Mouse models of Huntington’s disease

Current Topics in Behavioral Neurosciences

Volumn 22, Issue , 2014, Pages 101-133

  Brooks, Simon P ^a   Dunnett, Stephen B ^a  

^a CARDIFF UNIVERSITY   (United Kingdom)

Author keywords

Behaviour; Cognitive; Huntington s disease; Motor; Mouse; Neuropathology; Psychiatric

Indexed keywords

4 AMINOBUTYRIC ACID RECEPTOR; AMPA RECEPTOR; BRAIN DERIVED NEUROTROPHIC FACTOR; CANNABINOID 1 RECEPTOR; DOPAMINE 1 RECEPTOR; DOPAMINE 2 RECEPTOR; N METHYL DEXTRO ASPARTIC ACID RECEPTOR;

ANIMAL BEHAVIOR; ANIMAL MODEL; ARTICLE; ATTENTION DISTURBANCE; BASAL GANGLION; BEHAVIOR DISORDER; COGNITION; COGNITIVE DEFECT; CORPUS STRIATUM; FRONTAL CORTEX; HUMAN; HUNTINGTON CHOREA; LONG TERM DEPRESSION; LONG TERM POTENTIATION; MOTOR CONTROL; MOTOR COORDINATION; MOTOR DYSFUNCTION; MOTOR PERFORMANCE; MOUSE; NEUROBIOLOGY; NEUROPATHOLOGY; NONHUMAN; PRIORITY JOURNAL; THALAMUS; THALAMUS NUCLEUS; ANIMAL; COMPARATIVE STUDY; DISEASE MODEL; PATHOPHYSIOLOGY;

ANIMALS; DISEASE MODELS, ANIMAL; HUMANS; HUNTINGTON DISEASE; MICE;

EID: 84946030182     PISSN: 18663370     EISSN: 18663389     Source Type: Book Series    
DOI: 10.1007/7854_2013_256     Document Type: Article

References (256)

1. Abada YS, Schreiber R, Ellenbroek B (2013) Motor, emotional and cognitive deficits in adult BACHD mice: a model for Huntington's disease. Behav Brain Res 238:243-251
2. Agnoli L, Mainolfi P, Invernizzi RW, Carli M (2013) Dopamine D1-like and D2-like receptors in the dorsal striatum control different aspects of attentional performance in the five-choice serial reaction time task under a condition of increased activity of corticostriatal inputs. Neuropsychopharmacology 38:701-714
3. Albasser MM, Poirier GL, Aggleton JP (2010) Qualitatively different modes of perirhinalhippocampal engagement when rats explore novel vs. familiar objects as revealed by c-Fos imaging. Eur J Neurosci 31:134-147
4. Alexander GE, Crutcher MD, DeLong MR (1990) Basal ganglia-thalamocortical circuits: parallel substrates for motor, oculomotor, "prefrontal'' and "limbic'' functions. Prog Brain Res 85:119-146
5. Alexander GE, DeLong MR, Strick PL (1986) Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annu Rev Neurosci 9:357-381
6. Allen KL, Waldvogel HJ, Glass M, Faull RL (2009) Cannabinoid (CB(1)), GABA(A) and GABA(B) receptor subunit changes in the globus pallidus in Huntington's disease. J Chem Neuroanat 37:266-281
7. Andre VM, Cepeda C, Venegas A, Gomez Y, Levine MS (2006) Altered cortical glutamate receptor function in the R6/2 model of Huntington's disease. J Neurophysiol 95:2108-2119
8. Andre VM, Fisher YE, Levine MS (2011) Altered balance of activity in the striatal direct and indirect pathways in mouse models of Huntington's disease. Front Syst Neurosci 5:46
9. Ariano MA, Aronin N, DiFiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, Levine MS (2002) Striatal neurochemical changes in transgenic models of Huntington's disease. J Neurosci Res 68:716-729
10. Aylward EH, Nopoulos PC, Ross CA, Langbehn DR, Pierson RK, Mills JA, Johnson HJ, Magnotta VA, Juhl AR, Paulsen JS (2011) Longitudinal change in regional brain volumes in prodromal Huntington disease. J Neurol Neurosurg Psychiatry 82:405-410
11. Baquet ZC, Gorski JA, Jones KR (2004) Early striatal dendrite deficits followed by neuron loss with advanced age in the absence of anterograde cortical brain-derived neurotrophic factor. J Neurosci 24:4250-4258
12. Bari A, Robbins TW (2013) Inhibition and impulsivity: behavioral and neural basis of response control. Prog Neurobiol 108:44-79
13. Bari M, Battista N, Valenza M, Mastrangelo N, Malaponti M, Catanzaro G, Centonze D, Finazzi-Agro A, Cattaneo E, Maccarrone M (2013) In vitro and in vivo models of Huntington's disease show alterations in the endocannabinoid system. FEBS J 280(14):3376-88
14. Baudic S, Maison P, Dolbeau G, Boisse MF, Bartolomeo P, Dalla BG, Traykov L, Bachoud-Levi AC (2006) Cognitive impairment related to apathy in early Huntington's disease. Dement Geriatr Cogn Disord 21:316-321
15. Baumann CR, Hersberger M, Bassetti CL (2006) Hypocretin-1 (orexin A) levels are normal in Huntington's disease. J Neurol 253:1232-1233
16. Bayram-Weston Z, Jones L, Dunnett SB, Brooks SP (2012a) Light and electron microscopic characterization of the evolution of cellular pathology in HdhQ92 Huntington's disease knock-in mice. Brain Res Bull 88:171-181
17. Bayram-Weston Z, Jones L, Dunnett SB, Brooks SP (2012b) Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1 Huntington's disease transgenic mice. Brain Res Bull 88:104-112
18. Bayram-Weston Z, Jones L, Dunnett SB, Brooks SP (2012c) Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington's disease transgenic mice. Brain Res Bull 88:137-147
19. Bayram-Weston Z, Torres EM, Jones L, Dunnett SB, Brooks SP (2012d) Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse. Brain Res Bull 88:189-198
20. Benn CL, Landles C, Li H, Strand AD, Woodman B, Sathasivam K, Li SH, Ghazi-Noori S, Hockly E, Faruque SM, Cha JH, Sharpe PT, Olson JM, Li XJ, Bates GP (2005) Contribution of nuclear and extranuclear PolyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet 14:3065-3078
21. Bird ED, Spokes EG, Iversen LL (1980) Dopamine and noradrenaline in post-mortem brain in Huntington's disease and schizophrenic illness. Acta Psychiatr Scand Suppl 280:63-73
22. Bissonette GB, Martins GJ, Franz TM, Harper ES, Schoenbaum G, Powell EM (2008) Double dissociation of the effects of medial and orbital prefrontal cortical lesions on attentional and affective shifts in mice. J Neurosci 28:11124-11130
23. Bjorklund A, Dunnett SB (2007) Dopamine neuron systems in the brain: an update. Trends Neurosci 30:194-202
24. Bjorkqvist M, Fex M, Renstrom E, Wierup N, Petersen A, Gil J, Bacos K, Popovic N, Li JY, Sundler F, Brundin P, Mulder H (2005) The R6/2 transgenic mouse model of Huntington's disease develops diabetes due to deficient beta-cell mass and exocytosis. Hum Mol Genet 14:565-574
25. Braak H, Braak E (1992a) Allocortical involvement in Huntington's disease. Neuropathol Appl Neurobiol 18:539-547
26. Braak H, Braak E (1992b) The human entorhinal cortex: normal morphology and lamina-specific pathology in various diseases. Neurosci Res 15:6-31
27. Brooks S, Higgs G, Janghra N, Jones L, Dunnett SB (2012a) Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6 J) Huntington's disease transgenic mice. Brain Res Bull 88:113-120
28. Brooks S, Higgs G, Jones L, Dunnett SB (2012b) Longitudinal analysis of the behavioural phenotype in Hdh(CAG)150 Huntington's disease knock-in mice. Brain Res Bull 88:182-188
29. Brooks S, Higgs G, Jones L, Dunnett SB (2012c) Longitudinal analysis of the behavioural phenotype in HdhQ92 Huntington's disease knock-in mice. Brain Res Bull 88:148-155
30. Brooks SP, Betteridge H, Trueman RC, Jones L, Dunnett SB (2006) Selective extra-dimensional set shifting deficit in a knock-in mouse model of Huntington's disease. Brain Res Bull 69:452-457
31. Brooks SP, Janghra N, Higgs GV, Bayram-Weston Z, Heuer A, Jones L, Dunnett SB (2012d) Selective cognitive impairment in the YAC128 Huntington's disease mouse. Brain Res Bull 88:121-129
32. Brooks SP, Janghra N, Workman VL, Bayram-Weston Z, Jones L, Dunnett SB (2012e) Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6 J) Huntington's disease transgenic mice. Brain Res Bull 88:94-103
33. Brooks SP, Jones L, Dunnett SB (2012f) Longitudinal analyses of operant performance on the serial implicit learning task (SILT) in the YAC128 Huntington's disease mouse line. Brain Res Bull 88:130-136
34. Bussey TJ, Muir JL, Everitt BJ, Robbins TW (1997) Triple dissociation of anterior cingulate, posterior cingulate, and medial frontal cortices on visual discrimination tasks using a touchscreen testing procedure for the rat. Behav Neurosci 111:920-936
35. Calabresi P, Centonze D, Gubellini P, Bernardi G (1999) Activation of M1-like muscarinic receptors is required for the induction of corticostriatal LTP. Neuropharmacology 38:323-326
36. Calabresi P, Maj R, Pisani A, Mercuri NB, Bernardi G (1992a) Long-term synaptic depression in the striatum: physiological and pharmacological characterization. J Neurosci 12:4224-4233
37. Calabresi P, Pisani A, Mercuri NB, Bernardi G (1992b) Long-term potentiation in the striatum is unmasked by removing the voltage-dependent magnesium block of NMDA receptor channels. Eur J Neurosci 4:929-935
38. Callahan JW, Abercrombie ED (2011) In vivo Dopamine Efflux is decreased in striatum of both fragment (R6/2) and full-length (YAC128) transgenic mouse models of Huntington's disease. Front Syst Neurosci 5:61
39. Canals JM, Pineda JR, Torres-Peraza JF, Bosch M, Martin-Ibanez R, Munoz MT, Mengod G, Ernfors P, Alberch J (2004) Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease. J Neurosci 24:7727-7739
40. Carter RJ, Lione LA, Humby T, Mangiarini L, Mahal A, Bates GP, Dunnett SB, Morton AJ (1999) Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 19:3248-3257
41. Cepeda C, Ariano MA, Calvert CR, Flores-Hernandez J, Chandler SH, Leavitt BR, Hayden MR, Levine MS (2001) NMDA receptor function in mouse models of Huntington disease. J Neurosci Res 66:525-539
42. Chase EA, Tait DS, Brown VJ (2012) Lesions of the orbital prefrontal cortex impair the formation of attentional set in rats. Eur J Neurosci 36:2368-2375
43. Cheng Y, Peng Q, Hou Z, Aggarwal M, Zhang J, Mori S, Ross CA, Duan W (2011) Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington's disease mouse model. Neuroimage 56:1027-1034
44. Chiodi V, Uchigashima M, Beggiato S, Ferrante A, Armida M, Martire A, Potenza RL, Ferraro L, Tanganelli S, Watanabe M, Domenici MR, Popoli P (2012) Unbalance of CB1 receptors expressed in GABAergic and glutamatergic neurons in a transgenic mouse model of Huntington's disease. Neurobiol Dis 45:983-991
45. Chiu CT, Liu G, Leeds P, Chuang DM (2011) Combined treatment with the mood stabilizers lithium and valproate produces multiple beneficial effects in transgenic mouse models of Huntington's disease. Neuropsychopharmacology 36:2406-2421
46. Chudasama Y, Robbins TW (2006) Functions of frontostriatal systems in cognition: comparative neuropsychopharmacological studies in rats, monkeys and humans. Biol Psychol 73(1):19-38
47. 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
48. Clarke HF, Robbins TW, Roberts AC (2008) Lesions of the medial striatum in monkeys produce perseverative impairments during reversal learning similar to those produced by lesions of the orbitofrontal cortex. J Neurosci 28:10972-10982
49. Crook ZR, Housman DE (2012) Dysregulation of dopamine receptor D2 as a sensitive measure for Huntington disease pathology in model mice. Proc Natl Acad Sci U S A 109:7487-7492
50. Cross A, Rossor M (1983) Dopamine D-1 and D-2 receptors in Huntington's disease. Eur J Pharmacol 88:223-229
51. Cummings DM, Cepeda C, Levine MS (2010) Alterations in striatal synaptic transmission are consistent across genetic mouse models of Huntington's disease. ASN Neuro 2:e00036
52. Dalley JW, Mar AC, Economidou D, Robbins TW (2008) Neurobehavioral mechanisms of impulsivity: fronto-striatal systems and functional neurochemistry. Pharmacol Biochem Behav 90:250-260
53. Daoudal G, Debanne D (2003) Long-term plasticity of intrinsic excitability: learning rules and mechanisms. Learn Mem 10:456-465
54. Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, Bates GP (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90:537-548
55. de la Monte SM, Vonsattel JP, Richardson EP Jr (1988) Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease. J Neuropathol Exp Neurol 47:516-525
56. de Tommaso M, Serpino C, Sciruicchio V (2011) Management of Huntington's disease: role of tetrabenazine. Ther Clin Risk Manag 7:123-129
57. Deckel AW, Tang V, Nuttal D, Gary K, Elder R (2002) Altered neuronal nitric oxide synthase expression contributes to disease progression in Huntington's disease transgenic mice. Brain Res 939:76-86
58. Dey ND, Bombard MC, Roland BP, Davidson S, Lu M, Rossignol J, Sandstrom MI, Skeel RL, Lescaudron L, Dunbar GL (2010) Genetically engineered mesenchymal stem cells reduce behavioral deficits in the YAC 128 mouse model of Huntington's disease. Behav Brain Res 214:193-200
59. DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277:1990-1993
60. Divac I, Markowitsch HJ, Pritzel M (1978) Behavioral and anatomical consequences of small intrastriatal injections of kainic acid in the rat. Brain Res 151:523-532
61. Divac I, Rosvold HE, Szwarcbart MK (1967) Behavioral effects of selective ablation of the caudate nucleus. J Comp Physiol Psychol 63:184-190
62. Doria J, Silva F, de Souza J, Vieira L, Carvalho T, Reis H, Pereira G, Dobransky T, Ribeiro F (2013) Metabotropic glutamate receptor 5 positive allosteric modulators are neuroprotective in a mouse model of Huntington's disease. Br J Pharmacol 169:909-921
63. Dowie MJ, Bradshaw HB, Howard ML, Nicholson LF, Faull RL, Hannan AJ, Glass M (2009) Altered CB1 receptor and endocannabinoid levels precede motor symptom onset in a transgenic mouse model of Huntington's disease. Neuroscience 163:456-465
64. Dowie MJ, Howard ML, Nicholson LF, Faull RL, Hannan AJ, Glass M (2010) Behavioural and molecular consequences of chronic cannabinoid treatment in Huntington's disease transgenic mice. Neuroscience 170:324-336
65. Draganski B, Kherif F, Kloppel S, Cook PA, Alexander DC, Parker GJ, Deichmann R, Ashburner J, Frackowiak RS (2008) Evidence for segregated and integrative connectivity patterns in the human Basal Ganglia. J Neurosci 28:7143-7152
66. Duan W, Peng Q, Masuda N, Ford E, Tryggestad E, Ladenheim B, Zhao M, Cadet JL, Wong J, Ross CA (2008) Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease. Neurobiol Dis 30:312-322
67. Duff K, Paulsen JS, Beglinger LJ, Langbehn DR, Stout JC (2007) Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study. Biol Psychiatry 62:1341-1346
68. Dvorzhak A, Semtner M, Faber DS, Grantyn R (2013) Tonic mGluR5/CB1-dependent suppression of inhibition as a pathophysiological hallmark in the striatum of mice carrying a mutant form of huntingtin. J Physiol 591:1145-1166
69. Farrar AM, Callahan JW, Abercrombie ED (2011) Reduced striatal acetylcholine efflux in the R6/ 2 mouse model of Huntington's disease: an examination of the role of altered inhibitory and excitatory mechanisms. Exp Neurol 232:119-125
70. Filloux F, Wagster MV, Folstein S, Price DL, Hedreen JC, Dawson TM, Wamsley JK (1990) Nigral dopamine type-1 receptors are reduced in Huntington's disease: a postmortem autoradiographic study using [3H]SCH 23390 and correlation with [3H]forskolin binding. Exp Neurol 110:219-227
71. Floresco SB, Block AE, Tse MT (2008) Inactivation of the medial prefrontal cortex of the rat impairs strategy set-shifting, but not reversal learning, using a novel, automated procedure. Behav Brain Res 190:85-96
72. Furr A, Lapiz-Bluhm MD, Morilak DA (2012) 5-HT2A receptors in the orbitofrontal cortex facilitate reversal learning and contribute to the beneficial cognitive effects of chronic citalopram treatment in rats. Int J Neuropsychopharmacol 15:1295-1305
73. Furuyashiki T, Deguchi Y (2012) Roles of altered striatal function in major depression. Brain Nerve 64:919-926
74. Gaus SE, Lin L, Mignot E (2005) CSF hypocretin levels are normal in Huntington's disease patients. Sleep 28:1607-1608
75. Gerdeman GL, Ronesi J, Lovinger DM (2002) Postsynaptic endocannabinoid release is critical to long-term depression in the striatum. Nat Neurosci 5:446-451
76. Gibson HE, Reim K, Brose N, Morton AJ, Jones S (2005) A similar impairment in CA3 mossy fibre LTP in the R6/2 mouse model of Huntington's disease and in the complexin II knockout mouse. Eur J Neurosci 22:1701-1712
77. Giralt A, Carreton O, Lao-Peregrin C, Martin ED, Alberch J (2011a) Conditional BDNF release under pathological conditions improves Huntington's disease pathology by delaying neuronal dysfunction. Mol Neurodegener 6:71
78. Giralt A, Puigdellivol M, Carreton O, Paoletti P, Valero J, Parra-Damas A, Saura CA, Alberch J, Gines S (2012) Long-term memory deficits in Huntington's disease are associated with reduced CBP histone acetylase activity. Hum Mol Genet 21:1203-1216
79. Giralt A, Rodrigo T, Martin ED, Gonzalez JR, Mila M, Cena V, Dierssen M, Canals JM, Alberch J (2009) Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: involvement of phospholipaseC gamma activity and glutamate receptor expression. Neuroscience 158:1234-1250
80. Giralt A, Saavedra A, Carreton O, Xifro X, Alberch J, Perez-Navarro E (2011b) Increased PKA signaling disrupts recognition memory and spatial memory: role in Huntington's disease. Hum Mol Genet 20:4232-4247
81. Glass M, Dragunow M, Faull RL (2000) The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease. Neuroscience 97:505-519
82. Glass M, Faull RL, Dragunow M (1993) Loss of cannabinoid receptors in the substantia nigra in Huntington's disease. Neuroscience 56:523-527
83. Glass M, Van Dellen A, Blakemore C, Hannan AJ, Faull RL (2004) Delayed onset of Huntington's disease in mice in an enriched environment correlates with delayed loss of cannabinoid CB1 receptors. Neuroscience 123:207-212
84. Gomez-Tortosa E, MacDonald ME, Friend JC, Taylor SA, Weiler LJ, Cupples LA, Srinidhi J, Gusella JF, Bird ED, Vonsattel JP, Myers RH (2001) Quantitative neuropathological changes in presymptomatic Huntington's disease. Ann Neurol 49:29-34
85. Goodman AO, Barker RA (2010) How vital is sleep in Huntington's disease? J Neurol 257:882-897
86. Goodman AO, Rogers L, Pilsworth S, McAllister CJ, Shneerson JM, Morton AJ, Barker RA (2011) Asymptomatic sleep abnormalities are a common early feature in patients with Huntington's disease. Curr Neurol Neurosci Rep 11:211-217
87. Gorwood P (2008) Neurobiological mechanisms of anhedonia. Dialogues Clin Neurosci 10:291-299
88. Graveland GA, Williams RS, DiFiglia M (1985) Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science 227:770-773
89. Gray M, Shirasaki DI, Cepeda C, Andre VM, Wilburn B, Lu XH, Tao J, Yamazaki I, Li SH, Sun YE, Li XJ, Levine MS, Yang XW (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
90. Griffioen KJ, Wan R, Brown TR, Okun E, Camandola S, Mughal MR, Phillips TM, Mattson MP (2012) Aberrant heart rate and brainstem brain-derived neurotrophic factor (BDNF) signaling in a mouse model of Huntington's disease. Neurobiol Aging 33:1481-1485
91. Groenewegen HJ, Trimble M (2007) The ventral striatum as an interface between the limbic and motor systems. CNS Spectr 12:887-892
92. Gubellini P, Pisani A, Centonze D, Bernardi G, Calabresi P (2004) Metabotropic glutamate receptors and striatal synaptic plasticity: implications for neurological diseases. Prog Neurobiol 74:271-300
93. Gubellini P, Saulle E, Centonze D, Bonsi P, Pisani A, Bernardi G, Conquet F, Calabresi P (2001) Selective involvement of mGlu1 receptors in corticostriatal LTD. Neuropharmacology 40:839-846
94. Hampshire A, Chaudhry AM, Owen AM, Roberts AC (2012) Dissociable roles for lateral orbitofrontal cortex and lateral prefrontal cortex during preference driven reversal learning. Neuroimage 59:4102-4112
95. Hansson O, Guatteo E, Mercuri NB, Bernardi G, Li XJ, Castilho RF, Brundin P (2001) Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene. Eur J Neurosci 14:1492-1504
96. Heikkinen T, Lehtimaki K, Vartiainen N, Puolivali J, Hendricks SJ, Glaser JR, Bradaia A, Wadel K, Touller C, Kontkanen O, Yrjanheikki JM, Buisson B, Howland D, Beaumont V, Munoz-Sanjuan I, Park LC (2012) Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease. PLoS ONE 7:e50717
97. Heinsen H, Rub U, Bauer M, Ulmar G, Bethke B, Schuler M, Bocker F, Eisenmenger W, Gotz M, Korr H, Schmitz C (1999) Nerve cell loss in the thalamic mediodorsal nucleus in Huntington's disease. Acta Neuropathol 97:613-622
98. Heinsen H, Strik M, Bauer M, Luther K, Ulmar G, Gangnus D, Jungkunz G, Eisenmenger W, Gotz M (1994) Cortical and striatal neurone number in Huntington's disease. Acta Neuropathol 88:320-333
99. Heng MY, Tallaksen-Greene SJ, Detloff PJ, Albin RL (2007) Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease. J Neurosci 27:8989-8998
100. Hickey MA, Kosmalska A, Enayati J, Cohen R, Zeitlin S, Levine MS, Chesselet MF (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
101. Hickey MA, Reynolds GP, Morton AJ (2002) The role of dopamine in motor symptoms in the R6/2 transgenic mouse model of Huntington's disease. J Neurochem 81:46-59
102. Hickey MA, Zhu C, Medvedeva V, Lerner RP, Patassini S, Franich NR, Maiti P, Frautschy SA, Zeitlin S, Levine MS, Chesselet MF (2012) Improvement of neuropathology and transcriptional deficits in CAG 140 knock-in mice supports a beneficial effect of dietary curcumin in Huntington's disease. Mol Neurodegener 7:12
103. Hult LS, Nilsson N, Soylu R, Kirik D, Petersen A (2013) Hypothalamic expression of mutant huntingtin contributes to the development of depressive-like behavior in the BAC transgenic mouse model of Huntington's disease. Hum Mol Genet 22(17):3485-3497
104. Hurlbert MS, Zhou W, Wasmeier C, Kaddis FG, Hutton JC, Freed CR (1999) Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes. Diabetes 48:649-651
105. Jiang M, Peng Q, Liu X, Jin J, Hou Z, Zhang J, Mori S, Ross CA, Ye K, Duan W (2013) Smallmolecule TrkB receptor agonists improve motor function and extend survival in a mouse model of Huntington's disease. Hum Mol Genet 22(12):2462-2470
106. Josefsen K, Nielsen MD, Jorgensen KH, Bock T, Norremolle A, Sorensen SA, Naver B, Hasholt L (2008) Impaired glucose tolerance in the R6/1 transgenic mouse model of Huntington's disease. J Neuroendocrinol 20:165-172
107. Joshi PR, Wu NP, Andre VM, Cummings DM, Cepeda C, Joyce JA, Carroll JB, Leavitt BR, Hayden MR, Levine MS, Bamford NS (2009) Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J Neurosci 29:2414-2427
108. Josiassen RC, Curry LM, Mancall EL (1983) Development of neuropsychological deficits in Huntington's disease. Arch Neurol 40:791-796
109. Kerr JN, Wickens JR (2001) Dopamine D-1/D-5 receptor activation is required for long-term potentiation in the rat neostriatum in vitro. J Neurophysiol 85:117-124
110. Kim J, Ragozzino ME (2005) The involvement of the orbitofrontal cortex in learning under changing task contingencies. Neurobiol Learn Mem 83:125-133
111. Kingma EM, Van Duijn E, Timman R, Van der Mast RC, Roos RA (2008) Behavioural problems in Huntington's disease using the problem behaviours assessment. Gen Hosp Psychiatry 30:155-161
112. Kiriazis H, Jennings NL, Davern P, Lambert G, Su Y, Pang T, Du X, La Greca L, Head GA, Hannan AJ, Du XJ (2012) Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease. J Physiol 590:5845-5860
113. Klanker M, Post G, Joosten R, Feenstra M, Denys D (2013) Deep brain stimulation in the lateral orbitofrontal cortex impairs spatial reversal learning. Behav Brain Res 245:7-12
114. Klapstein GJ, Fisher RS, Zanjani H, Cepeda C, Jokel ES, Chesselet MF, Levine MS (2001) Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice. J Neurophysiol 86:2667-2677
115. Kloppel S, Stonnington CM, Petrovic P, Mobbs D, Tuscher O, Craufurd D, Tabrizi SJ, Frackowiak RS (2010) Irritability in pre-clinical Huntington's disease. Neuropsychologia 48:549-557
116. Kudo T, Schroeder A, Loh DH, Kuljis D, Jordan MC, Roos KP, Colwell CS (2011) Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease. Exp Neurol 228:80-90
117. Kung VW, Hassam R, Morton AJ, Jones S (2007) Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease. Neuroscience 146:1571-1580
118. Kurlan R, Goldblatt D, Zaczek R, Jeffries K, Irvine C, Coyle J, Shoulson I (1988) Cerebrospinal fluid homovanillic acid and parkinsonism in Huntington's disease. Ann Neurol 24:282-284
119. Labuschagne I, Jones R, Callaghan J, Whitehead D, Dumas EM, Say MJ, Hart EP, Justo D, Coleman A, Dar Santos RC, Frost C, Craufurd D, Tabrizi SJ, Stout JC (2013) Emotional face recognition deficits and medication effects in pre-manifest through stage-II Huntington's disease. Psychiatry Res 207:118-126
120. Lawrence AD, Hodges JR, Rosser AE, Kershaw A, Ffrench-Constant C, Rubinsztein DC, Robbins TW, Sahakian BJ (1998) Evidence for specific cognitive deficits in preclinical Huntington's disease. Brain 121(Pt 7):1329-1341
121. Lawrence AD, Sahakian BJ, Hodges JR, Rosser AE, Lange KW, Robbins TW (1996) Executive and mnemonic functions in early Huntington's disease. Brain 119(Pt 5):1633-1645
122. Lawrence AD, Sahakian BJ, Rogers RD, Hodge JR, Robbins TW (1999) Discrimination, reversal, and shift learning in Huntington's disease: mechanisms of impaired response selection. Neuropsychologia 37:1359-1374
123. Lawrence AD, Watkins LH, Sahakian BJ, Hodges JR, Robbins TW (2000) Visual object and visuospatial cognition in Huntington's disease: implications for information processing in corticostriatal circuits. Brain 123(Pt 7):1349-1364
124. Le Merrer J, Rezai X, Scherrer G, Becker JA, Kieffer BL (2013) Impaired hippocampusdependent and facilitated striatum-dependent behaviors in mice lacking the delta opioid receptor. Neuropsychopharmacology 38:1050-1059
125. Lehericy S, Ducros M, Van de Moortele PF, Francois C, Thivard L, Poupon C, Swindale N, Ugurbil K, Kim DS (2004) Diffusion tensor fiber tracking shows distinct corticostriatal circuits in humans. Ann Neurol 55:522-529
126. Lerner RP, Trejo Martinez LC, Zhu C, Chesselet MF, Hickey MA (2012) Striatal atrophy and dendritic alterations in a knock-in mouse model of Huntington's disease. Brain Res Bull 87:571-578
127. Levine MS, Klapstein GJ, Koppel A, Gruen E, Cepeda C, Vargas ME, Jokel ES, Carpenter EM, Zanjani H, Hurst RS, Efstratiadis A, Zeitlin S, Chesselet MF (1999) Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res 58:515-532
128. Lin CH, Tallaksen-Greene S, Chien WM, Cearley JA, Jackson WS, Crouse AB, Ren S, Li XJ, Albin RL, Detloff PJ (2001) Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 10:137-144
129. Lindgren HS, Wickens R, Tait DS, Brown VJ, Dunnett SB (2013) Lesions of the dorsomedial striatum impair formation of attentional set in rats. Neuropharmacology 71:148-153
130. Lione LA, Carter RJ, Hunt MJ, Bates GP, Morton AJ, Dunnett SB (1999) Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation. J Neurosci 19:10428-10437
131. Liot G, Zala D, Pla P, Mottet G, Piel M, Saudou F (2013) Mutant Huntingtin alters retrograde transport of TrkB receptors in striatal dendrites. J Neurosci 33:6298-6309
132. Mandal M, Wei J, Zhong P, Cheng J, Duffney LJ, Liu W, Yuen EY, Twelvetrees AE, Li S, Li XJ, Kittler JT, Yan Z (2011) Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin. J Biol Chem 286:33719-33728
133. Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C, Lawton M, Trottier Y, Lehrach H, Davies SW, Bates GP (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
134. Mann DM (1989) Subcortical afferent projection systems in Huntington's chorea. Acta Neuropathol 78:551-554
135. Marshall J, White K, Weaver M, Flury WL, Hui S, Stout JC, Johnson SA, Beristain X, Gray J, Wojcieszek J, Foroud T (2007) Specific psychiatric manifestations among preclinical Huntington disease mutation carriers. Arch Neurol 64:116-121
136. Martin B, Golden E, Carlson OD, Pistell P, Zhou J, Kim W, Frank BP, Thomas S, Chadwick WA, Greig NH, Bates GP, Sathasivam K, Bernier M, Maudsley S, Mattson MP, Egan JM (2009) Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's disease. Diabetes 58:318-328
137. Maywood ES, Fraenkel E, McAllister CJ, Wood N, Reddy AB, Hastings MH, Morton AJ (2010) Disruption of peripheral circadian timekeeping in a mouse model of Huntington's disease and its restoration by temporally scheduled feeding. J Neurosci 30:10199-10204
138. McCool MF, Patel S, Talati R, Ragozzino ME (2008) Differential involvement of M1-type and M4-type muscarinic cholinergic receptors in the dorsomedial striatum in task switching. Neurobiol Learn Mem 89:114-124
139. Meier A, Mollenhauer B, Cohrs S, Rodenbeck A, Jordan W, Meller J, Otto M (2005) Normal hypocretin-1 (orexin-A) levels in the cerebrospinal fluid of patients with Huntington's disease. Brain Res 1063:201-203
140. Menalled L, El Khodor BF, Patry M, Suarez-Farinas M, Orenstein SJ, Zahasky B, Leahy C, Wheeler V, Yang XW, MacDonald M, Morton AJ, Bates G, Leeds J, Park L, Howland D, Signer E, Tobin A, Brunner D (2009) Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models. Neurobiol Dis 35:319-336
141. Menalled LB, Kudwa AE, Miller S, Fitzpatrick J, Watson-Johnson J, Keating N, Ruiz M, Mushlin R, Alosio W, McConnell K, Connor D, Murphy C, Oakeshott S, Kwan M, Beltran J, Ghavami A, Brunner D, Park LC, Ramboz S, Howland D (2012) Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175. PLoS ONE 7:e49838
142. Menalled LB, Sison JD, Dragatsis I, Zeitlin S, Chesselet MF (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
143. Mievis S, Blum D, Ledent C (2011) Worsening of Huntington disease phenotype in CB1 receptor knockout mice. Neurobiol Dis 42:524-529
144. Miller J, Arrasate M, Shaby BA, Mitra S, Masliah E, Finkbeiner S (2010) Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis. J Neurosci 30:10541-10550
145. Milnerwood AJ, Raymond LA (2007) Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load. J Physiol 585:817-831
146. Mochel F, Durant B, Durr A, Schiffmann R (2011) Altered dopamine and serotonin metabolism in motorically asymptomatic R6/2 mice. PLoS ONE 6:e18336
147. Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP (2009) Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS ONE 4:e8025
148. Morton AJ, Lagan MA, Skepper JN, Dunnett SB (2000) Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation. J Neurocytol 29:679-702
149. Morton AJ, Wood NI, Hastings MH, Hurelbrink C, Barker RA, Maywood ES (2005) Disintegration of the sleep-wake cycle and circadian timing in Huntington's disease. J Neurosci 25:157-163
150. Muir JL, Page KJ, Sirinathsinghji DJ, Robbins TW, Everitt BJ (1993) Excitotoxic lesions of basal forebrain cholinergic neurons: effects on learning, memory and attention. Behav Brain Res 57:123-131
151. Murphy KP, Carter RJ, Lione LA, Mangiarini L, Mahal A, Bates GP, Dunnett SB, Morton AJ (2000) Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J Neurosci 20:5115-5123
152. Myers RH, Vonsattel JP, Paskevich PA, Kiely DK, Stevens TJ, Cupples LA, Richardson EP Jr, Bird ED (1991) Decreased neuronal and increased oligodendroglial densities in Huntington's disease caudate nucleus. J Neuropathol Exp Neurol 50:729-742
153. Nestler EJ, Carlezon WA Jr (2006) The mesolimbic dopamine reward circuit in depression. Biol Psychiatry 59:1151-1159
154. Nithianantharajah J, Barkus C, Murphy M, Hannan AJ (2008) Gene-environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington's disease transgenic mice. Neurobiol Dis 29:490-504
155. Norflus F, Nanje A, Gutekunst CA, Shi G, Cohen J, Bejarano M, Fox J, Ferrante RJ, Hersch SM (2004) Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic mice. Neurobiol Dis 17:319-325
156. Novak MJ, Warren JD, Henley SM, Draganski B, Frackowiak RS, Tabrizi SJ (2012) Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease. Brain 135:1165-1179
157. Ortiz AN, Kurth BJ, Osterhaus GL, Johnson MA (2010) Dysregulation of intracellular dopamine stores revealed in the R6/2 mouse striatum. J Neurochem 112:755-761
158. Orvoen S, Pla P, Gardier AM, Saudou F, David DJ (2012) Huntington's disease knock-in male mice show specific anxiety-like behaviour and altered neuronal maturation. Neurosci Lett 507:127-132
159. Pallier PN, Drew CJ, Morton AJ (2009) The detection and measurement of locomotor deficits in a transgenic mouse model of Huntington's disease are task-and protocol-dependent: influence of non-motor factors on locomotor function. Brain Res Bull 78:347-355
160. Pang TY, Stam NC, Nithianantharajah J, Howard ML, Hannan AJ (2006) Differential effects of voluntary physical exercise on behavioral and brain-derived neurotrophic factor expression deficits in Huntington's disease transgenic mice. Neuroscience 141:569-584
161. Paulsen JS, Magnotta VA, Mikos AE, Paulson HL, Penziner E, Andreasen NC, Nopoulos PC (2006) Brain structure in preclinical Huntington's disease. Biol Psychiatry 59:57-63
162. Paulsen JS, Nopoulos PC, Aylward E, Ross CA, Johnson H, Magnotta VA, Juhl A, Pierson RK, Mills J, Langbehn D, Nance M (2010) Striatal and white matter predictors of estimated diagnosis for Huntington disease. Brain Res Bull 82:201-207
163. Paulsen JS, Ready RE, Hamilton JM, Mega MS, Cummings JL (2001) Neuropsychiatric aspects of Huntington's disease. J Neurol Neurosurg Psychiatry 71:310-314
164. Peng Q, Masuda N, Jiang M, Li Q, Zhao M, Ross CA, Duan W (2008) The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model. Exp Neurol 210:154-163
165. Petersen A, Gil J, Maat-Schieman ML, Bjorkqvist M, Tanila H, Araujo IM, Smith R, Popovic N, Wierup N, Norlen P, Li JY, Roos RA, Sundler F, Mulder H, Brundin P (2005) Orexin loss in Huntington's disease. Hum Mol Genet 14:39-47
166. Pidgeon C, Rickards H (2013) The pathophysiology and pharmacological treatment of Huntington disease. Behav Neurol 26:245-253
167. Pignatelli M, Lebreton F, Cho YH, Leinekugel X (2012) "Ectopic'' theta oscillations and interictal activity during slow-wave state in the R6/1 mouse model of Huntington's disease. Neurobiol Dis 48:409-417
168. Pisa M, Cyr J (1990) Regionally selective roles of the rat's striatum in modality-specific discrimination learning and forelimb reaching. Behav Brain Res 37:281-292
169. Pouladi MA, Stanek LM, Xie Y, Franciosi S, Southwell AL, Deng Y, Butland S, Zhang W, Cheng SH, Shihabuddin LS, Hayden MR (2012) Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice. Hum Mol Genet 21:2219-2232
170. Ragozzino ME, Ragozzino KE, Mizumori SJ, Kesner RP (2002) Role of the dorsomedial striatum in behavioral flexibility for response and visual cue discrimination learning. Behav Neurosci 116:105-115
171. Ransome MI, Hannan AJ (2013) Impaired basal and running-induced hippocampal neurogenesis coincides with reduced Akt signaling in adult R6/1 HD mice. Mol Cell Neurosci 54:93-107
172. Rattray I, Smith E, Gale R, Matsumoto K, Bates GP, Modo M (2013) Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/ 2 mouse model of HD. PLoS ONE 8:e60012
173. Reddy PH, Charles V, Williams M, Miller G, Whetsell WO Jr, Tagle DA (1999) Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease. Philos Trans R Soc Lond B Biol Sci 354:1035-1045
174. Reedeker N, Bouwens JA, Giltay EJ, Le Mair SE, Roos RA, Van der Mast RC, Van Duijn E (2012) Irritability in Huntington's disease. Psychiatry Res 200:813-818
175. Reedeker N, Bouwens JA, Van Duijn E, Giltay EJ, Roos RA, Van der Mast RC (2011) Incidence, course, and predictors of apathy in Huntington's disease: a two-year prospective study. J Neuropsychiatry Clin Neurosci 23:434-441
176. Reiner A, Lafferty DC, Wang HB, Del Mar N, Deng YP (2012a) The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice. Neurobiol Dis 47:75-91
177. Reiner A, Wang HB, Del Mar N, Sakata K, Yoo W, Deng YP (2012b) BDNF may play a differential role in the protective effect of the mGluR2/3 agonist LY379268 on striatal projection neurons in R6/2 Huntington's disease mice. Brain Res 1473:161-172
178. Renoir T, Pang TY, Mo C, Chan G, Chevarin C, Lanfumey L, Hannan AJ (2013) Differential effects of early environmental enrichment on emotionality related behaviours in Huntington's disease transgenic mice. J Physiol 591:41-55
179. Renoir T, Pang TY, Zajac MS, Chan G, Du X, Leang L, Chevarin C, Lanfumey L, Hannan AJ (2012) Treatment of depressive-like behaviour in Huntington's disease mice by chronic sertraline and exercise. Br J Pharmacol 165:1375-1389
180. Renoir T, Zajac MS, Du X, Pang TY, Leang L, Chevarin C, Lanfumey L, Hannan AJ (2011) Sexually dimorphic serotonergic dysfunction in a mouse model of Huntington's disease and depression. PLoS ONE 6:e22133
181. Ribchester RR, Thomson D, Wood NI, Hinks T, Gillingwater TH, Wishart TM, Court FA, Morton AJ (2004) Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation. Eur J Neurosci 20:3092-3114
182. Rising AC, Xu J, Carlson A, Napoli VV, Denovan-Wright EM, Mandel RJ (2011) Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats. Exp Neurol 228:173-182
183. Robbins TW, Gillan CM, Smith DG, de Wit S, Ersche KD (2012) Neurocognitive endophenotypes of impulsivity and compulsivity: towards dimensional psychiatry. Trends Cogn Sci 16:81-91
184. Roizin L, Stellar S, Willson N, Whittier J, Liu JC (1974) Electron microscope and enzyme studies in cerebral biopsies of Huntington's chorea. Trans Am Neurol Assoc 99:240-243
185. Roman MJ, Delis DC, Filoteo JV, Demadura TL, Paulsen J, Swerdlow NR, Swenson MR, Salmon D, Butters N, Shults C (1998) Is there a "subcortical'' profile of attentional dysfunction? A comparison of patients with Huntington's and Parkinson's diseases on a global-local focused attention task. J Clin Exp Neuropsychol 20:873-884
186. Roos RA, Aziz NA (2007) Hypocretin-1 and secondary signs in Huntington's disease. Parkinsonism Relat Disord 13(Suppl 3):S387-S390
187. Rosas HD, Hevelone ND, Zaleta AK, Greve DN, Salat DH, Fischl B (2005) Regional cortical thinning in preclinical Huntington disease and its relationship to cognition. Neurology 65:745-747
188. Rosas HD, Liu AK, Hersch S, Glessner M, Ferrante RJ, Salat DH, Van der KA, Jenkins BG, Dale AM, Fischl B (2002) Regional and progressive thinning of the cortical ribbon in Huntington's disease. Neurology 58:695-701
189. Rosvold HE, Delgado JM (1956) The effect on delayed-alternation test performance of stimulating or destroying electrically structures within the frontal lobes of the monkey's brain. J Comp Physiol Psychol 49:365-372
190. Rosvold HE, Mishkin M, Szwarcbart MK (1958) Effects of subcortical lesions in monkeys on visual-discrimination and single-alternation performance. J Comp Physiol Psychol 51:437-444
191. Rub U, Hoche F, Brunt ER, Heinsen H, Seidel K, Del Turco D, Paulson HL, Bohl J, von Gall C, Vonsattel JP, Korf HW, den Dunnen WF (2013) Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. Brain Pathol 23:165-177
192. Ruskin DN, Ross JL, Kawamura M Jr, Ruiz TL, Geiger JD, Masino SA (2011) A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1 J mouse model of Huntington's disease. Physiol Behav 103:501-507
193. Samadi P, Boutet A, Rymar VV, Rawal K, Maheux J, Kvann JC, Tomaszewski M, Beaubien F, Cloutier JF, Levesque D, Sadikot AF (2013) Relationship between BDNF expression in major striatal afferents, striatum morphology and motor behavior in the R6/2 mouse model of Huntington's disease. Genes Brain Behav 12:108-124
194. Saudou F, Finkbeiner S, Devys D, Greenberg ME (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95:55-66
195. Schilling G, Klevytska A, Tebbenkamp AT, Juenemann K, Cooper J, Gonzales V, Slunt H, Poirer M, Ross CA, Borchelt DR (2007) Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models. J Neuropathol Exp Neurol 66:313-320
196. Schmitz C, Rub U, Korr H, Heinsen H (1999) Nerve cell loss in the thalamic mediodorsal nucleus in Huntington's disease. II. Optimization of a stereological estimation procedure. Acta Neuropathol 97:623-628
197. Schotanus SM, Chergui K (2008) Dopamine D1 receptors and group I metabotropic glutamate receptors contribute to the induction of long-term potentiation in the nucleus accumbens. Neuropharmacology 54:837-844
198. Slow EJ, Graham RK, Osmand AP, Devon RS, Lu G, Deng Y, Pearson J, Vaid K, Bissada N, Wetzel R, Leavitt BR, Hayden MR (2005) Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A 102:11402-11407
199. Slow EJ, Van Raamsdonk J, Rogers D, Coleman SH, Graham RK, Deng Y, Oh R, Bissada N, Hossain SM, Yang YZ, Li XJ, Simpson EM, Gutekunst CA, Leavitt BR, Hayden MR (2003) Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 12:1555-1567
200. Snowden J, Craufurd D, Griffiths H, Thompson J, Neary D (2001) Longitudinal evaluation of cognitive disorder in Huntington's disease. J Int Neuropsychol Soc 7:33-44
201. Snowden JS, Austin NA, Sembi S, Thompson JC, Craufurd D, Neary D (2008) Emotion recognition in Huntington's disease and frontotemporal dementia. Neuropsychologia 46:2638-2649
202. Snowden JS, Craufurd D, Griffiths HL, Neary D (1998) Awareness of involuntary movements in Huntington disease. Arch Neurol 55:801-805
203. Snowden JS, Craufurd D, Thompson J, Neary D (2002) Psychomotor, executive, and memory function in preclinical Huntington's disease. J Clin Exp Neuropsychol 24:133-145
204. Snowden JS, Gibbons ZC, Blackshaw A, Doubleday E, Thompson J, Craufurd D, Foster J, Happe F, Neary D (2003) Social cognition in frontotemporal dementia and Huntington's disease. Neuropsychologia 41:688-701
205. Solomon AC, Stout JC, Johnson SA, Langbehn DR, Aylward EH, Brandt J, Ross CA, Beglinger L, Hayden MR, Kieburtz K, Kayson E, Julian-Baros E, Duff K, Guttman M, Nance M, Oakes D, Shoulson I, Penziner E, Paulsen JS (2007) Verbal episodic memory declines prior to diagnosis in Huntington's disease. Neuropsychologia 45:1767-1776
206. Soontornniyomkij V, Risbrough VB, Young JW, Soontornniyomkij B, Jeste DV, Achim CL (2012) Increased hippocampal accumulation of autophagosomes predicts short-term recognition memory impairment in aged mice. Age (Dordr) 34:305-316
207. Soontornniyomkij V, Risbrough VB, Young JW, Wallace CK, Soontornniyomkij B, Jeste DV, Achim CL (2010) Short-term recognition memory impairment is associated with decreased expression of FK506 binding protein 51 in the aged mouse brain. Age (Dordr) 32:309-322
208. Spampanato J, Gu X, Yang XW, Mody I (2008) Progressive synaptic pathology of motor cortical neurons in a BAC transgenic mouse model of Huntington's disease. Neuroscience 157:606-620
209. Spires TL, Grote HE, Varshney NK, Cordery PM, Van Dellen A, Blakemore C, Hannan AJ (2004) Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism. J Neurosci 24:2270-2276
210. Spokes EG (1980) Neurochemical alterations in Huntington's chorea: a study of post-mortem brain tissue. Brain 103:179-210
211. Sprengelmeyer R, Lange H, Homberg V (1995) The pattern of attentional deficits in Huntington's disease. Brain 118(Pt 1):145-152
212. Starling AJ, Andre VM, Cepeda C, de Lima M, Chandler SH, Levine MS (2005) Alterations in N-methyl-D-aspartate receptor sensitivity and magnesium blockade occur early in development in the R6/2 mouse model of Huntington's disease. J Neurosci Res 82:377-386
213. Stout JC, Johnson SA (2005) Cognitive impairment and dementia in basal ganglia disorders. Curr Neurol Neurosci Rep 5:355-363
214. Stout JC, Jones R, Labuschagne I, O'Regan AM, Say MJ, Dumas EM, Queller S, Justo D, Santos RD, Coleman A, Hart EP, Durr A, Leavitt BR, Roos RA, Langbehn DR, Tabrizi SJ, Frost C (2012) Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease. J Neurol Neurosurg Psychiatry 83:687-694
215. Tabrizi SJ, Scahill RI, Durr A, Roos RA, Leavitt BR, Jones R, Landwehrmeyer GB, Fox NC, Johnson H, Hicks SL, Kennard C, Craufurd D, Frost C, Langbehn DR, Reilmann R, Stout JC (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
216. Tabrizi SJ, Scahill RI, Owen G, Durr A, Leavitt BR, Roos RA, Borowsky B, Landwehrmeyer B, Frost C, Johnson H, Craufurd D, Reilmann R, Stout JC, Langbehn DR (2013) Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data. Lancet Neurol 12(7):637-649
217. Tatro ET, Risbrough V, Soontornniyomkij B, Young J, Shumaker-Armstrong S, Jeste DV, Achim CL (2013) Short-term recognition memory correlates with regional CNS expression of microRNA-138 in mice. Am J Geriatr Psychiatry 21:461-473
218. Temel Y, Cao C, Vlamings R, Blokland A, Ozen H, Steinbusch HW, Michelsen KA, von Horsten S, Schmitz C, Visser-Vandewalle V (2006) Motor and cognitive improvement by deep brain stimulation in a transgenic rat model of Huntington's disease. Neurosci Lett 406:138-141
219. 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. The Huntington's Disease Collaborative Research Group. Cell 72:971-983
220. Thompson JC, Harris J, Sollom AC, Stopford CL, Howard E, Snowden JS, Craufurd D (2012) Longitudinal evaluation of neuropsychiatric symptoms in Huntington's disease. J Neuropsychiatry Clin Neurosci 24:53-60
221. Thompson JC, Poliakoff E, Sollom AC, Howard E, Craufurd D, Snowden JS (2010) Automaticity and attention in Huntington's disease: when two hands are not better than one. Neuropsychologia 48:171-178
222. Thompson JC, Snowden JS, Craufurd D, Neary D (2002) Behavior in Huntington's disease: dissociating cognition-based and mood-based changes. J Neuropsychiatry Clin Neurosci 14:37-43
223. Trueman RC, Brooks SP, Jones L, Dunnett SB (2007) The operant serial implicit learning task reveals early onset motor learning deficits in the Hdh knock-in mouse model of Huntington's disease. Eur J Neurosci 25:551-558
224. Trueman RC, Brooks SP, Jones L, Dunnett SB (2008) Time course of choice reaction time deficits in the Hdh(Q92) knock-in mouse model of Huntington's disease in the operant serial implicit learning task (SILT). Behav Brain Res 189:317-324
225. Trueman RC, Brooks SP, Jones L, Dunnett SB (2009) Rule learning, visuospatial function and motor performance in the Hdh(Q92) knock-in mouse model of Huntington's disease. Behav Brain Res 203:215-222
226. Trueman RC, Dunnett SB, Jones L, Brooks SP (2012a) Five choice serial reaction time performance in the HdhQ92 mouse model of Huntington's disease. Brain Res Bull 88:163-170
227. Trueman RC, Jones L, Dunnett SB, Brooks SP (2012b) Early onset deficits on the delayed alternation task in the Hdh(Q92) knock-in mouse model of Huntington's disease. Brain Res Bull 88:156-162
228. Twelvetrees AE, Yuen EY, Arancibia-Carcamo IL, MacAskill AF, Rostaing P, Lumb MJ, Humbert S, Triller A, Saudou F, Yan Z, Kittler JT (2010) Delivery of GABAARs to synapses is mediated by HAP1-KIF5 and disrupted by mutant huntingtin. Neuron 65:53-65
229. Vaccarino AL, Sills T, Anderson KE, Bachoud-Levi AC, Borowsky B, Craufurd D, Duff K, Giuliano J, Groves M, Guttman M, Kupchak P, Ho AK, Paulsen JS, Pedersen KF, Van Duijn E, Van Kammen DP, Evans K (2011) Assessment of depression, anxiety and apathy in prodromal and early Huntington disease. PLoS Curr 3: RRN1242
230. Van Dellen A, Cordery PM, Spires TL, Blakemore C, Hannan AJ (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
231. Van Duijn E, Kingma EM, Timman R, Zitman FG, Tibben A, Roos RA, Van der Mast RC (2008) Cross-sectional study on prevalences of psychiatric disorders in mutation carriers of Huntington's disease compared with mutation-negative first-degree relatives. J Clin Psychiatry 69:1804-1810
232. Van Duijn E, Kingma EM, Van der Mast RC (2007) Psychopathology in verified Huntington's disease gene carriers. J Neuropsychiatry Clin Neurosci 19:441-448
233. Van Duijn E, Reedeker N, Giltay EJ, Roos RA, Van der Mast RC (2010) Correlates of apathy in Huntington's disease. J Neuropsychiatry Clin Neurosci 22:287-294
234. Van Laere K, Casteels C, Dhollander I, Goffin K, Grachev I, Bormans G, Vandenberghe W (2010) Widespread decrease of type 1 cannabinoid receptor availability in Huntington disease in vivo. J Nucl Med 51:1413-1417
235. Van Raamsdonk JM, Murphy Z, Slow EJ, Leavitt BR, Hayden MR (2005a) Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum Mol Genet 14:3823-3835
236. Van Raamsdonk JM, Pearson J, Slow EJ, Hossain SM, Leavitt BR, Hayden MR (2005b) Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci 25:4169-4180
237. Van Wamelen DJ, Aziz NA, Anink JJ, Van Steenhoven R, Angeloni D, Fraschini F, Jockers R, Roos RA, Swaab DF (2013) Suprachiasmatic nucleus neuropeptide expression in patients with Huntington's Disease. Sleep 36:117-125
238. Verny C, Allain P, Prudean A, Malinge MC, Gohier B, Scherer C, Bonneau D, Dubas F, Le Gall D (2007) Cognitive changes in asymptomatic carriers of the Huntington disease mutation gene. Eur J Neurol 14:1344-1350
239. Vetter JM, Jehle T, Heinemeyer J, Franz P, Behrens PF, Jackisch R, Landwehrmeyer GB, Feuerstein TJ (2003) Mice transgenic for exon 1 of Huntington's disease: properties of cholinergic and dopaminergic pre-synaptic function in the striatum. J Neurochem 85:1054-1063
240. Voermans NC, Petersson KM, Daudey L, Weber B, Van Spaendonck KP, Kremer HP, Fernandez G (2004) Interaction between the human hippocampus and the caudate nucleus during route recognition. Neuron 43:427-435
241. Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP Jr (1985) Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 44:559-577
242. Voorn P, Vanderschuren LJ, Groenewegen HJ, Robbins TW, Pennartz CM (2004) Putting a spin on the dorsal-ventral divide of the striatum. Trends Neurosci 27:468-474
243. Wade A, Jacobs P, Morton AJ (2008) Atrophy and degeneration in sciatic nerve of presymptomatic mice carrying the Huntington's disease mutation. Brain Res 1188:61-68
244. Warby, SC., Graham, RK., and Hayden, M (2008) Huntington's disease. Gene Rev. http://www. ncbi.nim.nih.gov
245. Wheeler VC, Auerbach W, White JK, Srinidhi J, Auerbach A, Ryan A, Duyao MP, Vrbanac V, Weaver M, Gusella JF, Joyner AL, MacDonald ME (1999) Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 8:115-122
246. Wheeler VC, Gutekunst CA, Vrbanac V, Lebel LA, Schilling G, Hersch S, Friedlander RM, Gusella JF, Vonsattel JP, Borchelt DR, MacDonald ME (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
247. Wheeler VC, White JK, Gutekunst CA, Vrbanac V, Weaver M, Li XJ, Li SH, Yi H, Vonsattel JP, Gusella JF, Hersch S, Auerbach W, Joyner AL, MacDonald ME (2000) 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 9:503-513
248. White A, Dunnett SB (2006) Fronto-striatal disconnection disrupts operant delayed alternation performance in the rat. NeuroReport 17:435-441
249. Williams RH, Morton AJ, Burdakov D (2011) Paradoxical function of orexin/hypocretin circuits in a mouse model of Huntington's disease. Neurobiol Dis 42:438-445
250. Wolf RC, Gron G, Sambataro F, Vasic N, Wolf ND, Thomann PA, Saft C, Landwehrmeyer GB, Orth M (2012) Brain activation and functional connectivity in premanifest Huntington's disease during states of intrinsic and phasic alertness. Hum Brain Mapp 33:2161-2173
251. Wood NI, McAllister CJ, Cuesta M, Aungier J, Fraenkel E, Morton AJ (2013) Adaptation to experimental jet-lag in R6/2 mice despite circadian dysrhythmia. PLoS ONE 8:e55036
252. Xie Y, Hayden MR, Xu B (2010) BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice. J Neurosci 30:14708-14718
253. Yuen EY, Wei J, Zhong P, Yan Z (2012) Disrupted GABAAR trafficking and synaptic inhibition in a mouse model of Huntington's disease. Neurobiol Dis 46:497-502
254. Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L, MacDonald ME, Friedlander RM, Silani V, Hayden MR, Timmusk T, Sipione S, Cattaneo E (2001) Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293:493-498
255. Zuccato C, Liber D, Ramos C, Tarditi A, Rigamonti D, Tartari M, Valenza M, Cattaneo E (2005) Progressive loss of BDNF in a mouse model of Huntington's disease and rescue by BDNF delivery. Pharmacol Res 52:133-139
256. Zuccato C, Marullo M, Conforti P, MacDonald ME, Tartari M, Cattaneo E (2008) Systematic assessment of BDNF and its receptor levels in human cortices affected by Huntington's disease. Brain Pathol 18:225-238