- About this Journal ·
- Abstracting and Indexing ·
- Advance Access ·
- Aims and Scope ·
- Annual Issues ·
- Article Processing Charges ·
- Articles in Press ·
- Author Guidelines ·
- Bibliographic Information ·
- Citations to this Journal ·
- Contact Information ·
- Editorial Board ·
- Editorial Workflow ·
- Free eTOC Alerts ·
- Publication Ethics ·
- Reviewers Acknowledgment ·
- Submit a Manuscript ·
- Subscription Information ·
- Table of Contents
Volume 2012 (2012), Article ID 682712, 8 pages
Lessons Learned from the Transgenic Huntington's Disease Rats
1Department of Neuroscience, Maastricht University Medical Center, 6229 ER Maastricht, The Netherlands
2Department of Neurology, Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands
3Department of Neurosurgery, Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands
Received 13 February 2012; Revised 21 May 2012; Accepted 4 June 2012
Academic Editor: Emma Frost
Copyright © 2012 Rinske Vlamings et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
- F. O. Walker, “Huntington's disease,” The Lancet, vol. 369, no. 9557, pp. 218–228, 2007.
- R. M. Bonelli and P. Hofmann, “A systematic review of the treatment studies in Huntington's disease since 1990,” Expert Opinion on Pharmacotherapy, vol. 8, no. 2, pp. 141–153, 2007.
- R. H. Myers, “Huntington's disease genetics,” NeuroRx, vol. 1, no. 2, pp. 255–262, 2004.
- J. M. Andresen, J. Gayan, L. Djousse et al., “The relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset,” Annals of Human Genetics, vol. 71, no. 3, pp. 295–301, 2007.
- S. E. Andrew, Y. P. Goldberg, B. Kremer et al., “The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease,” Nature Genetics, vol. 4, no. 4, pp. 398–403, 1993.
- J. F. Gusella, N. S. Wexler, and P. M. Conneally, “A polymorphic DNA marker genetically linked to Huntington's disease,” Nature, vol. 306, no. 5940, pp. 234–238, 1983.
- M. E. MacDonald, C. M. Ambrose, M. P. Duyao et al., “A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes,” Cell, vol. 72, no. 6, pp. 971–983, 1993.
- R. Vlamings, A. Benazzouz, J. Chetrit, et al., “Metabolic and electrophysiological changes in the basal ganglia of transgenic Huntington's disease rats,” Neurobiology of Disease. In press.
- O. Kantor, Y. Temel, C. Holzmann et al., “Selective striatal neuron loss and alterations in behavior correlate with impaired striatal function in Huntington's disease transgenic rats,” Neurobiology of Disease, vol. 22, no. 3, pp. 538–547, 2006.
- A. Jahanshahi, R. Vlamings, A. H. Kaya et al., “Hyperdopaminergic status in experimental Huntington disease,” Journal of Neuropathology and Experimental Neurology, vol. 69, no. 9, pp. 910–917, 2010.
- G. R. Jackson, I. Salecker, X. Dong et al., “Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons,” Neuron, vol. 21, no. 3, pp. 633–642, 1998.
- P. W. Faber, C. Voisine, D. C. King, E. A. Bates, and A. C. Hart, “Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity,” Proceedings of the National Academy of Sciences of the United States of America, vol. 99, no. 26, pp. 17131–17136, 2002.
- J. C. Jacobsen, C. S. Bawden, S. R. Rudiger et al., “An ovine transgenic Huntington's disease model,” Human Molecular Genetics, vol. 19, no. 10, Article ID ddq063, pp. 1873–1882, 2010.
- A. J. Morton and L. Avanzo, “Executive decision-making in the domestic sheep,” PloS one, vol. 6, no. 1, p. e15752, 2011.
- D. Yang, C. E. Wang, B. Zhao et al., “Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs,” Human Molecular Genetics, vol. 19, no. 20, Article ID ddq313, pp. 3983–3994, 2010.
- S. H. Yang, P. H. Cheng, H. Banta et al., “Towards a transgenic model of Huntington's disease in a non-human primate,” Nature, vol. 453, no. 7197, pp. 921–924, 2008.
- S. von Horsten, I. Schmitt, H. P. Nguyen et al., “Transgenic rat model of Huntington's disease,” Human Molecular Genetics, vol. 12, no. 6, pp. 617–624, 2003.
- G. Huntington, “On Chorea,” The Medical and Surgical Reporter, vol. 26, no. 4, 1872.
- J. S. Paulsen, R. E. Ready, J. M. Hamilton, M. S. Mega, and J. L. Cummings, “Neuropsychiatric aspects of Huntington's disease,” Journal of Neurology Neurosurgery and Psychiatry, vol. 71, no. 3, pp. 310–314, 2001.
- N. Reedeker, R. C. Van Der Mast, E. J. Giltay, E. Van Duijn, and R. A. C. Roos, “Hypokinesia in Huntington's disease co-occurs with cognitive and global dysfunctioning,” Movement Disorders, vol. 25, no. 11, pp. 1612–1618, 2010.
- S. E. File, A. Mahal, L. Mangiarini, and G. P. Bates, “Striking changes in anxiety in Huntington's disease transgenic mice,” Brain Research, vol. 805, no. 1-2, pp. 234–240, 1998.
- H. P. Nguyen, P. Kobbe, H. Rahne et al., “Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease,” Human Molecular Genetics, vol. 15, no. 21, pp. 3177–3194, 2006.
- D. H. Zeef, R. Vlamings, L. W. Lim et al., “Motor and non-motor behaviour in experimental Huntington's disease,” Behavioural Brain Research, vol. 226, no. 2, pp. 435–439, 2012.
- D. H. Zeef, N. P. van Goethem, R. Vlamings et al., “Memory deficits in the transgenic rat model of Huntington's disease,” Behavioural Brain Research, vol. 227, no. 1, pp. 194–198, 2012.
- A. Ciamei and A. Jennifer Morton, “Progressive imbalance in the interaction between spatial and procedural memory systems in the R6/2 mouse model of Huntington's disease,” Neurobiology of Learning and Memory, vol. 92, no. 3, pp. 417–428, 2009.
- J. A. Grahn, J. A. Parkinson, and A. M. Owen, “The role of the basal ganglia in learning and memory: neuropsychological studies,” Behavioural Brain Research, vol. 199, no. 1, pp. 53–60, 2009.
- V. Ghiglieri, C. Sgobio, C. Costa, B. Picconi, and P. Calabresi, “Striatum-hippocampus balance: from physiological behavior to interneuronal pathology,” Progress in Neurobiology, vol. 94, no. 2, pp. 102–114, 2011.
- D. G. Mumby, P. Piterkin, V. Lecluse, and H. Lehmann, “Perirhinal cortex damage and anterograde object-recognition in rats after long retention intervals,” Behavioural Brain Research, vol. 185, no. 2, pp. 82–87, 2007.
- H. Eichenbaum, A. P. Yonelinas, and C. Ranganath, “The medial temporal lobe and recognition memory,” Annual Review of Neuroscience, vol. 30, pp. 123–152, 2007.
- H. Abe, Y. Ishida, H. Nonaka, and T. Iwasaki, “Functional difference between rat perirhinal cortex and hippocampus in object and place discrimination tasks,” Behavioural Brain Research, vol. 197, no. 2, pp. 388–397, 2009.
- A. Giralt, T. Rodrigo, E. D. Martín et al., “Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: involvement of phospholipaseCγ activity and glutamate receptor expression,” Neuroscience, vol. 158, no. 4, pp. 1234–1250, 2009.
- K. P. S. J. Murphy, R. J. Carter, L. A. Lione et al., “Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation,” Journal of Neuroscience, vol. 20, no. 13, pp. 5115–5123, 2000.
- A. J. Milnerwood and L. A. Raymond, “Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load,” Journal of Physiology, vol. 585, no. 3, pp. 817–831, 2007.
- D. A. Simmons, C. S. Rex, L. Palmer et al., “Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice,” Proceedings of the National Academy of Sciences of the United States of America, vol. 106, no. 12, pp. 4906–4911, 2009.
- S. Höhn, G. Dallérac, A. Faure et al., “Behavioral and in vivo electrophysiological evidence for presymptomatic alteration of prefrontostriatal processing in the transgenic rat model for huntington disease,” Journal of Neuroscience, vol. 31, no. 24, pp. 8986–8997, 2011.
- C. Cao, Y. Temel, A. Blokland et al., “Progressive deterioration of reaction time performance and choreiform symptoms in a new Huntington's disease transgenic ratmodel,” Behavioural Brain Research, vol. 170, no. 2, pp. 257–261, 2006.
- J. O. Dostrovsky and A. M. Lozano, “Mechanisms of deep brain stimulation,” Movement Disorders, vol. 17, supplement 3, pp. S63–S68, 2002.
- N. Urbain, N. Rentéro, D. Gervasoni, B. Renaud, and G. Chouvet, “The switch of subthalamic neurons from an irregular to a bursting pattern does not solely depend on their GABAergic inputs in the anesthetic-free rat,” Journal of Neuroscience, vol. 22, no. 19, pp. 8665–8675, 2002.
- P. J. Magill, J. P. Bolam, and M. D. Bevan, “Relationship of activity in the subthalamic nucleus-globus pallidus network to cortical electroencephalogram,” Journal of Neuroscience, vol. 20, no. 2, pp. 820–833, 2000.
- A. Benazzouz and M. Hallett, “Mechanism of action of deep brain stimulation,” Neurology, vol. 55, no. 12, pp. S13–S16, 2000.
- X. Liu, H. L. Ford-Dunn, G. N. Hayward et al., “The oscillatory activity in the Parkinsonian subthalamic nucleus investigated using the macro-electrodes for deep brain stimulation,” Clinical Neurophysiology, vol. 113, no. 11, pp. 1667–1672, 2002.
- M. D. Bevan, P. J. Magill, D. Terman, J. P. Bolam, and C. J. Wilson, “Move to the rhythm: oscillations in the subthalamic nucleus-external globus pallidus network,” Trends in Neurosciences, vol. 25, no. 10, pp. 525–531, 2002.
- W. C. Miller and M. R. DeLong, “Altered tonic activity of neurons in the globus pallidus and subthalamic nucleus in the primate model of parkinsonism,” in The Basal Ganglia II: Structure and Function, M. B. Carpenter and A. Jayaraman, Eds., pp. 415–427, Plenum, New York, NY, USA, 1987.
- H. Bergman, T. Wichmann, and M. R. DeLong, “Reversal of experimental Parkinsonism by lesions of the subthalamic nucleus,” Science, vol. 249, no. 4975, pp. 1436–1438, 1990.
- A. L. Benabid, P. Pollak, A. Louveau, S. Henry, and J. De Rougemont, “Combined (thalamotomy and stimulation) stereotactic surgery of the VIM thalamic nucleus for bilateral Parkinson disease,” Applied Neurophysiology, vol. 50, no. 1–6, pp. 344–346, 1987.
- A. Benazzouz, C. Gross, J. Feger, T. Boraud, and B. Bioulac, “Reversal of rigidity and improvement in motor performance by subthalamic high-frequency stimulation in MPTP-treated monkeys,” European Journal of Neuroscience, vol. 5, no. 4, pp. 382–389, 1993.
- P. Pollak, A. L. Benabid, C. Gross et al., “Effects of subthalamic nucleus stimulation in Parkinson's disease,” Revue Neurologique, vol. 149, no. 3, pp. 175–176, 1993.
- P. Limousin, P. Pollak, A. Benazzouz et al., “Effect on parkinsonian signs and symptoms of bilateral subthalamic nucleus stimulation,” The Lancet, vol. 345, no. 8942, pp. 91–95, 1995.
- P. Puigserver and B. M. Spiegelman, “Peroxisome proliferator-activated receptor-γ coactivator 1α (PGC-1α): transcriptional coactivator and metabolic regulator,” Endocrine Reviews, vol. 24, no. 1, pp. 78–90, 2003.
- D. Joel, “Open interconnected model of basal ganglia-thalamocortical circuitry and its relevance to the clinical syndrome of Huntington's disease,” Movement Disorders, vol. 16, no. 3, pp. 407–423, 2001.
- Y. Temel, A. Blokland, H. W. M. Steinbusch, and V. Visser-Vandewalle, “The functional role of the subthalamic nucleus in cognitive and limbic circuits,” Progress in Neurobiology, vol. 76, no. 6, pp. 393–413, 2005.
- Z. Zhu, M. Bartol, K. Shen, and S. W. Johnson, “Excitatory effects of dopamine on subthalamic nucleus neurons: In vitro study of rats pretreated with 6-hydroxydopamine and levodopa,” Brain Research, vol. 945, no. 1, pp. 31–40, 2002.
- H. Heinsen, M. Strik, M. Bauer et al., “Cortical and striatal neurone number in Huntington's disease,” Acta Neuropathologica, vol. 88, no. 4, pp. 320–333, 1994.
- M. F. Beal and R. J. Ferrante, “Experimental therapeutics in transgenic mouse models of Huntington's disease,” Nature Reviews Neuroscience, vol. 5, no. 5, pp. 373–384, 2004.
- E. J. Slow, J. van Raamsdonk, D. Rogers et al., “Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease,” Human Molecular Genetics, vol. 12, no. 13, pp. 1555–1567, 2003.
- J. P. Vonsattel, R. H. Myers, and T. J. Stevens, “Neuropathological classification of Huntington's disease,” Journal of Neuropathology and Experimental Neurology, vol. 44, no. 6, pp. 559–577, 1985.
- B. R. Miller, A. G. Walker, S. C. Fowler et al., “Dysregulation of coordinated neuronal firing patterns in striatum of freely behaving transgenic rats that model Huntington's disease,” Neurobiology of Disease, vol. 37, no. 1, pp. 106–113, 2010.
- V. MacDonald and G. M. Halliday, “Selective loss of pyramidal neurons in the pre-supplementary motor cortex in Parkinson's disease,” Movement Disorders, vol. 17, no. 6, pp. 1166–1173, 2002.
- E. D. Bird, “Chemical pathology of Huntington's disease,” Annual Review of Pharmacology and Toxicology, vol. 20, pp. 533–551, 1980.
- E. D. Bird, E. G. Spokes, and L. L. Iversen, “Dopamine and noradrenaline in post-mortem brain in Huntington's disease and schizophrenic illness,” Acta Psychiatrica Scandinavica, vol. 280, pp. 63–73, 1980.
- E. G. S. Spokes, “Neurochemical alterations in Huntington's chorea. A study of post mortem brain tissue,” Brain, vol. 103, no. 1, pp. 179–210, 1980.
- S. L. Mason and R. A. Barker, “Emerging drug therapies in Huntington's disease,” Expert Opinion on Emerging Drugs, vol. 14, no. 2, pp. 273–297, 2009.
- S. N. Haber and J. L. Fudge, “The primate substantia Nigra and VTA: integrative circuitry and function,” Critical Reviews in Neurobiology, vol. 11, no. 4, pp. 323–342, 1997.
- D. Joel and I. Weiner, “The connections of the dopaminergic system with the striatum in rats and primates: an analysis with respect to the functional and compartmental organization of the striatum,” Neuroscience, vol. 96, no. 3, pp. 451–474, 2000.
- G. J. Yohrling, G. C. T. Jiang, M. M. DeJohn et al., “Analysis of cellular, transgenic and human models of Huntington's disease reveals tyrosine hydroxylase alterations and substantia nigra neuropathology,” Molecular Brain Research, vol. 119, no. 1, pp. 28–36, 2003.
- D. M. A. Mann, “Subcortical afferent projection systems in Huntington's chorea,” Acta Neuropathologica, vol. 78, no. 5, pp. 551–554, 1989.
- Y. Temel, V. Visser-Vandewalle, S. Kaplan et al., “Protection of nigral cell death by bilateral subthalamic nucleus stimulation,” Brain Research, vol. 1120, no. 1, pp. 100–105, 2006.
- T. D. Aumann, I. Gantois, K. Egan et al., “SK channel function regulates the dopamine phenotype of neurons in the substantia nigra pars compacta,” Experimental Neurology, vol. 213, no. 2, pp. 419–430, 2008.
- G. Paul, W. Meissner, S. Rein et al., “Ablation of the subthalamic nucleus protects dopaminergic phenotype but not cell survival in a rat model of Parkinson's disease,” Experimental Neurology, vol. 185, no. 2, pp. 272–280, 2004.
- F. J. Bode, M. Stephan, H. Suhling et al., “Sex differences in a transgenic rat model of Huntington's disease: decreased 17β-estradiol levels correlate with reduced numbers of DARPP32+ neurons in males,” Human Molecular Genetics, vol. 17, no. 17, pp. 2595–2609, 2008.
- S. A. Fielding, S. P. Brooks, A. Klein, Z. Bayram-Weston, L. Jones, and S. B. Dunnett, “Profiles of motor and cognitive impairment in the transgenic rat model of Huntington's disease,” Brain Research Bulletin, vol. 88, no. 2-3, pp. 223–236, 2012.
- S. Brooks, S. Fielding, M. Döbrössy, S. von Hörsten, and S. Dunnett, “Subtle but progressive cognitive deficits in the female tgHD hemizygote rat as demonstrated by operant SILT performance,” Brain Research Bulletin, vol. 79, no. 5, pp. 310–315, 2009.