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Behavioral Assessment of Genetic Mouse Models of Huntington’s Disease

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Animal Models of Movement Disorders

Part of the book series: Neuromethods ((NM,volume 62))

Abstract

Huntington’s disease (HD) is a monogenetic, neurodegenerative disease. It is fatal, and although treatments are available for minor symptomatic relief, it remains incurable. Careful study of models of HD remains critical for elucidation of disease mechanisms and for the development of therapeutics. Models that rapidly develop to end stage are useful to assess efficacy of therapeutics for neuroprotection in relatively short experiments. However, the striatum, an area of brain traditionally associated with degeneration in HD already shows extensive atrophy by the time the symptoms manifest in patients, indicating that substantial degeneration occurs in the years preceding clinical onset. Thus, it is vital to also study models that allow analysis of the early pre-manifest disease stage. This requires the development of sensitive output measures to reveal deficits before the onset of obvious anomalies. Here, we briefly review the characteristics of ­several mouse models of HD and outline methods for analysis of behavioral deficits in both severe fast-­progressing models and for early stages of disease in slowly progressive models.

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Notes

  1. 1.

     Transgene: exogenous fragments of DNA. Generally inserted into the host DNA. May be introduced via a viral vector. If present in the germline, can be used to generate a transgenic line.

  2. 2.

     Knock-in: targeted insertion of cDNA into specific site in host organism’s genome.

  3. 3.

     Congenic: To generate a congenic strain mice from two genetically different strains are bred together. Resulting progeny are then bred again to the desired strain, up to 10 times (usually). Speed congenics may reduce the time taken, and involves analysis of the progeny for genetic markers of the desired strain, thus allowing only progeny with the most genetic markers of the desired strain to move forward to breeding.

References

  1. The Huntington’s Disease Collaborative Research Group. (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell 72: 971–983.

    Google Scholar 

  2. Paulsen JS, Langbehn DR, Stout JC et al (2008) Detection of Huntington’s disease decades before diagnosis: the Predict-HD study. J Neurol Neurosurg Psychiatry 79: 874–880.

    Google Scholar 

  3. Vonsattel JP, DiFiglia M (1998) Huntington disease. J Neuropathol Exp Neurol 57: 369–384.

    Google Scholar 

  4. Harper PS (1996) Huntington’s Disease. London, Saunders.

    Google Scholar 

  5. Lemiere J, Decruyenaere M, Evers-Kiebooms G et al (2004) Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation--a longitudinal follow-up study. J Neurol 251: 935–942.

    Google Scholar 

  6. Peinemann A, Schuller S, Pohl C et al (2005) Executive dysfunction in early stages of Huntington’s disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study. J Neurol Sci 239: 11–19.

    Google Scholar 

  7. Watkins LH, Rogers RD, Lawrence AD et al (2000) Impaired planning but intact decision making in early Huntington’s disease: implications for specific fronto-striatal pathology. Neuropsychologia 38: 1112–1125.

    Google Scholar 

  8. Craufurd D, Thompson JC, Snowden JS (2001) Behavioral changes in Huntington Disease. Neuropsychiatry Neuropsychol Behav Neurol 14: 219–226.

    Google Scholar 

  9. Paulsen JS, Nehl C, Hoth KF et al (2005) Depression and stages of Huntington’s disease. J Neuropsychiatry Clin Neurosci 17: 496–502

    Google Scholar 

  10. Tabrizi SJ, Langbehn DR, Leavitt BR et al (2009) Biological and clinical manifestations of Huntington’s disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 8: 791–801.

    Google Scholar 

  11. Walker FO (2007) Huntington’s Disease. Semin Neurol 27: 143–150.

    Google Scholar 

  12. Dumas EM, van den Bogaard SJ, Ruber ME et al (2011) Early changes in white matter pathways of the sensorimotor cortex in premanifest Huntington’s disease. Hum Brain Mapp. doi: 10.1002/hbm.21205. [Epub ahead of print].

    Google Scholar 

  13. Nopoulos PC, Aylward EH, Ross CA et al (2011) Smaller intracranial volume in prodromal Huntington’s disease: evidence for abnormal neurodevelopment. Brain 134: 137–142.

    Google Scholar 

  14. Say MJ, Jones R, Scahill RI et al (2011) Visuomotor integration deficits precede clinical onset in Huntington’s disease. Neuropsy­chologia 49: 264–270.

    Google Scholar 

  15. Stout JC, Paulsen JS, Queller S et al (2011) Neurocognitive signs in prodromal Huntington disease. Neuropsychology 25: 1–14.

    Google Scholar 

  16. Tabrizi SJ, Scahill RI, Durr A et al (2011) Biological and clinical changes in premanifest and early stage Huntington’s disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol 10: 31–42.

    Google Scholar 

  17. Gomez-Tortosa E, MacDonald ME, Friend JC et al (2001) Quantitative neuropathological changes in presymptomatic Huntington’s disease. Ann Neurol 49: 29–34.

    Google Scholar 

  18. Politis M, Pavese N, Tai YF et al (2008) Hypothalamic involvement in Huntington’s disease: an in vivo PET study. Brain 131: 2860–2869.

    Google Scholar 

  19. Rosas HD, Tuch DS, Hevelone ND et al (2006) Diffusion tensor imaging in presymptomatic and early Huntington’s disease: Selective white matter pathology and its relationship to clinical measures. Mov Disord 21: 1317–1325.

    Google Scholar 

  20. Rosas HD, Salat DH, Lee SY et al (2008) Cerebral cortex and the clinical expression of Huntington’s disease: complexity and heterogeneity. Brain 131: 1057–1068.

    Google Scholar 

  21. Factor SA, Weiner WJ (2008) Parkinson’s Disease: Diagnosis and Clinical Management. New York, Demos Medical Publishing.

    Google Scholar 

  22. Louis ED, Lee P, Quinn L et al (1999) Dystonia in Huntington’s disease: prevalence and clinical characteristics. Mov Disord 14: 95–101.

    Google Scholar 

  23. Crossman AR (2000) Functional anatomy of movement disorders. J Anat 196 (Pt 4): 519–525.

    Google Scholar 

  24. Gauthier S (2007) Clinical Diagnosis and Management of Alzheimer’s Disease. Abingdon, Informa Healthcare.

    Google Scholar 

  25. Watson GS, Leverenz JB (2010) Profile of cognitive impairment in Parkinson’s disease. Brain Pathol 20: 640–645.

    Google Scholar 

  26. Bassetti CL (2010) Nonmotor Disturbances in Parkinson’s Disease. Neurodegener Dis. [Epub ahead of print].

    Google Scholar 

  27. Petit D, Gagnon JF, Fantini ML et al (2004) Sleep and quantitative EEG in neurodegenerative disorders. J Psychosom Res 56: 487–496.

    Google Scholar 

  28. Aylward EH (2007) Change in MRI striatal volumes as a biomarker in preclinical Huntington’s disease. Brain Res Bull 72: 152–158.

    Google Scholar 

  29. Levine MS, Cepeda C, Hickey MA et al (2004) Genetic mouse models of Huntington’s and Parkinson’s diseases: illuminating but imperfect. Trends Neurosci 27: 691–697.

    Google Scholar 

  30. Schwarcz R, Guidetti P, Sathyasaikumar KV et al (2009) Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington’s disease. Prog Neurobiol. 90: 230–245

    Google Scholar 

  31. Palfi S, Brouillet E, Jarraya B et al (2007) Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates. Mol Ther 15: 1444–1451.

    Google Scholar 

  32. Yang SH, Cheng PH, Banta H et al (2008) Towards a transgenic model of Huntington’s disease in a non-human primate. Nature 453: 921–924.

    Google Scholar 

  33. Franich NR, Fitzsimons HL, Fong DM et al (2008) AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington’s disease. Mol Ther 16: 947–956.

    Google Scholar 

  34. Hickey MA, Chesselet MF (2003) Apoptosis in Huntington’s disease. Prog Neuropsy­chopharmacol Biol Psychiatry 27: 255–265.

    Google Scholar 

  35. Reiner A, Dragatsis I, Zeitlin S et al (2003) Wild-type huntingtin plays a role in brain development and neuronal survival. Mol Neurobiol 28: 259–276.

    Google Scholar 

  36. Phan J, Hickey MA, Zhang P et al (2009) Adipose tissue dysfunction tracks disease progression in two Huntington’s disease mouse models. Hum Mol Genet 18: 1006–1016.

    Google Scholar 

  37. Sassone J, Colciago C, Cislaghi G et al (2009) Huntington’s disease: the current state of research with peripheral tissues. Exp Neurol 219: 385–397.

    Google Scholar 

  38. Kantor O, Temel Y, Holzmann C et al (2006) Selective striatal neuron loss and alterations in behavior correlate with impaired striatal function in Huntington’s disease transgenic rats. Neurobiol Dis 22: 538–547.

    Google Scholar 

  39. Nguyen HP, Kobbe P, Rahne H et al (2006) Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington’s disease. Hum Mol Genet 15: 3177–3194.

    Google Scholar 

  40. Jacobsen JC, Bawden CS, Rudiger SR et al (2010) An ovine transgenic Huntington’s ­disease model. Hum Mol Genet 19: 1873–1882.

    Google Scholar 

  41. Yang D, Wang CE, Zhao B et al (2010) Expression of Huntington’s disease protein results in apoptotic neurons in the brains of cloned transgenic pigs. Hum Mol Genet 19: 3983–3994.

    Google Scholar 

  42. Morton AJ, Avanzo L (2011) Executive decision-making in the domestic sheep. PLoS One 6: e15752.

    Google Scholar 

  43. Mangiarini L, Sathasivam K, Seller M et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87: 493–506.

    Google Scholar 

  44. Hickey MA, Gallant K, Gross GG et al (2005) Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing. Neurobiol Dis 20: 1–11.

    Google Scholar 

  45. Hickey MA, Kosmalska A, Enayati J et al (2008) Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington’s disease mice. Neuroscience 157: 280–295.

    Google Scholar 

  46. Woodman B, Butler R, Landles C et al (2007) The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes. Brain Res Bull 72: 83–97.

    Google Scholar 

  47. Dragatsis I, Goldowitz D, Del Mar N et al (2009) CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington’s disease transgenic mouse. Neurobiol Dis 33: 315–330.

    Google Scholar 

  48. Morton AJ, Glynn D, Leavens W et al (2009) Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice. Neurobiol Dis 33: 331–341.

    Google Scholar 

  49. Menalled L, El-Khodor BF, Patry M et al (2009) Systematic behavioral evaluation of Huntington’s disease transgenic and knock-in mouse models. Neurobiol Dis 35: 319–336.

    Google Scholar 

  50. Stack EC, Kubilus JK, Smith K et al (2005) Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington’s disease transgenic mice. J Comp Neurol 490: 354–370.

    Google Scholar 

  51. Carter RJ, Hunt MJ, Morton AJ (2000) Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington’s disease gene. Mov Disord 15: 925–937.

    Google Scholar 

  52. van der Burg JM, Bacos K, Wood NI et al (2008) Increased metabolism in the R6/2 mouse model of Huntington’s disease. Neurobiol Dis 29: 41–51.

    Google Scholar 

  53. Weydt P, Pineda VV, Torrence AE et al (2006) Thermoregulatory and metabolic defects in Huntington’s disease transgenic mice implicate PGC-1alpha in Huntington’s disease neurodegeneration. Cell Metab 4: 349–362.

    Google Scholar 

  54. Bolivar VJ, Manley K, Messer A (2003) Exploratory activity and fear conditioning abnormalities develop early in R6/2 Huntington’s disease transgenic mice. Behav Neurosci 117: 1233–1242.

    Google Scholar 

  55. Lione LA, Carter RJ, Hunt MJ et al (1999) Selective discrimination learning impairments in mice expressing the human Huntington’s disease mutation. J Neurosci 19: 10428–10437.

    Google Scholar 

  56. Murphy KPSJ, Carter RJ, Lione LA et al (2000) Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the Huntington’s disease mutation. J. Neurosci. 20: 5115–5123.

    Google Scholar 

  57. Ciamei A, Morton AJ (2009) Progressive imbalance in the interaction between spatial and procedural memory systems in the R6/2 mouse model of Huntington’s disease. Neurobiol Learn Mem 92: 417–428.

    Google Scholar 

  58. File SE, Mahal A, Mangiarini L et al (1998) Striking changes in anxiety in Huntington’s disease transgenic mice. Brain Research 805: 234–240.

    Google Scholar 

  59. Beal MF, Ferrante RJ (2004) Experimental therapeutics in transgenic mouse models of Huntington’s disease. Nat Rev Neurosci 5: 373–384.

    Google Scholar 

  60. Hockly E, Woodman B, Mahal A et al (2003) Standardization and statistical approaches to therapeutic trials in the R6/2 mouse. Brain Res Bull 61: 469–479.

    Google Scholar 

  61. Mihm MJ, Amann DM, Schanbacher BL et al (2007) Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease. Neurobiol Dis 25: 297–308.

    Google Scholar 

  62. Ribchester RR, Thomson D, Wood NI et al (2004) Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington’s disease mutation. Eur J Neurosci 20: 3092–3114.

    Google Scholar 

  63. Brooks SP, Janghra N, Workman VL et al (2011) Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington’s disease transgenic mice. Brain Res Bull.

    Google Scholar 

  64. Naver B, Stub C, Moller M et al (2003) Molecular and behavioral analysis of the R6/1 Huntington’s disease transgenic mouse. Neuroscience 122: 1049–1057.

    Google Scholar 

  65. Pang TY, Du X, Zajac MS et al (2009) Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington’s disease. Hum Mol Genet 18: 753–766.

    Google Scholar 

  66. van Dellen A, Cordery PM, Spires TL et al (2008) Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington’s disease. BMC Neurosci 9: 34.

    Google Scholar 

  67. Lloret A, Dragileva E, Teed A et al (2006) Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington’s disease knock-in mice. Hum Mol Genet 15: 2015–2024.

    Google Scholar 

  68. Hodgson JG, Agopyan N, Gutekunst CA et al (1999) A YAC mouse model for Huntington’s disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23: 181–192.

    Google Scholar 

  69. Slow EJ, van Raamsdonk J, Rogers D et al (2003) Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 12: 1555–1567.

    Google Scholar 

  70. Gray M, Shirasaki DI, Cepeda C et al (2008) Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J Neurosci 28: 6182–6195.

    Google Scholar 

  71. Van Raamsdonk JM, Pearson J, Slow EJ et al (2005) Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington’s disease. J Neurosci 25: 4169–4180.

    Google Scholar 

  72. Van Raamsdonk JM, Murphy Z, Slow EJ et al (2005) Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum Mol Genet 14: 3823–3835.

    Google Scholar 

  73. Stoy N, McKay E (2000) Weight loss in Huntington’s disease. Ann Neurol 48: 130–131.

    Google Scholar 

  74. Van Raamsdonk JM, Gibson WT, Pearson J et al (2006) Body weight is modulated by levels of full-length huntingtin. Hum Mol Genet 15: 1513–1523.

    Google Scholar 

  75. Menalled LB (2005) Knock-in mouse models of Huntington’s disease. NeuroRx 2: 465–470.

    Google Scholar 

  76. Menalled LB, Sison JD, Dragatsis I et al (2003) Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington’s disease with 140 CAG repeats. J Comp Neurol 465: 11–26.

    Google Scholar 

  77. Heng MY, Tallaksen-Greene SJ, Detloff PJ et al (2007) Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington’s disease. J Neurosci 27: 8989–8998.

    Google Scholar 

  78. Lin C-H, Tallaksen-Greene S, Chien W-M et al (2001) Neurological abnormalities in a knock-in mouse model of Huntington’s disease. Human Molecular Genetics 10: 137–144.

    Google Scholar 

  79. Wheeler VC, Gutekunst CA, Vrbanac V et al (2002) Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet 11: 633–640.

    Google Scholar 

  80. Hickey MA, Franich NR, Medvedeva V et al (in press) Mouse models of mental illness and neurological disease: Huntington’s disease. In: The Mouse Nervous System (Watson C, Paxinos G, Puelles L, eds).

    Google Scholar 

  81. Zhu C, Hickey MA, Medvedeva V et al (2009) Comparison of fully backcrossed CAG 140 and CAG 150 Knock-in mouse models of Huntington’s disease: Neuropathological, behavioral, and transcriptional analysis. In: Society for Neuroscience. Chicago: 240.16.

    Google Scholar 

  82. McKhann GM, 2nd, Wenzel HJ, Robbins CA et al (2003) Mouse strain differences in kainic acid sensitivity, seizure behavior, mortality, and hippocampal pathology. Neuroscience 122: 551–561.

    Google Scholar 

  83. Wahlsten D, Bachmanov A, Finn DA et al (2006) Stability of inbred mouse strain differences in behavior and brain size between laboratories and across decades. Proc Natl Acad Sci U S A 103: 16364–16369.

    Google Scholar 

  84. Voikar V, Koks S, Vasar E et al (2001) Strain and gender differences in the behavior of mouse lines commonly used in transgenic studies. Physiol Behav 72: 271–281.

    Google Scholar 

  85. Cryan JF, Mombereau C, Vassout A (2005) The tail suspension test as a model for assessing antidepressant activity: review of pharmacological and genetic studies in mice. Neurosci Biobehav Rev 29: 571–625.

    Google Scholar 

  86. Kent S, Hurd M, Satinoff E (1991) Interactions between body temperature and wheel running over the estrous cycle in rats. Physiol Behav 49: 1079–1084.

    Google Scholar 

  87. Kopp C, Ressel V, Wigger E et al (2006) Influence of estrus cycle and ageing on activity patterns in two inbred mouse strains. Behav Brain Res 167: 165–174.

    Google Scholar 

  88. Viberg H, Fredriksson A, Eriksson P (2004) Investigations of strain and/or gender differences in developmental neurotoxic effects of polybrominated diphenyl ethers in mice. Toxicol Sci 81: 344–353.

    Google Scholar 

  89. Benn CL, Luthi-Carter R, Kuhn A et al (2010) Environmental enrichment reduces neuronal intranuclear inclusion load but has no effect on messenger RNA expression in a mouse model of Huntington disease. J Neuropathol Exp Neurol 69: 817–827.

    Google Scholar 

  90. Hockly E, Cordery PM, Woodman B et al (2002) Environmental enrichment slows disease progression in R6/2 Huntington’s disease mice. Ann Neurol 51: 235–242.

    Google Scholar 

  91. Kurosaki R, Akasaka M, Michimata M et al (2003) Effects of Ca2+ antagonists on motor activity and the dopaminergic system in aged mice. Neurobiol Aging 24: 315–319.

    Google Scholar 

  92. Luesse HG, Schiefer J, Spruenken A et al (2001) Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington’s disease: behavioral testing and impact of diabetes mellitus. Behav Brain Res 126: 185–195.

    Google Scholar 

  93. Menalled LB, Sison JD, Wu Y et al (2002) Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington’s disease knock-in mice. J Neurosci 22: 8266–8276.

    Google Scholar 

  94. Jahkel M, Rilke O, Koch R et al (2000) Open field locomotion and neurotransmission in mice evaluated by principal component factor analysis-effects of housing condition, individual activity disposition and psychotropic drugs. Prog Neuropsychopharmacol Biol Psychiatry 24: 61–84.

    Google Scholar 

  95. Ramos A, Berton O, Mormede P et al (1997) A multiple-test study of anxiety-related behaviours in six inbred rat strains. Behav Brain Res 85: 57–69.

    Google Scholar 

  96. Monville C, Torres EM, Dunnett SB (2006) Comparison of incremental and accelerating protocols of the rotarod test for the assessment of motor deficits in the 6-OHDA model. J Neurosci Methods 158: 219–223.

    Google Scholar 

  97. Kennedy L, Evans E, Chen CM et al (2003) Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis. Hum Mol Genet 12: 3359–3367.

    Google Scholar 

  98. Menalled LB, Patry M, Ragland N et al (2010) Comprehensive behavioral testing in the R6/2 mouse model of Huntington’s disease shows no benefit from CoQ10 or minocycline. PLoS One 5: e9793.

    Google Scholar 

  99. Southwell AL, Ko J, Patterson PH (2009) Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington’s disease. J Neurosci 29: 13589–13602.

    Google Scholar 

  100. Cepeda C, Cummings DM, Hickey MA et al (2010) Rescuing the Corticostriatal Synaptic Disconnection in the R6/2 Mouse Model of Huntington’s Disease: Exercise, Adenosine Receptors and Ampakines. PLoS Curr 2: RRN1182.

    Google Scholar 

  101. Fernagut PO, Hutson CB, Fleming SM et al (2007) Behavioral and histopathological consequences of paraquat intoxication in mice: effects of alpha-synuclein over-expression. Synapse 61: 991–1001.

    Google Scholar 

  102. Fleming SM, Salcedo J, Fernagut PO et al (2004) Early and progressive sensorimotor anomalies in mice overexpressing wild-type human alpha-synuclein. J Neurosci 24: 9434–9440.

    Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, David Geffen School of Medicine, UCLA, Los Angeles, CA, USA

    Miriam A. Hickey & Marie-Françoise Chesselet

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  1. Miriam A. Hickey
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  2. Marie-Françoise Chesselet
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Correspondence to Marie-Françoise Chesselet .

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  1. , Welsh School of Pharmacology, Cardiff University, Edward VII Avenue, Cardiff, CF10 3NB, United Kingdom

    Emma L. Lane

  2. School of Biosciences, Brain Repair Group, Cardiff University, Museum Avenue, Cardiff, CF10 3AX, United Kingdom

    Stephen B. Dunnett

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Hickey, M.A., Chesselet, MF. (2011). Behavioral Assessment of Genetic Mouse Models of Huntington’s Disease. In: Lane, E., Dunnett, S. (eds) Animal Models of Movement Disorders. Neuromethods, vol 62. Humana Press. https://doi.org/10.1007/978-1-61779-301-1_1

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