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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

Ruth Luthi-Carter, Andrew Strand1, Nikki L. Peters, Steven M. Solano, Zane R. Hollingsworth, Anil S. Menon, Ariel S. Frey, Boris S. Spektor, Ellen B. Penney, Gabriele Schilling, Christopher A. Ross, David R. Borchelt, Stephen J. Tapscott1, Anne B. Young, Jang-Ho J. Cha and James M. Olson1,+

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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F. Li, T. Macfarlan, R. N. Pittman, and D. Chakravarti
Ataxin-3 Is a Histone-binding Protein with Two Independent Transcriptional Corepressor Activities
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Z.-L. Wu, T. M. O'Kane, R. W. Scott, M. J. Savage, and D. Bozyczko-Coyne
Protein Tyrosine Phosphatases Are Up-regulated and Participate in Cell Death Induced by Polyglutamine Expansion
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A. D. Strand, J. M. Olson, and C. Kooperberg
Estimating the statistical significance of gene expression changes observed with oligonucleotide arrays
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C. Kooperberg, S. Sipione, M. LeBlanc, A. D. Strand, E. Cattaneo, and J. M. Olson
Evaluating test statistics to select interesting genes in microarray experiments
Hum. Mol. Genet., September 15, 2002; 11(19): 2223 - 2232.
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E. Fossale, V. C. Wheeler, V. Vrbanac, L.-A. Lebel, A. Teed, J. S. Mysore, J. F. Gusella, M. E. MacDonald, and F. Persichetti
Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice
Hum. Mol. Genet., September 15, 2002; 11(19): 2233 - 2241.
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Caspase Cleavage of Mutant Huntingtin Precedes Neurodegeneration in Huntington's Disease
J. Neurosci., September 15, 2002; 22(18): 7862 - 7872.
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R. Luthi-Carter, S. A. Hanson, A. D. Strand, D. A. Bergstrom, W. Chun, N. L. Peters, A. M. Woods, E. Y. Chan, C. Kooperberg, D. Krainc, et al.
Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain
Hum. Mol. Genet., August 15, 2002; 11(17): 1911 - 1926.
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R. Luthi-Carter, A. D. Strand, S. A. Hanson, C. Kooperberg, G. Schilling, A. R. La Spada, D. E. Merry, A. B. Young, C. A. Ross, D. R. Borchelt, et al.
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
Hum. Mol. Genet., August 15, 2002; 11(17): 1927 - 1937.
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E. Y.W. Chan, R. Luthi-Carter, A. Strand, S. M. Solano, S. A. Hanson, M. M. DeJohn, C. Kooperberg, K. O. Chase, M. DiFiglia, A. B. Young, et al.
Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease
Hum. Mol. Genet., August 15, 2002; 11(17): 1939 - 1951.
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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
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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S.-H. Li, A. L. Cheng, H. Zhou, S. Lam, M. Rao, H. Li, and X.-J. Li
Interaction of Huntington Disease Protein with Transcriptional Activator Sp1
Mol. Cell. Biol., March 1, 2002; 22(5): 1277 - 1287.
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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.
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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.
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M. B. Kelz, G. W. Dent, S. Therianos, P. G. Marciano, T. K. McIntosh, P. D. Coleman, and J. H. Eberwine
Single-Cell Antisense RNA Amplification and Microarray Analysis as a Tool for Studying Neurological Degeneration and Restoration
Sci. Aging Knowl. Environ., January 9, 2002; 2002(1): re1 - 1.
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G. J. Klapstein, R. S. Fisher, H. Zanjani, C. Cepeda, E. S. Jokel, M.-F. Chesselet, and M. S. Levine
Electrophysiological and Morphological Changes in Striatal Spiny Neurons in R6/2 Huntington's Disease Transgenic Mice
J Neurophysiol, December 1, 2001; 86(6): 2667 - 2677.
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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
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R. N. Rosenberg
Genomic Neurology: A New Beginning
Arch Neurol, November 1, 2001; 58(11): 1739 - 1741.
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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.
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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.
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A. Wyttenbach, J. Swartz, H. Kita, T. Thykjaer, J. Carmichael, J. Bradley, R. Brown, M. Maxwell, A. Schapira, T. F. Orntoft, et al.
Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease
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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.
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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.
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S. Waelter, A. Boeddrich, R. Lurz, E. Scherzinger, G. Lueder, H. Lehrach, and E. E. Wanker
Accumulation of Mutant Huntingtin Fragments in Aggresome-like Inclusion Bodies as a Result of Insufficient Protein Degradation
Mol. Biol. Cell, May 1, 2001; 12(5): 1393 - 1407.
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F. C. Nucifora Jr., M. Sasaki, M. F. Peters, H. Huang, J. K. Cooper, M. Yamada, H. Takahashi, S. Tsuji, J. Troncoso, V. L. Dawson, et al.
Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity
Science, March 23, 2001; 291(5512): 2423 - 2428.
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S.-H. Li, S. Lam, A. L. Cheng, and X.-J. Li
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Hum. Mol. Genet., November 1, 2000; 9(19): 2859 - 2867.
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J. D. Wood, F. C. Nucifora Jr., K. Duan, C. Zhang, J. Wang, Y. Kim, G. Schilling, N. Sacchi, J. M. Liu, and C. A. Ross
Atrophin-1, the Dentato-Rubral and Pallido-Luysian Atrophy Gene Product, Interacts with Eto/Mtg8 in the Nuclear Matrix and Represses Transcription
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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.
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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
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R. E. Hughes, R. S. Lo, C. Davis, A. D. Strand, C. L. Neal, J. M. Olson, and S. Fields
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