Human Molecular Genetics, Vol 8, 397-407, Copyright © 1999 by Oxford University Press
G Schilling, MW Becher, AH Sharp, HA Jinnah, K Duan, JA Kotzuk, HH Slunt, T Ratovitski, JK Cooper, NA Jenkins, NG Copeland, DL Price, CA Ross and DR Borchelt
Huntington's disease (HD) is an inherited, neurodegenerative disorder
caused by the expansion of a glutamine repeat in the N-terminus of the
huntingtin protein. To gain insight into the pathogenesis of HD, we
generated transgenic mice that express a cDNA encoding an N-terminal
fragment (171 amino acids) of huntingtin with 82, 44 or 18 glutamines. Mice
expressing relatively low steady-state levels of N171 huntingtin with 82
glutamine repeats (N171-82Q) develop behavioral abnormalities, including
loss of coordination, tremors, hypokinesis and abnormal gait, before dying
prematurely. In mice exhibiting these abnormalities, diffuse nuclear
labeling, intranuclear inclusions and neuritic aggregates, all
immunoreactive with an antibody to the N-terminus (amino acids 1-17) of
huntingtin (AP194), were found in multiple populations of neurons. None of
these behavioral or pathological phenotypes were seen in mice expressing
N171-18Q. These findings are consistent with the idea that N-terminal
fragments of huntingtin with a repeat expansion are toxic to neurons, and
that N-terminal fragments are prone to form both intranuclear inclusions
and neuritic aggregates.
ARTICLES
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin [published erratum appears in Hum Mol Genet 1999 May;8(5):943]
Department of Psychiatry, Division of Neuropathology, Johns Hopkins University, Baltimore, MD 21205-2196, USA.
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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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 M. Yamada, T. Sato, T. Shimohata, S. Hayashi, S. Igarashi, S. Tsuji, and H. Takahashi Interaction between Neuronal Intranuclear Inclusions and Promyelocytic Leukemia Protein Nuclear and Coiled Bodies in CAG Repeat Diseases Am. J. Pathol., November 1, 2001; 159(5): 1785 - 1795. [Abstract] [Full Text] [PDF]
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W. Auerbach, M. S. Hurlbert, P. Hilditch-Maguire, Y. Z. Wadghiri, V. C. Wheeler, S. I. Cohen, A. L. Joyner, M. E. MacDonald, and D. H. Turnbull The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin Hum. Mol. Genet., October 1, 2001; 10(22): 2515 - 2523. [Abstract] [Full Text] [PDF]
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C. Lesuisse, G. Xu, J. Anderson, M. Wong, J. Jankowsky, G. Holtz, V. Gonzalez, P. C. Y. Wong, D. L. Price, F. Tang, et al. Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice Hum. Mol. Genet., October 1, 2001; 10(22): 2525 - 2537. [Abstract] [Full Text] [PDF]
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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 Hum. Mol. Genet., August 1, 2001; 10(17): 1829 - 1845. [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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W. Chun, M. Lesort, J. Tucholski, C. A. Ross, and G. V.W. Johnson Tissue Transglutaminase Does Not Contribute to the Formation of Mutant Huntingtin Aggregates J. Cell Biol., April 2, 2001; 153(1): 25 - 34. [Abstract] [Full Text] [PDF]
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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. [Abstract] [Full Text]
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A. Abel, J. Walcott, J. Woods, J. Duda, and D. E. Merry Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice Hum. Mol. Genet., January 1, 2001; 10(2): 107 - 116. [Abstract] [Full Text] [PDF]
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G. Cancel, C. Duyckaerts, M. Holmberg, C. Zander, G. Yvert, A.-S. Lebre, M. Ruberg, B. Faucheux, Y. Agid, E. Hirsch, et al. Distribution of ataxin-7 in normal human brain and retina Brain, December 1, 2000; 123(12): 2519 - 2530. [Abstract] [Full Text] [PDF]
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G. Yvert, K. S. Lindenberg, S. Picaud, G. B. Landwehrmeyer, J.-A. Sahel, and J.-L. Mandel Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice Hum. Mol. Genet., October 1, 2000; 9(17): 2491 - 2506. [Abstract] [Full Text] [PDF]
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K. P. S. J. Murphy, R. J. Carter, L. A. Lione, L. Mangiarini, A. Mahal, G. P. Bates, S. B. Dunnett, and A. J. Morton Abnormal Synaptic Plasticity and Impaired Spatial Cognition in Mice Transgenic for Exon 1 of the Human Huntington's Disease Mutation J. Neurosci., July 1, 2000; 20(13): 5115 - 5123. [Abstract] [Full Text] [PDF]
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R. Luthi-Carter, A. Strand, N. L. Peters, S. M. Solano, Z. R. Hollingsworth, A. S. Menon, A. S. Frey, B. S. Spektor, E. B. Penney, G. Schilling, et al. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease Hum. Mol. Genet., May 22, 2000; 9(9): 1259 - 1271. [Abstract] [Full Text] [PDF]
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A. Wyttenbach, J. Carmichael, J. Swartz, R. A. Furlong, Y. Narain, J. Rankin, and D. C. Rubinsztein Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease PNAS, March 14, 2000; 97(6): 2898 - 2903. [Abstract] [Full Text] [PDF]
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V. C. Wheeler, J. K. White, C.-A. Gutekunst, V. Vrbanac, M. Weaver, X.-J. Li, S.-H. Li, H. Yi, J.-P. Vonsattel, J. F. Gusella, et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice Hum. Mol. Genet., March 1, 2000; 9(4): 503 - 513. [Abstract] [Full Text] [PDF]
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L. A. Lione, R. J. Carter, M. J. Hunt, G. P. Bates, A. J. Morton, and S. B. Dunnett Selective Discrimination Learning Impairments in Mice Expressing the Human Huntington's Disease Mutation J. Neurosci., December 1, 1999; 19(23): 10428 - 10437. [Abstract] [Full Text] [PDF]
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H. Li, S.-H. Li, A. L. Cheng, L. Mangiarini, G. P. Bates, and X.-J. Li Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice Hum. Mol. Genet., July 1, 1999; 8(7): 1227 - 1236. [Abstract] [Full Text] [PDF]
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M. J. Miller, H. Rauer, H. Tomita, H. Rauer, J. J. Gargus, G. A. Gutman, M. D. Cahalan, and K. G. Chandy Nuclear Localization and Dominant-negative Suppression by a Mutant SKCa3 N-terminal Channel Fragment Identified in a Patient with Schizophrenia J. Biol. Chem., July 20, 2001; 276(30): 27753 - 27756. [Abstract] [Full Text] [PDF]
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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. [Abstract] [Full Text] [PDF]
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J. A. Parker, J. B. Connolly, C. Wellington, M. Hayden, J. Dausset, and C. Neri Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death PNAS, November 6, 2001; 98(23): 13318 - 13323. [Abstract] [Full Text] [PDF]
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