.mgh.harvard.edu


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M. F. Peters and C. A. Ross
Isolation of a 40-kDa Huntingtin-associated Protein
J. Biol. Chem., January 26, 2001; 276(5): 3188 - 3194.
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