Human Molecular Genetics, 2000, Vol. 9, No. 9 1259-1271
© 2000 Oxford University Press
Decreased expression of striatal signaling genes in a mouse model of Huntington’s disease
Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA, 1Human Biology Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA, 2Department of Neuropathology and 3Department of Psychiatry, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
To understand gene expression changes mediated by a polyglutamine repeat expansion in the human huntingtin protein, we used oligonucleotide DNA arrays to profile ~6000 striatal mRNAs in the R6/2 mouse, a transgenic Huntington’s disease (HD) model. We found diminished levels of mRNAs encoding components of the neurotransmitter, calcium and retinoid signaling pathways at both early and late symptomatic time points (6 and 12 weeks of age). We observed similar changes in gene expression in another HD mouse model (N171-82Q). These results demonstrate that mutant huntingtin directly or indirectly reduces the expression of a distinct set of genes involved in signaling pathways known to be critical to striatal neuron function.
+ To whom correspondence should be addressed. Tel: +1 206 667 7955; Fax: +1 206 667 6524; Email: jimmyo@u.washington.edu
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S. Sipione, D. Rigamonti, M. Valenza, C. Zuccato, L. Conti, J. Pritchard, C. Kooperberg, J. M. Olson, and E. Cattaneo Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses Hum. Mol. Genet., August 15, 2002; 11(17): 1953 - 1965. [Abstract] [Full Text] [PDF]
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A. P. Lieberman, G. Harmison, A. D. Strand, J. M. Olson, and K. H. Fischbeck Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor Hum. Mol. Genet., August 15, 2002; 11(17): 1967 - 1976. [Abstract] [Full Text] [PDF]
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X. L. Xu, J. M. Olson, and L. P. Zhao A regression-based method to identify differentially expressed genes in microarray time course studies and its application in an inducible Huntington's disease transgenic model Hum. Mol. Genet., August 15, 2002; 11(17): 1977 - 1985. [Abstract] [Full Text] [PDF]
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J. Kirby, F. M. Menzies, M. R. Cookson, K. Bushby, and P. J. Shaw Differential gene expression in a cell culture model of SOD1-related familial motor neurone disease Hum. Mol. Genet., August 15, 2002; 11(17): 2061 - 2075. [Abstract] [Full Text] [PDF]
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C. D. Keene, C. M. P. Rodrigues, T. Eich, M. S. Chhabra, C. J. Steer, and W. C. Low Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease PNAS, August 6, 2002; 99(16): 10671 - 10676. [Abstract] [Full Text] [PDF]
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P. F. Behrens, P. Franz, B. Woodman, K. S. Lindenberg, and G. B. Landwehrmeyer Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation Brain, August 1, 2002; 125(8): 1908 - 1922. [Abstract] [Full Text] [PDF]
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Y. Chai, J. Shao, V. M. Miller, A. Williams, and H. L. Paulson Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis PNAS, July 9, 2002; 99(14): 9310 - 9315. [Abstract] [Full Text] [PDF]
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J. Gafni and L. M. Ellerby Calpain Activation in Huntington's Disease J. Neurosci., June 15, 2002; 22(12): 4842 - 4849. [Abstract] [Full Text] [PDF]
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L. P. de Almeida, C. A. Ross, D. Zala, P. Aebischer, and N. Deglon Lentiviral-Mediated Delivery of Mutant Huntingtin in the Striatum of Rats Induces a Selective Neuropathology Modulated by Polyglutamine Repeat Size, Huntingtin Expression Levels, and Protein Length J. Neurosci., May 1, 2002; 22(9): 3473 - 3483. [Abstract] [Full Text] [PDF]
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C. Zabel, D. C. Chamrad, J. Priller, B. Woodman, H. E. Meyer, G. P. Bates, and J. Klose Alterations in the Mouse and Human Proteome Caused by Huntington's Disease Mol. Cell. Proteomics, May 1, 2002; 1(5): 366 - 375. [Abstract] [Full Text] [PDF]
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Z.-X. Yu, S.-H. Li, H.-P. Nguyen, and X.-J. Li Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice Hum. Mol. Genet., April 15, 2002; 11(8): 905 - 914. [Abstract] [Full Text] [PDF]
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R. J. Ferrante, O. A. Andreassen, A. Dedeoglu, K. L. Ferrante, B. G. Jenkins, S. M. Hersch, and M. F. Beal Therapeutic Effects of Coenzyme Q10 and Remacemide in Transgenic Mouse Models of Huntington's Disease J. Neurosci., March 1, 2002; 22(5): 1592 - 1599. [Abstract] [Full Text] [PDF]
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K. B. Kegel, A. R. Meloni, Y. Yi, Y. J. Kim, E. Doyle, B. G. Cuiffo, E. Sapp, Y. Wang, Z.-H. Qin, J. D. Chen, et al. Huntingtin Is Present in the Nucleus, Interacts with the Transcriptional Corepressor C-terminal Binding Protein, and Represses Transcription J. Biol. Chem., February 22, 2002; 277(9): 7466 - 7476. [Abstract] [Full Text] [PDF]
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H. Li, S.-H. Li, Z.-X. Yu, P. Shelbourne, and X.-J. Li Huntingtin Aggregate-Associated Axonal Degeneration is an Early Pathological Event in Huntington's Disease Mice J. Neurosci., November 1, 2001; 21(21): 8473 - 8481. [Abstract] [Full Text] [PDF]
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R. N. Rosenberg Genomic Neurology: A New Beginning Arch Neurol, November 1, 2001; 58(11): 1739 - 1741. [Full Text] [PDF]
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R. E. Hughes, R. S. Lo, C. Davis, A. D. Strand, C. L. Neal, J. M. Olson, and S. Fields Altered transcription in yeast expressing expanded polyglutamine PNAS, October 25, 2001; (2001) 191498198. [Abstract] [Full Text] [PDF]
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K. Sathasivam, B. Woodman, A. Mahal, F. Bertaux, E. E. Wanker, D. T. Shima, and G. P. Bates Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients Hum. Mol. Genet., October 1, 2001; 10(21): 2425 - 2435. [Abstract] [Full Text] [PDF]
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G. Yvert, K. S. Lindenberg, D. Devys, D. Helmlinger, G. B. Landwehrmeyer, and J.-L. Mandel SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types Hum. Mol. Genet., August 1, 2001; 10(16): 1679 - 1692. [Abstract] [Full Text] [PDF]
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B. O. Evert, I. R. Vogt, C. Kindermann, L. Ozimek, R. A. I. de Vos, E. R. P. Brunt, I. Schmitt, T. Klockgether, and U. Wullner Inflammatory Genes Are Upregulated in Expanded Ataxin-3-Expressing Cell Lines and Spinocerebellar Ataxia Type 3 Brains J. Neurosci., August 1, 2001; 21(15): 5389 - 5396. [Abstract] [Full Text] [PDF]
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A. Petersen, K. E. Larsen, G. G. Behr, N. Romero, S. Przedborski, P. Brundin, and D. Sulzer Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration Hum. Mol. Genet., June 1, 2001; 10(12): 1243 - 1254. [Abstract] [Full Text] [PDF]
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S.-H. Li, S. Lam, A. L. Cheng, and X.-J. Li Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis Hum. Mol. Genet., November 1, 2000; 9(19): 2859 - 2867. [Abstract] [Full Text] [PDF]
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C. Zuccato, A. Ciammola, D. Rigamonti, B. R. Leavitt, D. Goffredo, L. Conti, M. E. MacDonald, R. M. Friedlander, V. Silani, M. R. Hayden, et al. Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease Science, July 20, 2001; 293(5529): 493 - 498. [Abstract] [Full Text] [PDF]
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Y. Chai, L. Wu, J. D. Griffin, and H. L. Paulson The Role of Protein Composition in Specifying Nuclear Inclusion Formation in Polyglutamine Disease J. Biol. Chem., November 21, 2001; 276(48): 44889 - 44897. [Abstract] [Full Text] [PDF]
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R. E. Hughes, R. S. Lo, C. Davis, A. D. Strand, C. L. Neal, J. M. Olson, and S. Fields Altered transcription in yeast expressing expanded polyglutamine PNAS, November 6, 2001; 98(23): 13201 - 13206. [Abstract] [Full Text] [PDF]
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