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Previous Article | Next Article
The Journal of Neuroscience, April 15, 1999, 19(8):3248-3257
Characterization of Progressive Motor Deficits in Mice Transgenic for the Human Huntington's Disease Mutation
Rebecca J. Carter1, Lisa A. Lione2, 3, Trevor Humby2, Laura Mangiarini5, Amarbirpal Mahal5, Gillian P. Bates5, Stephen B. Dunnett2, 4, and A. Jennifer Morton1 1 Department of Pharmacology, 2 Centre for Brain Repair, 3 Parke-Davis Neuroscience Research, and 4 Department of Experimental Psychology, University of Cambridge, Cambridge, CB2 1QJ, United Kingdom, and 5 Division of Medical and Molecular Genetics, Guy's Hospital, London SE1 9RT, United Kingdom
Transgenic mice expressing exon 1 of the human Huntington's disease (HD) gene carrying a 141-157 CAG repeat (line R6/2) develop a progressive neurological phenotype with motor symptoms resembling those seen in HD. We have characterized the motor deficits in R6/2 mice using a battery of behavioral tests selected to measure motor aspects of swimming, fore- and hindlimb coordination, balance, and sensorimotor gating [swimming tank, rotarod, raised beam, fore- and hindpaw footprinting, and acoustic startle/prepulse inhibition (PPI)]. Behavioral testing was performed on female hemizygotic R6/2 transgenic mice (n = 9) and female wild-type littermates (n = 22) between 5 and 14 weeks of age. Transgenic mice did not show an overt behavioral phenotype until around 8 weeks of age. However, as early as 5-6 weeks of age they had significant difficulty swimming, traversing the narrowest square (5 mm) raised beam, and maintaining balance on the rotarod at rotation speeds of 33-44 rpm. Furthermore, they showed significant impairment in prepulse inhibition (an impairment also seen in patients with HD). Between 8 and 15 weeks, R6/2 transgenic mice showed a progressive deterioration in performance on all of the motor tests. Thus R6/2 mice show measurable deficits in motor behavior that begin subtly and increase progressively until death. Our data support the use of R6/2 mice as a model of HD and indicate that they may be useful for evaluating therapeutic strategies for HD, particularly those aimed at reducing the severity of motor symptoms or slowing the course of the disease.
Key words: transgenic mice; Huntington's disease; CAG repeat; motor behavior; prepulse inhibition; sensorimotor gating; polyglutamine repeat diseases
Copyright © 1999 Society for Neuroscience 0270-6474/99/1983248-10$05.00/0
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Mol. Cell. Proteomics, May 1, 2002; 1(5): 366 - 375.
[Abstract] [Full Text] [PDF]
Q. Ding, J. J. Lewis, K. M. Strum, E. Dimayuga, A. J. Bruce-Keller, J. C. Dunn, and J. N. Keller
Polyglutamine Expansion, Protein Aggregation, Proteasome Activity, and Neural Survival
J. Biol. Chem., April 12, 2002; 277(16): 13935 - 13942.
[Abstract] [Full Text] [PDF]
D. M. Koeller, M. Woontner, L. S. Crnic, B. Kleinschmidt-DeMasters, J. Stephens, E. L. Hunt, and S. I. Goodman
Biochemical, pathologic and behavioral analysis of a mouse model of glutaric acidemia type I
Hum. Mol. Genet., February 1, 2002; 11(4): 347 - 357.
[Abstract] [Full Text] [PDF]
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.
[Abstract] [Full Text] [PDF]
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]
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]
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]
L. M. Mende-Mueller, T. Toneff, S.-R. Hwang, M.-F. Chesselet, and V. Y. H. Hook
Tissue-Specific Proteolysis of Huntingtin (htt) in Human Brain: Evidence of Enhanced Levels of N- and C-Terminal htt Fragments in Huntington's Disease Striatum
J. Neurosci., March 15, 2001; 21(6): 1830 - 1837.
[Abstract] [Full Text] [PDF]
J. N. Fain, N. A. Del Mar, C. A. Meade, A. Reiner, and D. Goldowitz
Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington's disease mutation
Hum. Mol. Genet., January 1, 2001; 10(2): 145 - 152.
[Abstract] [Full Text] [PDF]
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]
N. R. Swerdlow, Z. A. Martinez, F. M. Hanlon, A. Platten, M. Farid, P. Auerbach, D. L. Braff, and M. A. Geyer
Toward Understanding the Biology of a Complex Phenotype: Rat Strain and Substrain Differences in the Sensorimotor Gating-Disruptive Effects of Dopamine Agonists
J. Neurosci., June 1, 2000; 20(11): 4325 - 4336.
[Abstract] [Full Text] [PDF]
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]
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]
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]
J. A. Bibb, Z. Yan, P. Svenningsson, G. L. Snyder, V. A. Pieribone, A. Horiuchi, A. C. Nairn, A. Messer, and P. Greengard
Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice
PNAS, June 6, 2000; 97(12): 6809 - 6814.
[Abstract] [Full Text] [PDF]
G. V. Rebec, S. J. Barton, and M. D. Ennis
Dysregulation of Ascorbate Release in the Striatum of Behaving Mice Expressing the Huntington's Disease Gene
J. Neurosci., January 15, 2002; 22(2): RC202 - RC202.
[Abstract] [Full Text] [PDF]
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