The therapeutic potential of G-protein coupled receptors in Huntington's disease

Pharmacology and Therapeutics

Volumn 128, Issue 2, 2010, Pages 305-323

  Dowie, Megan J ^a   Scotter, Emma L ^a   Molinari, Emanuela ^a   Glass, Michelle ^a  

^a UNIVERSITY OF AUCKLAND   (New Zealand)

Author keywords

Adenosine receptor; Cannabinoid receptor; Dopamine receptor; Drug therapy; G protein; Huntington's disease; Metabotropic glutamate receptor; Receptor

Indexed keywords

2 DIMETHYLAMINO 6,7 DIHYDROXYTETRALIN; ADENYLATE CYCLASE; ALPHA 2A ADRENERGIC RECEPTOR; ANTIPARKINSON AGENT; APOMORPHINE; BROMOCRIPTINE; CABERGOLINE; DOPAMINE 2 RECEPTOR; DOPAMINE 3 RECEPTOR; DOPAMINE RECEPTOR STIMULATING AGENT; GLYCOGEN SYNTHASE KINASE 3BETA; LEVODOPA; LISURIDE; PERGOLIDE; PHOSPHOTRANSFERASE; PIRIBEDIL; PRAMIPEXOLE; PROLACTIN; PROTEIN KINASE B; QUINELORANE; QUINPIROLE; ROPINIROLE; ROTIGOTINE; ROXINDOLE; SEROTONIN; SEROTONIN 2B RECEPTOR; SEROTONIN RECEPTOR; TALIPEXOLE; TERGURIDE;

AFFECT; CELL MIGRATION; CLINICAL TRIAL; COGNITION; COGNITIVE DEFECT; CONTINUOUS INFUSION; DEPRESSION; DOPAMINERGIC ACTIVITY; DRUG BINDING; DRUG DOSE TITRATION; DRUG EFFICACY; DRUG MECHANISM; ENVIRONMENTAL FACTOR; HEREDITY; HUMAN; IN VIVO STUDY; MONOTHERAPY; MOTOR DYSFUNCTION; MOTOR PERFORMANCE; NEUROPROTECTION; NONHUMAN; PARKINSON DISEASE; PRIORITY JOURNAL; PROLACTIN RELEASE; PROTEIN PHOSPHORYLATION; REVIEW;

ANIMALS; CEREBRAL CORTEX; DRUG DELIVERY SYSTEMS; HUMANS; HUNTINGTON DISEASE; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; RECEPTORS, G-PROTEIN-COUPLED; SIGNAL TRANSDUCTION; TRINUCLEOTIDE REPEAT EXPANSION;

EID: 77957018891     PISSN: 01637258     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.pharmthera.2010.07.008     Document Type: Review

References (317)

1. Abe T., Sugihara H., Nawa H., Shigemoto R., Mizuno N., Nakanishi S. Molecular characterization of a novel metabotropic glutamate receptor mGluR5 coupled to inositol phosphate/Ca2+ signal transduction. J Biol Chem 1992, 267(19):13361-13368.
2. Adam O.R., Jankovic J. Symptomatic treatment of Huntington disease. Neurotherapeutics 2008, 5(2):181-197.
3. Aiken C.T., Tobin A.J., Schweitzer E.S. A cell-based screen for drugs to treat Huntington's disease. Neurobiol Dis 2004, 16(3):546-555.
4. Albanese A., Cassetta E., Carretta D., Bentivoglio A.R., Tonali P. Acute challenge with apomorphine in Huntington's disease: a double-blind study. Clin Neuropharmacol 1995, 18(5):427-434.
5. Alfinito P.D., Wang S.P., Manzino L., Rijhsinghani S., Zeevalk G.D., Sonsalla P.K. Adenosinergic protection of dopaminergic and GABAergic neurons against mitochondrial inhibition through receptors located in the substantia nigra and striatum, respectively. J Neurosci 2003, 23(34):10982-10987.
6. Allen K.L., Waldvogel H.J., Glass M., Faull R.L. Cannabinoid (CB(1)), GABA(A) and GABA(B) receptor subunit changes in the globus pallidus in Huntington's disease. J Chem Neuroanat 2009, 37(4):266-281.
7. Altar C.A., Cai N., Bliven T., Juhasz M., Conner J.M., Acheson A.L., et al. Anterograde transport of brain-derived neurotrophic factor and its role in the brain. Nature 1997, 389(6653):856-860.
8. Altar C.A., DiStefano P.S. Neurotrophin trafficking by anterograde transport. Trends Neurosci 1998, 21(10):433-437.
9. Anborgh P.H., Godin C., Pampillo M., Dhami G.K., Dale L.B., Cregan S.P., et al. Inhibition of metabotropic glutamate receptor signaling by the huntingtin-binding protein optineurin. J Biol Chem 2005, 280(41):34840-34848.
10. Anborgh P.H., Godin C., Pampillo M., Dhami G.K., Dale L.B., Cregan S.P., et al. Inhibition of metabotropic glutamate receptor signaling by the huntingtin-binding protein optineurin. J Biol Chem 2005, 280(41):34840-34848.
11. Apostol B.L., Illes K., Pallos J., Bodai L., Wu J., Strand A., et al. Mutant huntingtin alters MAPK signaling pathways in PC12 and striatal cells: ERK1/2 protects against mutant huntingtin-associated toxicity. Hum Mol Genet 2006, 15(2):273-285.
12. Apostol B.L., Kazantsev A., Raffioni S., Illes K., Pallos J., Bodai L., et al. A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc Natl Acad Sci USA 2003, 100(10):5950-5955.
13. Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431(7010):805-810.
14. Aubeeluck A., Buchanan H. The Huntington's disease quality of life battery for carers: reliability and validity. Clin Genet 2007, 71(5):434-445.
15. Bachurin S.O., Shevtsova E.P., Kireeva E.G., Oxenkrug G.F., Sablin S.O. Mitochondria as a target for neurotoxins and neuroprotective agents. Ann NY Acad Sci 2003, 993:334-344. discussion 345-339.
16. Bagayogo I.P., Dreyfus C.F. Regulated release of BDNF by cortical oligodendrocytes is mediated through metabotropic glutamate receptors and the PLC pathway. ASN Neuro 2009, 1(1).
17. Balasubramanian S., Teissere J.A., Raju D.V., Hall R.A. Hetero-oligomerization between GABAA and GABAB receptors regulates GABAB receptor trafficking. J Biol Chem 2004, 279(18):18840-18850.
18. Baquet Z.C., Gorski J.A., Jones K.R. Early striatal dendrite deficits followed by neuron loss with advanced age in the absence of anterograde cortical brain-derived neurotrophic factor. J Neurosci 2004, 24(17):4250-4258.
19. Bates G.P., Hockly E. Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?. Curr Opin Neurol 2003, 16(4):465-470.
20. Battista N., Bari M., Tarditi A., Mariotti C., Bachoud-Levi A.C., Zuccato C., et al. Severe deficiency of the fatty acid amide hydrolase (FAAH) activity segregates with the Huntington's disease mutation in peripheral lymphocytes. Neurobiol Dis 2007, 27(1):108-116.
21. Bauer P.O., Nukina N. The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies. J Neurochem 2009, 110(6):1737-1765.
22. Bauer P.O., Wong H.K., Oyama F., Goswami A., Okuno M., Kino Y., et al. Inhibition of Rho kinases enhances the degradation of mutant huntingtin. J Biol Chem 2009, 284(19):13153-13164.
23. Beal M.F., Brouillet E., Jenkins B., Henshaw R., Rosen B., Hyman B.T. Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate. J Neurochem 1993, 61(3):1147-1150.
24. Beal M.F., Kowall N.W., Ellison D.W., Mazurek M.F., Swartz K.J., Martin J.B. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature 1986, 321(6066):168-171.
25. Beal M.F., Kowall N.W., Swartz K.J., Ferrante R.J., Martin J.B. Systemic approaches to modifying quinolinic acid striatal lesions in rats. J Neurosci 1988, 8(10):3901-3908.
26. Becher M.W., Kotzuk J.A., Sharp A.H., Davies S.W., Bates G.P., Price D.L., et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998, 4(6):387-397.
27. Benchoua A., Trioulier Y., Diguet E., Malgorn C., Gaillard M.C., Dufour N., et al. Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II. Hum Mol Genet 2008, 17(10):1446-1456.
28. Bithell A., Johnson R., Buckley N.J. Transcriptional dysregulation of coding and non-coding genes in cellular models of Huntington's disease. Biochem Soc Trans 2009, 37(Pt 6):1270-1275.
29. Blum D., Gall D., Galas M.C., d'Alcantara P., Bantubungi K., Schiffmann S.N. The adenosine A1 receptor agonist adenosine amine congener exerts a neuroprotective effect against the development of striatal lesions and motor impairments in the 3-nitropropionic acid model of neurotoxicity. J Neurosci 2002, 22(20):9122-9133.
30. Blum D., Hourez R., Galas M.C., Popoli P., Schiffmann S.N. Adenosine receptors and Huntington's disease: implications for pathogenesis and therapeutics. Lancet Neurol 2003, 2(6):366-374.
31. Bodner R.A., Outeiro T.F., Altmann S., Maxwell M.M., Cho S.H., Hyman B.T., et al. Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases. Proc Natl Acad Sci USA 2006, 103(11):4246-4251.
32. Boehning D., Patterson R.L., Sedaghat L., Glebova N.O., Kurosaki T., Snyder S.H. Cytochrome c binds to inositol (1, 4, 5) trisphosphate receptors, amplifying calcium-dependent apoptosis. Nat Cell Biol 2003, 5(12):1051-1061.
33. Bonelli R.M. Mirtazapine in suicidal Huntington's disease. Ann Pharmacother 2003, 37(3):452.
34. Bonelli R.M., Wenning G.K. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des 2006, 12(21):2701-2720.
35. Borrell-Pages M., Canals J.M., Cordelieres F.P., Parker J.A., Pineda J.R., Grange G., et al. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J Clin Invest 2006, 116(5):1410-1424.
36. Borrell-Pages M., Zala D., Humbert S., Saudou F. Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies. Cell Mol Life Sci 2006, 63(22):2642-2660.
37. Borsello T., Forloni G. JNK signalling: a possible target to prevent neurodegeneration. Curr Pharm Des 2007, 13(18):1875-1886.
38. Bouaboula M., Poinot-Chazel C., Bourrie B., Canat X., Calandra B., Rinaldi-Carmona M., et al. Activation of mitogen-activated protein kinases by stimulation of the central cannabinoid receptor CB1. Biochem J 1995, 312(2):637-641.
39. Brambilla R., Cottini L., Fumagalli M., Ceruti S., Abbracchio M.P. Blockade of A2A adenosine receptors prevents basic fibroblast growth factor-induced reactive astrogliosis in rat striatal primary astrocytes. Glia 2003, 43(2):190-194.
40. Brami-Cherrier K., Valjent E., Garcia M., Pages C., Hipskind R.A., Caboche J. Dopamine induces a PI3-kinase-independent activation of Akt in striatal neurons: a new route to cAMP response element-binding protein phosphorylation. J Neurosci 2002, 22(20):8911-8921.
41. Brandt J., Folstein S.E., Folstein M.F. Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol 1988, 23(6):555-561.
42. Browne S.E., Bowling A.C., MacGarvey U., Baik M.J., Berger S.C., Muqit M.M., et al. Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann Neurol 1997, 41(5):646-653.
43. Bruno V., Ksiazek I., Battaglia G., Lukic S., Leonhardt T., Sauer D., et al. Selective blockade of metabotropic glutamate receptor subtype 5 is neuroprotective. Neuropharmacology 2000, 39(12):2223-2230.
44. Butovsky E., Juknat A., Goncharov I., Elbaz J., Eilam R., Zangen A., et al. In vivo up-regulation of brain-derived neurotrophic factor in specific brain areas by chronic exposure to Delta-tetrahydrocannabinol. J Neurochem 2005, 93(4):802-811.
45. Castro M.E., Pascual J., Romon T., Berciano J., Figols J., Pazos A. 5-HT1B receptor binding in degenerative movement disorders. Brain Res 1998, 790(1-2):323-328.
46. Centonze D., Battistini L., Maccarrone M. The endocannabinoid system in peripheral lymphocytes as a mirror of neuroinflammatory diseases. Curr Pharm Des 2008, 14(23):2370-2382.
47. Cha J.H., Frey A.S., Alsdorf S.A., Kerner J.A., Kosinski C.M., Mangiarini L., et al. Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 1999, 354(1386):981-989.
48. Cha J.H., Kosinski C.M., Kerner J.A., Alsdorf S.A., Mangiarini L., Davies S.W., et al. Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc Natl Acad Sci USA 1998, 95(11):6480-6485.
49. Charvin D., Roze E., Perrin V., Deyts C., Betuing S., Pages C., et al. Haloperidol protects striatal neurons from dysfunction induced by mutated huntingtin in vivo. Neurobiol Dis 2008, 29(1):22-29.
50. Charvin D., Vanhoutte P., Pages C., Borrelli E., Caboche J. Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulation. Proc Natl Acad Sci USA 2005, 102(34):12218-12223.
51. Chen J.F., Xu K., Petzer J.P., Staal R., Xu Y.H., Beilstein M., et al. Neuroprotection by caffeine and A(2A) adenosine receptor inactivation in a model of Parkinson's disease. J Neurosci 2001, 21(10):RC143.
52. Chen S., Berthelier V., Yang W., Wetzel R. Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity. J Mol Biol 2001, 311(1):173-182.
53. Chen S., Zhang X., Yang D., Du Y., Li L., Li X., et al. D2/D3 receptor agonist ropinirole protects dopaminergic cell line against rotenone-induced apoptosis through inhibition of caspase- and JNK-dependent pathways. FEBS Lett 2008, 582(5):603-610.
54. Chiang M.C., Chen H.M., Lai H.L., Chen H.W., Chou S.Y., Chen C.M., et al. The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin-proteasome system. Hum Mol Genet 2009, 18(16):2929-2942.
55. Choe E.S., Wang J.Q. Group I metabotropic glutamate receptors control phosphorylation of CREB, Elk-1 and ERK via a CaMKII-dependent pathway in rat striatum. Neurosci Lett 2001, 313(3):129-132.
56. Chou S.Y., Lee Y.C., Chen H.M., Chiang M.C., Lai H.L., Chang H.H., et al. CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model. J Neurochem 2005, 93(2):310-320.
57. Cipriani S., Bizzoco E., Gianfriddo M., Melani A., Vannucchi M.G., Pedata F. Adenosine A2A receptor antagonism increases nNOS-immunoreactive neurons in the striatum of Huntington transgenic mice. Exp Neurol 2008, 213(1):163-170.
58. Cooper J.K., Schilling G., Peters M.F., Herring W.J., Sharp A.H., Kaminsky Z., et al. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum Mol Genet 1998, 7(5):783-790.
59. Corbascio A.N. Acetylcholine replacement therapy in the menagement of huntington's disease. West J Med 1981, 135(5):426-427.
60. Corsini G.U., Onali P., Masala C., Cianchetti C., Mangoni A., Gessa G. Apomorphine hydrochloride-induced improvement in Huntington's chorea: stimulation of dopamine receptor. Arch Neurol 1978, 35(1):27-30.
61. Curtis A., Mitchell I., Patel S., Ives N., Rickards H. A pilot study using nabilone for symptomatic treatment in Huntington's disease. Mov Disord 2009, 24(15):2254-2259.
62. Curtis A., Rickards H. Nabilone could treat chorea and irritability in Huntington's disease. J Neuropsychiatry Clin Neurosci 2006, 18(4):553-554.
63. Cyr M., Sotnikova T.D., Gainetdinov R.R., Caron M.G. Dopamine enhances motor and neuropathological consequences of polyglutamine expanded huntingtin. FASEB J 2006, 20(14):2541-2543.
64. D'Onofrio M., Cuomo L., Battaglia G., Ngomba R.T., Storto M., Kingston A.E., et al. Neuroprotection mediated by glial group-II metabotropic glutamate receptors requires the activation of the MAP kinase and the phosphatidylinositol-3-kinase pathways. J Neurochem 2001, 78(3):435-445.
65. Davis K.L., Berger P.A. Pharmacological investigations of the cholinergic imbalance hypotheses of movement disorders and psychosis. Biol Psychiatry 1978, 13(1):23-49.
66. de Almeida L.P., Ross C.A., Zala D., Aebischer P., Deglon N. Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length. J Neurosci 2002, 22(9):3473-3483.
67. De Duve C. The lysosome. Sci Am 1963, 208:64-72.
68. de Lago E., Fernandez-Ruiz J., Ortega-Gutierrez S., Cabranes A., Pryce G., Baker D., et al. UCM707, an inhibitor of the anandamide uptake, behaves as a symptom control agent in models of Huntington's disease and multiple sclerosis, but fails to delay/arrest the progression of different motor-related disorders. Eur Neuropsychopharmacol 2006, 16(1):7-18.
69. de Lago E., Urbani P., Ramos J.A., Di Marzo V., Fernandez-Ruiz J. Arvanil, a hybrid endocannabinoid and vanilloid compound, behaves as an antihyperkinetic agent in a rat model of Huntington's disease. Brain Res 2005, 1050(1-2):210-216.
70. De Marco N., Buono M., Troise F., Diez-Roux G. Optineurin increases cell survival and translocates to the nucleus in a Rab8-dependent manner upon an apoptotic stimulus. J Biol Chem 2006, 281(23):16147-16156.
71. Derkinderen P., Valjent E., Toutant M., Corvol J.C., Enslen H., Ledent C., et al. Regulation of extracellular signal-regulated kinase by cannabinoids in hippocampus. J Neurosci 2003, 23(6):2371-2382.
72. Deyts C., Galan-Rodriguez B., Martin E., Bouveyron N., Roze E., Charvin D., et al. Dopamine D2 receptor stimulation potentiates PolyQ-Huntingtin-induced mouse striatal neuron dysfunctions via Rho/ROCK-II activation. PLoS ONE 2009, 4(12):e8287.
73. Doble A. The role of excitotoxicity in neurodegenerative disease: implications for therapy. Pharmacol Ther 1999, 81(3):163-221.
74. Domenici M.R., Scattoni M.L., Martire A., Lastoria G., Potenza R.L., Borioni A., et al. Behavioral and electrophysiological effects of the adenosine A2A receptor antagonist SCH 58261 in R6/2 Huntington's disease mice. Neurobiol Dis 2007, 28(2):197-205.
75. Doody R.S., Gavrilova S.I., Sano M., Thomas R.G., Aisen P.S., Bachurin S.O., et al. Effect of dimebon on cognition, activities of daily living, behaviour, and global function in patients with mild-to-moderate Alzheimer's disease: a randomised, double-blind, placebo-controlled study. Lancet 2008, 372(9634):207-215.
76. Dowie, M. J., Howard, M. L., Nicholson, L. F., Faull, R. L., Hannan, A. J., & Glass, M. (2010). Behavioural and molecular consequences of chronic cannabinoid treatment in Huntington's disease transgenic mice. Neuroscience. doi:10.1016/j.neuroscience.2010.06.056.
77. Duan W., Guo Z., Jiang H., Ladenheim B., Xu X., Cadet J.L., et al. Paroxetine retards disease onset and progression in Huntingtin mutant mice. Ann Neurol 2004, 55(4):590-594.
78. Duncan R.S., Goad D.L., Grillo M.A., Kaja S., Payne A.J., Koulen P. Control of intracellular calcium signaling as a neuroprotective strategy. Molecules 2010, 15(3):1168-1195.
79. Duvernay M.T., Filipeanu C.M., Wu G. The regulatory mechanisms of export trafficking of G protein-coupled receptors. Cell Signal 2005, 17(12):1457-1465.
80. Duyao M., Ambrose C., Myers R., Novelletto A., Persichetti F., Frontali M., et al. Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet 1993, 4(4):387-392.
81. Enna S.J., Bird E.D., Bennett J.P., Bylund D.B., Yamamura H.I., Iversen L.L., et al. Huntington's chorea. Changes in neurotransmitter receptors in the brain. N Engl J Med 1976, 294(24):1305-1309.
82. Fan M.M., Fernandes H.B., Zhang L.Y., Hayden M.R., Raymond L.A. Altered NMDA receptor trafficking in a yeast artificial chromosome transgenic mouse model of Huntington's disease. J Neurosci 2007, 27(14):3768-3779.
83. Fasano A., Cadeddu F., Guidubaldi A., Piano C., Soleti F., Zinzi P., et al. The long-term effect of tetrabenazine in the management of Huntington disease. Clin Neuropharmacol 2008, 31(6):313-318.
84. Ferrante R.J., Kowall N.W., Beal M.F., Richardson E.P., Bird E.D., Martin J.B. Selective sparing of a class of striatal neurons in Huntington's disease. Science 1985, 230(4725):561-563.
85. Ferre S., Snaprud P., Fuxe K. Opposing actions of an adenosine A2 receptor agonist and a GTP analogue on the regulation of dopamine D2 receptors in rat neostriatal membranes. Eur J Pharmacol 1993, 244(3):311-315.
86. Fink J.S., Kalda A., Ryu H., Stack E.C., Schwarzschild M.A., Chen J.F., et al. Genetic and pharmacological inactivation of the adenosine A2A receptor attenuates 3-nitropropionic acid-induced striatal damage. J Neurochem 2004, 88(3):538-544.
87. Ford M.F. Treatment of depression in Huntington's disease with monoamine oxidase inhibitors. Br J Psychiatry 1986, 149:654-656.
88. Freiman R.N., Tjian R. Neurodegeneration. A glutamine-rich trail leads to transcription factors. Science 2002, 296(5576):2149-2150.
89. Garcia M., Vanhoutte P., Pages C., Besson M.J., Brouillet E., Caboche J. The mitochondrial toxin 3-nitropropionic acid induces striatal neurodegeneration via a c-Jun N-terminal kinase/c-Jun module. J Neurosci 2002, 22(6):2174-2184.
90. Gasque P., Dean Y.D., McGreal E.P., VanBeek J., Morgan B.P. Complement components of the innate immune system in health and disease in the CNS. Immunopharmacology 2000, 49(1-2):171-186.
91. Genovese T., Melani A., Esposito E., Mazzon E., Di Paola R., Bramanti P., et al. The selective adenosine A2A receptor agonist CGS 21680 reduces JNK MAPK activation in oligodendrocytes in injured spinal cord. Shock 2009, 32(6):578-585.
92. Giampa C., Patassini S., Borreca A., Laurenti D., Marullo F., Bernardi G., et al. Phosphodiesterase 10 inhibition reduces striatal excitotoxicity in the quinolinic acid model of Huntington's disease. Neurobiol Dis 2009, 34(3):450-456.
93. Gianfriddo M., Melani A., Turchi D., Giovannini M.G., Pedata F. Adenosine and glutamate extracellular concentrations and mitogen-activated protein kinases in the striatum of Huntington transgenic mice. Selective antagonism of adenosine A2A receptors reduces transmitter outflow. Neurobiol Dis 2004, 17(1):77-88.
94. Gines S., Seong I.S., Fossale E., Ivanova E., Trettel F., Gusella J.F., et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet 2003, 12(5):497-508.
95. Gines S., Seong I.S., Fossale E., Ivanova E., Trettel F., Gusella J.F., et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet 2003, 12(5):497-508.
96. Giorgini F., Moller T., Kwan W., Zwilling D., Wacker J.L., Hong S., et al. Histone deacetylase inhibition modulates kynurenine pathway activation in yeast, microglia, and mice expressing a mutant huntingtin fragment. J Biol Chem 2008, 283(12):7390-7400.
97. Glass M., Dragunow M., Faull R.L. 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 2000, 97(3):505-519.
98. Glass M., Felder C.C. Concurrent stimulation of cannabinoid CB1 and dopamine D2 receptors augments cAMP accumulation in striatal neurons: evidence for a Gs linkage to the CB1 receptor. J Neurosci 1997, 17(14):5327-5333.
99. Glass M., van Dellen A., Blakemore C., Hannan A.J., Faull R.L.M. Delayed onset of Huntington's Disease in mice in an enriched environment correlates with delayed loss of cannabinoid CB1 receptors. Neuroscience 2004, 123(1):207-212.
100. Goehler H., Lalowski M., Stelzl U., Waelter S., Stroedicke M., Worm U., et al. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. Mol Cell 2004, 15(6):853-865.
101. Goffredo D., Rigamonti D., Tartari M., De Micheli A., Verderio C., Matteoli M., et al. Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons. J Biol Chem 2002, 277(42):39594-39598.
102. Gokce O., Runne H., Kuhn A., Luthi-Carter R. Short-term striatal gene expression responses to brain-derived neurotrophic factor are dependent on MEK and ERK activation. PLoS ONE 2009, 4(4):e5292.
103. Goldberg Y.P., Nicholson D.W., Rasper D.M., Kalchman M.A., Koide H.B., Graham R.K., et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat Genet 1996, 13(4):442-449.
104. Goodchild R.E., Court J.A., Hobson I., Piggott M.A., Perry R.H., Ince P., et al. Distribution of histamine H3-receptor binding in the normal human basal ganglia: comparison with Huntington's and Parkinson's disease cases. Eur J Neurosci 1999, 11(2):449-456.
105. Graveland G.A., Williams R.S., DiFiglia M. Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science 1985, 227(4688):770-773.
106. Gray M., Shirasaki D.I., Cepeda C., Andre V.M., Wilburn B., Lu X.H., et al. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J Neurosci 2008, 28(24):6182-6195.
107. Griffiths M., Neal J.W., Gasque P. Innate immunity and protective neuroinflammation: new emphasis on the role of neuroimmune regulatory proteins. Int Rev Neurobiol 2007, 82:29-55.
108. Grote H.E., Bull N.D., Howard M.L., van Dellen A., Blakemore C., Bartlett P.F., et al. Cognitive disorders and neurogenesis deficits in Huntington's disease mice are rescued by fluoxetine. Eur J Neurosci 2005, 22(8):2081-2088.
109. Growdon J.H., Cohen E.L., Wurtman R.J. Huntington's disease: clinical and chemical effects of choline administration. Ann Neurol 1977, 1(5):418-422.
110. Guidetti P., Luthi-Carter R.E., Augood S.J., Schwarcz R. Neostriatal and cortical quinolinate levels are increased in early grade Huntington's disease. Neurobiol Dis 2004, 17(3):455-461.
111. Guidetti P., Bates G.P., Graham R.K., Hayden M.R., Leavitt B.R., MacDonald M.E., et al. Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol Dis 2006, 2(1):190-197.
112. Gutekunst C.A., Li S.H., Yi H., Mulroy J.S., Kuemmerle S., Jones R., et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci 1999, 19(7):2522-2534.
113. Hannon J., Hoyer D. Molecular biology of 5-HT receptors. Behav Brain Res 2008, 195(1):198-213.
114. Hanyaloglu A.C., von Zastrow M. Regulation of GPCRs by endocytic membrane trafficking and its potential implications. Annu Rev Pharmacol Toxicol 2008, 48:537-568.
115. Harjes P., Wanker E.E. The hunt for huntingtin function: interaction partners tell many different stories. Trends Biochem Sci 2003, 28(8):425-433.
116. Hasbi A., Fan T., Alijaniaram M., Nguyen T., Perreault M.L., O'Dowd B.F., et al. Calcium signaling cascade links dopamine D1-D2 receptor heteromer to striatal BDNF production and neuronal growth. Proc Natl Acad Sci USA 2009, 106(50):21377-21382.
117. Hayden M.R., Leavitt B.R., Yasothan U., Kirkpatrick P. Tetrabenazine. Nat Rev Drug Discov 2009, 8(1):17-18.
118. Hays S.E., Goodwin F.K., Paul S.M. Cholecystokinin receptors are decreased in basal ganglia and cerebral cortex of Huntington's disease. Brain Res 1981, 225(2):452-456.
119. Hettinger B.D., Lee A., Linden J., Rosin D.L. Ultrastructural localization of adenosine A2A receptors suggests multiple cellular sites for modulation of GABAergic neurons in rat striatum. J Comp Neurol 2001, 431(3):331-346.
120. Hisatsune C., Kuroda Y., Akagi T., Torashima T., Hirai H., Hashikawa T., et al. Inositol 1, 4, 5-trisphosphate receptor type 1 in granule cells, not in Purkinje cells, regulates the dendritic morphology of Purkinje cells through brain-derived neurotrophic factor production. J Neurosci 2006, 26(42):10916-10924.
121. Ho L.W., Carmichael J., Swartz J., Wyttenbach A., Rankin J., Rubinsztein D.C. The molecular biology of Huntington's disease. Psychol Med 2001, 31(1):3-14.
122. Hockly E., Richon V.M., Woodman B., Smith D.L., Zhou X., Rosa E., et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc Natl Acad Sci USA 2003, 100(4):2041-2046.
123. Hodges A., Strand A.D., Aragaki A.K., Kuhn A., Sengstag T., Hughes G., et al. Regional and cellular gene expression changes in human Huntington's disease brain. Hum Mol Genet 2006, 15(6):965-977.
124. Hodgson J.G., Agopyan N., Gutekunst C.A., Leavitt B.R., LePiane F., Singaraja R., et al. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 1999, 23(1):181-192.
125. Horn S.C., Lalowski M., Goehler H., Droge A., Wanker E.E., Stelzl U. Huntingtin interacts with the receptor sorting family protein GASP2. J Neural Transm 2006, 113(8):1081-1090.
126. Hoyt K.R., Reynolds I.J., Hastings T.G. Mechanisms of dopamine-induced cell death in cultured rat forebrain neurons: interactions with and differences from glutamate-induced cell death. Exp Neurol 1997, 143(2):269-281.
127. Huang C.C., Faber P.W., Persichetti F., Mittal V., Vonsattel J.P., MacDonald M.E., et al. Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins. Somat Cell Mol Genet 1998, 24(4):217-233.
128. Huntington's Disease Collaborative Research Group A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993, 72(6):971-983.
129. Huntington Study Group Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 2003, 61(11):1551-1556.
130. Igarashi S., Morita H., Bennett K.M., Tanaka Y., Engelender S., Peters M.F., et al. Inducible PC12 cell model of Huntington's disease shows toxicity and decreased histone acetylation. NeuroReport 2003, 14(4):565-568.
131. Jackson G.R., Salecker I., Dong X., Yao X., Arnheim N., Faber P.W., et al. Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron 1998, 21(3):633-642.
132. Jacobson K.A., Hoffmann C., Cattabeni F., Abbracchio M.P. Adenosine-induced cell death: evidence for receptor-mediated signalling. Apoptosis 1999, 4(3):197-211.
133. Jana N.R., Zemskov E.A., Wang G., Nukina N. Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Hum Mol Genet 2001, 10(10):1049-1059.
134. Jankovic J., Orman J. Tetrabenazine therapy of dystonia, chorea, tics, and other dyskinesias. Neurology 1988, 38(3):391-394.
135. Jenner P., Mori A., Hauser R., Morelli M., Fredholm B.B., Chen J.F. Adenosine, adenosine A 2A antagonists, and Parkinson's disease. Parkinsonism Relat Disord 2009, 15(6):406-413.
136. Jung C.H., Jun C.B., Ro S.-H., Kim Y.-M., Otto N.M., Cao J., et al. ULK-Atg13-FIP200 complexes mediate mTOR signaling to the autophagy machinery. Mol Biol Cell 2009, 20(7):1992-2003.
137. Kazantsev A., Preisinger E., Dranovsky A., Goldgaber D., Housman D. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc Natl Acad Sci USA 1999, 96(20):11404-11409.
138. Kazantsev A., Preisinger E., Dranovsky A., Goldgaber D., Housman D. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc Natl Acad Sci USA 1999, 96(20):11404-11409.
139. Kenakin T. Agonist-receptor efficacy. I: Mechanisms of efficacy and receptor promiscuity. Trends Pharmacol Sci 1995, 16(6):188-192.
140. Kenakin T. Ligand-selective receptor conformations revisited: the promise and the problem. Trends Pharmacol Sci 2003, 24(7):346-354.
141. Khaspekov L.G., Brenz Verca M.S., Frumkina L.E., Hermann H., Marsicano G., Lutz B. Involvement of brain-derived neurotrophic factor in cannabinoid receptor-dependent protection against excitotoxicity. Eur J Neurosci 2004, 19(7):1691-1699.
142. Kieburtz K., McDermott M.P., Voss T.S., Corey-Bloom J., Deuel L.M., Dorsey E.R., et al. A randomized, placebo-controlled trial of latrepirdine in Huntington disease. Arch Neurol 2010, 67(2):154-160.
143. Kim M., Velier J., Chase K., Laforet G., Kalchman M.A., Hayden M.R., et al. Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin. Neuroscience 1999, 89(4):1159-1167.
144. Kim Y.J., Yi Y., Sapp E., Wang Y., Cuiffo B., Kegel K.B., et al. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci USA 2001, 98(22):12784-12789.
145. Kittler J.T., Thomas P., Tretter V., Bogdanov Y.D., Haucke V., Smart T.G., et al. Huntingtin-associated protein 1 regulates inhibitory synaptic transmission by modulating gamma-aminobutyric acid type A receptor membrane trafficking. Proc Natl Acad Sci USA 2004, 101(34):12736-12741.
146. Klivenyi P., Bende Z., Hartai Z., Penke Z., Nemeth H., Toldi J., et al. Behaviour changes in a transgenic model of Huntington's disease. Behav Brain Res 2006, 169(1):137-141.
147. Kuemmerle S., Gutekunst C.A., Klein A.M., Li X.J., Li S.H., Beal M.F., et al. Huntington aggregates may not predict neuronal death in Huntington's disease. Ann Neurol 1999, 46(6):842-849.
148. Kumar U. Somatostatin in medium-sized aspiny interneurons of striatum is responsible for their preservation in quinolinic acid and N-methyl-d-asparate-induced neurotoxicity. J Mol Neurosci 2008, 35(3):345-354.
149. Landwehrmeyer G.B., Dubois B., de Yebenes J.G., Kremer B., Gaus W., Kraus P.H., et al. Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol 2007, 62(3):262-272.
150. Lastres-Becker I., Bizat N., Boyer F., Hantraye P., Brouillet E., Fernandez-Ruiz J. Effects of cannabinoids in the rat model of Huntington's disease generated by an intrastriatal injection of malonate. NeuroReport 2003, 14(6):813-816.
151. Lastres-Becker I., Bizat N., Boyer F., Hantraye P., Fernandez-Ruiz J., Brouillet E. Potential involvement of cannabinoid receptors in 3-nitropropionic acid toxicity in vivo. NeuroReport 2004, 15(15):2375-2379.
152. Lastres-Becker I., de Miguel R., De Petrocellis L., Makriyannis A., Di Marzo V., Fernandez-Ruiz J. Compounds acting at the endocannabinoid and/or endovanilloid systems reduce hyperkinesia in a rat model of Huntington's disease. J Neurochem 2003, 84(5):1097-1109.
153. Lastres-Becker I., Hansen H.H., Berrendero F., De Miguel R., Perez-Rosado A., Manzanares J., et al. Alleviation of motor hyperactivity and neurochemical deficits by endocannabinoid uptake inhibition in a rat model of Huntington's disease. Synapse 2002, 44(1):23-35.
154. Lauckner J.E., Hille B., Mackie K. The cannabinoid agonist WIN55, 212-2 increases intracellular calcium via CB1 receptor coupling to Gq/11 G proteins. Proc Natl Acad Sci USA 2005, 102(52):19144-19149.
155. Leach K., Sexton P.M., Christopoulos A. Allosteric GPCR modulators: taking advantage of permissive receptor pharmacology. Trends Pharmacol Sci 2007, 28(8):382-389.
156. Lee F.S., Chao M.V. Activation of Trk neurotrophin receptors in the absence of neurotrophins. Proc Natl Acad Sci USA 2001, 98(6):3555-3560.
157. Li S.H., Cheng A.L., Zhou H., Lam S., Rao M., Li H., et al. Interaction of Huntington disease protein with transcriptional activator Sp1. Mol Cell Biol 2002, 22(5):1277-1287.
158. Li S.H., Li X.J. Huntingtin-protein interactions and the pathogenesis of Huntington's disease. Trends Genet 2004, 20(3):146-154.
159. Lievens J.C., Iche M., Laval M., Faivre-Sarrailh C., Birman S. AKT-sensitive or insensitive pathways of toxicity in glial cells and neurons in Drosophila models of Huntington's disease. Hum Mol Genet 2008, 17(6):882-894.
160. Lin C.H., Tallaksen-Greene S., Chien W.M., Cearley J.A., Jackson W.S., Crouse A.B., et al. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 2001, 10(2):137-144.
161. Liu Y.F. Expression of polyglutamine-expanded huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line. J Biol Chem 1998, 273(44):28873-28877.
162. Liu Z., Zhang J., Zhang A. Design of multivalent ligand targeting G-protein-coupled receptors. Curr Pharm Des 2009, 15(6):682-718.
163. Lunkes A., Mandel J.L. A cellular model that recapitulates major pathogenic steps of Huntington's disease. Hum Mol Genet 1998, 7(9):1355-1361.
164. Luo Y., Kokkonen G.C., Wang X., Neve K.A., Roth G.S. D2 dopamine receptors stimulate mitogenesis through pertussis toxin-sensitive G proteins and Ras-involved ERK and SAP/JNK pathways in rat C6-D2L glioma cells. J Neurochem 1998, 71(3):980-990.
165. Luthi-Carter R., Strand A., Peters N.L., Solano S.M., Hollingsworth Z.R., Menon A.S., et al. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet 2000, 9(9):1259-1271.
166. Maccarrone M., Battista N., Centonze D. The endocannabinoid pathway in Huntington's disease: a comparison with other neurodegenerative diseases. Prog Neurobiol 2007, 81(5-6):349-379.
167. Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87(3):493-506.
168. Marder K., Zhao H., Myers R.H., Cudkowicz M., Kayson E., Kieburtz K., et al. Rate of functional decline in Huntington's disease. Huntington Study Group. Neurology 2000, 54(2):452-458.
169. Marsicano G., Goodenough S., Monory K., Hermann H., Eder M., Cannich A., et al. CB1 cannabinoid receptors and on-demand defense against excitotoxicity. Science 2003, 302(5642):84-88.
170. Martel J., Chopin P., Colpaert F., Marien M. Neuroprotective effects of the alpha2-adrenoceptor antagonists, (+)-efaroxan and (+/-)-idazoxan, against quinolinic acid-induced lesions of the rat striatum. Exp Neurol 1998, 154(2):595-601.
171. Martel J.C., Chopin P., Colpaert F., Marien M. Drug treatments to reduce excitotoxicity in vivo: a potential for alpha2-adrenoceptor antagonists?. Amino Acids 2000, 19(1):239-252.
172. Martinez-Mir M.I., Pollard H., Moreau J., Traiffort E., Ruat M., Schwartz J.C., et al. Loss of striatal histamine H2 receptors in Huntington's chorea but not in Parkinson's disease: comparison with animal models. Synapse 1993, 15(3):209-220.
173. Martire A., Calamandrei G., Felici F., Scattoni M.L., Lastoria G., Domenici M.R., et al. Opposite effects of the A2A receptor agonist CGS21680 in the striatum of Huntington's disease versus wild-type mice. Neurosci Lett 2007, 417(1):78-83.
174. Mary V., Wahl F., Stutzmann J.M. Effect of riluzole on quinolinate-induced neuronal damage in rats: comparison with blockers of glutamatergic neurotransmission. Neurosci Lett 1995, 201(1):92-96.
175. Mason S.L., Barker R.A. Emerging drug therapies in Huntington's disease. Expert Opin Emerg Drugs 2009, 14(2):273-297.
176. Masu M., Tanabe Y., Tsuchida K., Shigemoto R., Nakanishi S. Sequence and expression of a metabotropic glutamate receptor. Nature 1991, 349(6312):760-765.
177. Mattammal M.B., Haring J.H., Chung H.D., Raghu G., Strong R. An endogenous dopaminergic neurotoxin: implication for Parkinson's disease. Neurodegeneration 1995, 4(3):271-281.
178. McGeer E.G., Jakubovic A., Singh E.A. Ethanol, baclofen, and kainic acid neurotoxicity. Exp Neurol 1980, 69(2):359-364.
179. McGeer P.L., McGeer E.G., Fibiger H.C. Choline acetylase and glutamic acid decarboxylase in Huntington's chorea. A preliminary study. Neurology 1973, 23(9):912-917.
180. McLaughlin B.A., Nelson D., Erecinska M., Chesselet M.F. Toxicity of dopamine to striatal neurons in vitro and potentiation of cell death by a mitochondrial inhibitor. J Neurochem 1998, 70(6):2406-2415.
181. Melani A., Cipriani S., Vannucchi M.G., Nosi D., Donati C., Bruni P., et al. Selective adenosine A2a receptor antagonism reduces JNK activation in oligodendrocytes after cerebral ischaemia. Brain 2009, 132(Pt 6):1480-1495.
182. Menalled L.B., Sison J.D., Dragatsis I., Zeitlin S., Chesselet M.F. 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 2003, 465(1):11-26.
183. Metzler M., Li B., Gan L., Georgiou J., Gutekunst C.A., Wang Y., et al. Disruption of the endocytic protein HIP1 results in neurological deficits and decreased AMPA receptor trafficking. EMBO J 2003, 22(13):3254-3266.
184. Micale V., Mazzola C., Drago F. Endocannabinoids and neurodegenerative diseases. Pharmacol Res 2007, 56(5):382-392.
185. Monopoli A., Lozza G., Forlani A., Mattavelli A., Ongini E. Blockade of adenosine A2A receptors by SCH 58261 results in neuroprotective effects in cerebral ischaemia in rats. NeuroReport 1998, 9(17):3955-3959.
186. Montminy M.R., Sevarino K.A., Wagner J.A., Mandel G., Goodman R.H. Identification of a cyclic-AMP-responsive element within the rat somatostatin gene. Proc Natl Acad Sci USA 1986, 83(18):6682-6686.
187. Morelli M., Wardas J. Adenosine A(2a) receptor antagonists: potential therapeutic and neuroprotective effects in Parkinson's disease. Neurotox Res 2001, 3(6):545-556.
188. Mori Y., Higuchi M., Masuyama N., Gotoh Y. Adenosine A2A receptor facilitates calcium-dependent protein secretion through the activation of protein kinase A and phosphatidylinositol-3 kinase in PC12 cells. Cell Struct Funct 2004, 29(4):101-110.
189. Mukherjee P., Pasinetti G.M. The role of complement anaphylatoxin C5a in neurodegeneration: implications in Alzheimer's disease. J Neuroimmunol 2000, 105(2):124-130.
190. Muller-Vahl K.R., Schneider U., Emrich H.M. Nabilone increases choreatic movements in Huntington's disease. Mov Disord 1999, 14(6):1038-1040.
191. Nance M.A., Myers R.H. Juvenile onset Huntington's disease-clinical and research perspectives. Ment Retard Dev Disabil Res Rev 2001, 7(3):153-157.
192. Nemeroff C.B., Youngblood W.W., Manberg P.J., Prange A.J., Kizer J.S. Regional brain concentrations of neuropeptides in Huntington's chorea and schizophrenia. Science 1983, 221(4614):972-975.
193. Neubig R.R., Siderovski D.P. Regulators of G-protein signalling as new central nervous system drug targets. Nat Rev Drug Discov 2002, 1(3):187-197.
194. Niswender C.M., Conn P.J. Metabotropic glutamate receptors: physiology, pharmacology, and disease. Annu Rev Pharmacol Toxicol 2010, 50:295-322.
195. Nithianantharajah J., Hannan A.J. Enriched environments, experience-dependent plasticity and disorders of the nervous system. Nat Rev Neurosci 2006, 7(9):697-709.
196. Norris P.J., Waldvogel H.J., Faull R.L., Love D.R., Emson P.C. Decreased neuronal nitric oxide synthase messenger RNA and somatostatin messenger RNA in the striatum of Huntington's disease. Neuroscience 1996, 72(4):1037-1047.
197. Nucifora F.C., Sasaki M., Peters M.F., Huang H., Cooper J.K., Yamada M., et al. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 2001, 291(5512):2423-2428.
198. Nutt J.G. Effect of cholinergic agents in Huntington's disease: a reappraisal. Neurology 1983, 33(7):932-935.
199. Overington J.P., Al-Lazikani B., Hopkins A.L. How many drug targets are there?. Nat Rev Drug Discov 2006, 5(12):993-996.
200. Pal A., Severin F., Lommer B., Shevchenko A., Zerial M. Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease. J Cell Biol 2006, 172(4):605-618.
201. Palacios J.M., Rigo M., Chinaglia G., Probst A. Reduced density of striatal somatostatin receptors in Huntington's chorea. Brain Res 1990, 522(2):342-346.
202. Palazuelos J., Aguado T., Pazos M.R., Julien B., Carrasco C., Resel E., et al. Microglial CB2 cannabinoid receptors are neuroprotective in Huntington's disease excitotoxicity. Brain 2009, 132(Pt 11):3152-3164.
203. Paleacu D. Tetrabenazine in the treatment of Huntington's disease. Neuropsychiatr Dis Treat 2007, 3(5):545-551.
204. Palfi S., Brouillet E., Jarraya B., Bloch J., Jan C., Shin M., et al. Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates. Mol Ther 2007, 15(8):1444-1451.
205. Panetta R., Greenwood M.T. Physiological relevance of GPCR oligomerization and its impact on drug discovery. Drug Discov Today 2008, 13(23-24):1059-1066.
206. Pang T.Y., Du X., Zajac M.S., Howard M.L., Hannan A.J. Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease. Hum Mol Genet 2009, 18(4):753-766.
207. Panov A.V., Gutekunst C.A., Leavitt B.R., Hayden M.R., Burke J.R., Strittmatter W.J., et al. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci 2002, 5(8):731-736.
208. Parker J.A., Connolly J.B., Wellington C., Hayden M., Dausset J., Neri C. Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc Natl Acad Sci USA 2001, 98(23):13318-13323.
209. Patel S.V., Tariot P.N., Asnis J. l-Deprenyl augmentation of fluoxetine in a patient with Huntington's disease. Ann Clin Psychiatry 1996, 8(1):23-26.
210. Peng Q., Masuda N., Jiang M., Li Q., Zhao M., Ross C.A., et al. The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model. Exp Neurol 2008, 210(1):154-163.
211. Penney J.B., Young A.B., Shoulson I., Starosta-Rubenstein S., Snodgrass S.R., Sanchez-Ramos J., et al. Huntington's disease in Venezuela: 7years of follow-up on symptomatic and asymptomatic individuals. Mov Disord 1990, 5(2):93-99.
212. Picconi B., Passino E., Sgobio C., Bonsi P., Barone I., Ghiglieri V., et al. Plastic and behavioral abnormalities in experimental Huntington's disease: a crucial role for cholinergic interneurons. Neurobiol Dis 2006, 22(1):143-152.
213. Pintor A., Tebano M.T., Martire A., Grieco R., Galluzzo M., Scattoni M.L., et al. The cannabinoid receptor agonist WIN 55,212-2 attenuates the effects induced by quinolinic acid in the rat striatum. Neuropharmacology 2006.
214. Piolti R., Appollonio I., Perego M., Pozzi C., Rovati L., Ferrarese C., et al. Proglumide, a cholecystokinin receptor antagonist, reduces neuroleptic action in Huntington's chorea. Eur Neurol 1995, 35(6):344-348.
215. Pisani A., Bernardi G., Bonsi P., Centonze D., Giacomini P., Calabresi P. Cell-type specificity of mGluR activation in striatal neuronal subtypes. Amino Acids 2000, 19(1):119-129.
216. Pisani A., Bernardi G., Ding J., Surmeier D.J. Re-emergence of striatal cholinergic interneurons in movement disorders. Trends Neurosci 2007, 30(10):545-553.
217. Pisani A., Calabresi P., Centonze D., Bernardi G. Enhancement of NMDA responses by group I metabotropic glutamate receptor activation in striatal neurones. Br J Pharmacol 1997, 120(6):1007-1014.
218. Pollitt S.K., Pallos J., Shao J., Desai U.A., Ma A.A., Thompson L.M., et al. A rapid cellular FRET assay of polyglutamine aggregation identifies a novel inhibitor. Neuron 2003, 40(4):685-694.
219. Popoli P., Betto P., Reggio R., Ricciarello G. Adenosine A2A receptor stimulation enhances striatal extracellular glutamate levels in rats. Eur J Pharmacol 1995, 287(2):215-217.
220. Popoli P., Blum D., Martire A., Ledent C., Ceruti S., Abbracchio M.P. Functions, dysfunctions and possible therapeutic relevance of adenosine A2A receptors in Huntington's disease. Prog Neurobiol 2007, 81(5-6):331-348.
221. Popoli P., Pintor A., Domenici M.R., Frank C., Tebano M.T., Pezzola A., et al. Blockade of striatal adenosine A2A receptor reduces, through a presynaptic mechanism, quinolinic acid-induced excitotoxicity: possible relevance to neuroprotective interventions in neurodegenerative diseases of the striatum. J Neurosci 2002, 22(5):1967-1975.
222. Popoli P., Pintor A., Tebano M.T., Frank C., Pepponi R., Nazzicone V., et al. Neuroprotective effects of the mGlu5R antagonist MPEP towards quinolinic acid-induced striatal toxicity: involvement of pre- and post-synaptic mechanisms and lack of direct NMDA blocking activity. J Neurochem 2004, 89(6):1479-1489.
223. Potenza R.L., Tebano M.T., Martire A., Domenici M.R., Pepponi R., Armida M., et al. Adenosine A(2A) receptors modulate BDNF both in normal conditions and in experimental models of Huntington's disease. Purinergic Signal 2007, 3(4):333-338.
224. Priller J., Ecker D., Landwehrmeyer B., Craufurd D. A Europe-wide assessment of current medication choices in Huntington's disease. Mov Disord 2008, 23(12):1788.
225. Quinn N., Marsden C.D. A double blind trial of sulpiride in Huntington's disease and tardive dyskinesia. J Neurol Neurosurg Psychiatry 1984, 47(8):844-847.
226. Rao D.S., Bradley S.V., Kumar P.D., Hyun T.S., Saint-Dic D., Oravecz-Wilson K., et al. Altered receptor trafficking in Huntingtin Interacting Protein 1-transformed cells. Cancer Cell 2003, 3(5):471-482.
227. Ravikumar B., Vacher C., Berger Z., Davies J.E., Luo S., Oroz L.G., et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet 2004, 36(6):585-595.
228. Reggio R., Pezzola A., Popoli P. The intrastratial injection of an adenosine A(2) receptor antagonist prevents frontal cortex EEG abnormalities in a rat model of Huntington's disease. Brain Res 1999, 831(1-2):315-318.
229. Reggiori F., Monastyrska I., Shintani T., Klionsky D.J. The actin cytoskeleton is required for selective types of autophagy, but not nonspecific autophagy, in the yeast Saccharomyces cerevisiae. Mol Biol Cell 2005, 16(12):5843-5856.
230. Reilly C.E. Wild-type huntingtin up-regulates BDNF transcription in Huntington's disease. J Neurol 2001, 248(10):920-922.
231. Reiner A., Albin R.L., Anderson K.D., D'Amato C.J., Penney J.B., Young A.B. Differential loss of striatal projection neurons in Huntington disease. Proc Natl Acad Sci USA 1988, 85(15):5733-5737.
232. Robinson P., Lebel M., Cyr M. Dopamine D1 receptor-mediated aggregation of N-terminal fragments of mutant huntingtin and cell death in a neuroblastoma cell line. Neuroscience 2008, 153(3):762-772.
233. Rosas H.D., Koroshetz W.J., Jenkins B.G., Chen Y.I., Hayden D.L., Beal M.F., et al. Riluzole therapy in Huntington's disease (HD). Mov Disord 1999, 14(2):326-330.
234. Rosas H.D., Salat D.H., Lee S.Y., Zaleta A.K., Pappu V., Fischl B., et al. Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity. Brain 2008, 131(Pt 4):1057-1068.
235. Ross C.A., Margolis R.L. Huntington's disease. Clin Neurosci Res 2001, 1(1-2):142-152.
236. Roux P.P., Blenis J. ERK and p38 MAPK-activated protein kinases: a family of protein kinases with diverse biological functions. Microbiol Mol Biol Rev 2004, 68(2):320-344.
237. Roze E., Betuing S., Deyts C., Marcon E., Brami-Cherrier K., Pages C., et al. Mitogen- and stress-activated protein kinase-1 deficiency is involved in expanded-huntingtin-induced transcriptional dysregulation and striatal death. FASEB J 2008, 22(4):1083-1093.
238. Ruan Q., Lesort M., MacDonald M.E., Johnson G.V. Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway. Hum Mol Genet 2004, 13(7):669-681.
239. Rubinsztein D.C., Leggo J., Coles R., Almqvist E., Biancalana V., Cassiman J.J., et al. Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats. Am J Hum Genet 1996, 59(1):16-22.
240. Rueda D., Galve-Roperh I., Haro A., Guzman M. The CB1 cannabinoid receptor is coupled to the activation of c-jun N-terminal kinase. Mol Pharmacol 2000, 58(4):814-820.
241. Runne H., Regulier E., Kuhn A., Zala D., Gokce O., Perrin V., et al. Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. J Neurosci 2008, 28(39):9723-9731.
242. Sadri-Vakili G., Bouzou B., Benn C.L., Kim M.-O., Chawla P., Overland R.P., et al. Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models. Hum Mol Genet 2007, 16(11):1293-1306.
243. Sagredo O., Gonzalez S., Aroyo I., Pazos M.R., Benito C., Lastres-Becker I., et al. Cannabinoid CB2 receptor agonists protect the striatum against malonate toxicity: relevance for Huntington's disease. Glia 2009, 57(11):1154-1167.
244. Sagredo O., Ramos J.A., Decio A., Mechoulam R., Fernandez-Ruiz J. Cannabidiol reduced the striatal atrophy caused 3-nitropropionic acid in vivo by mechanisms independent of the activation of cannabinoid, vanilloid TRPV1 and adenosine A2A receptors. Eur J Neurosci 2007, 26(4):843-851.
245. Sanchez I., Mahlke C., Yuan J. Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 2003, 421(6921):373-379.
246. Sapp E., Ge P., Aizawa H., Bird E., Penney J., Young A.B., et al. Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis. Neuroscience 1995, 64(2):397-404.
247. Saudou F., Finkbeiner S., Devys D., Greenberg M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998, 95(1):55-66.
248. Scattoni M.L., Valanzano A., Pezzola A., March Z.D., Fusco F.R., Popoli P., et al. Adenosine A2A receptor blockade before striatal excitotoxic lesions prevents long term behavioural disturbances in the quinolinic rat model of Huntington's disease. Behav Brain Res 2007, 176(2):216-221.
249. Schafer B., Gschwind A., Ullrich A. Multiple G-protein-coupled receptor signals converge on the epidermal growth factor receptor to promote migration and invasion. Oncogene 2004, 23(4):991-999.
250. Schaffhauser H., Mathiasen J.R., Dicamillo A., Huffman M.J., Lu L.D., McKenna B.A., et al. Dimebolin is a 5-HT6 antagonist with acute cognition enhancing activities. Biochem Pharmacol 2009, 78(8):1035-1042.
251. Schiefer J., Landwehrmeyer G.B., Luesse H.G., Sprunken A., Puls C., Milkereit A., et al. Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease. Mov Disord 2002, 17(4):748-757.
252. Schiefer J., Sprunken A., Puls C., Luesse H.G., Milkereit A., Milkereit E., et al. The metabotropic glutamate receptor 5 antagonist MPEP and the mGluR2 agonist LY379268 modify disease progression in a transgenic mouse model of Huntington's disease. Brain Res 2004, 1019(1-2):246-254.
253. Schilling G., Becher M.W., Sharp A.H., Jinnah H.A., Duan K., Kotzuk J.A., et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 1999, 8(3):397-407.
254. Schulte G., Fredholm B.B. Human adenosine A(1), A(2A), A(2B), and A(3) receptors expressed in Chinese hamster ovary cells all mediate the phosphorylation of extracellular-regulated kinase 1/2. Mol Pharmacol 2000, 58(3):477-482.
255. Schwarcz R., Whetsell W.O., Mangano R.M. Quinolinic acid: an endogenous metabolite that produces axon-sparing lesions in rat brain. Science 1983, 219(4582):316-318.
256. Scotter E.L., Abood M.E., Glass M. The endocannabinoid system as a target for the treatment of neurodegenerative disease. Br J Pharmacol 2010, 160(3):480-498.
257. Scotter E.L., Goodfellow C.E., Graham E.S., Dragunow M., Glass M. Neuroprotective potential of CB1 receptor agonists in an in vitro model of Huntington's disease. Br J Pharmacol 2010, 160(3):747-761.
258. Sealfon S.C., Olanow C.W. Dopamine receptors: from structure to behavior. Trends Neurosci 2000, 23(10 Suppl):S34-40.
259. Seppi K., Mueller J., Bodner T., Brandauer E., Benke T., Weirich-Schwaiger H., et al. Riluzole in Huntington's disease (HD): an open label study with one year follow up. J Neurol 2001, 248(10):866-869.
260. Shao J., Welch W.J., Diamond M.I. ROCK and PRK-2 mediate the inhibitory effect of Y-27632 on polyglutamine aggregation. FEBS Lett 2008, 582(12):1637-1642.
261. Shao J., Welch W.J., Diprospero N.A., Diamond M.I. Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation. Mol Cell Biol 2008, 28(17):5196-5208.
262. Shoulson I., Odoroff C., Oakes D., Behr J., Goldblatt D., Caine E., et al. A controlled clinical trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol 1989, 25(3):252-259.
263. Shults C., Steardo L., Barone P., Mohr E., Juncos J., Serrati C., et al. Huntington's disease: effect of cysteamine, a somatostatin-depleting agent. Neurology 1986, 36(8):1099-1102.
264. Sieradzan K.A., Mechan A.O., Jones L., Wanker E.E., Nukina N., Mann D.M. Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein. Exp Neurol 1999, 156(1):92-99.
265. Singhrao S.K., Neal J.W., Morgan B.P., Gasque P. Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease. Exp Neurol 1999, 159(2):362-376.
266. Skirboll L.R., Grace A.A., Bunney B.S. Dopamine auto- and postsynaptic receptors: electrophysiological evidence for differential sensitivity to dopamine agonists. Science 1979, 206(4414):80-82.
267. Smith R., Chung H., Rundquist S., Maat-Schieman M.L., Colgan L., Englund E., et al. Cholinergic neuronal defect without cell loss in Huntington's disease. Hum Mol Genet 2006, 15(21):3119-3131.
268. Soriano A., Ventura R., Molero A., Hoen R., Casado V., Cortes A., et al. Adenosine A2A receptor-antagonist/dopamine D2 receptor-agonist bivalent ligands as pharmacological tools to detect A2A-D2 receptor heteromers. J Med Chem 2009, 52(18):5590-5602.
269. Spektor B.S., Miller D.W., Hollingsworth Z.R., Kaneko Y.A., Solano S.M., Johnson J.M., et al. Differential D1 and D2 receptor-mediated effects on immediate early gene induction in a transgenic mouse model of Huntington's disease. Brain Res Mol Brain Res 2002, 102(1-2):118-128.
270. Spires T.L., Grote H.E., Varshney N.K., Cordery P.M., van Dellen A., Blakemore C., et al. Environmental enrichment rescues protein deficits in a mouse model of Huntington's Disease, indicating a possible disease mechanism. J Neurosci 2004, 24(9):2270-2276.
271. Steffan J.S., Bodai L., Pallos J., Poelman M., McCampbell A., Apostol B.L., et al. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 2001, 413(6857):739-743.
272. Steffan J.S., Kazantsev A., Spasic-Boskovic O., Greenwald M., Zhu Y.-Z., Gohler H., et al. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci USA 2000, 97(12):6763-6768.
273. Steffan J.S., Kazantsev A., Spasic-Boskovic O., Greenwald M., Zhu Y.Z., Gohler H., et al. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci USA 2000, 97(12):6763-6768.
274. Stephan J.S., Yeh Y.-Y., Ramachandran V., Deminoff S.J., Herman P.K. The Tor and PKA signaling pathways independently target the Atg1/Atg13 protein kinase complex to control autophagy. Proceedings of the National Academy of Sciences 2009, 106(40):17049-17054.
275. Steward L.J., Bufton K.E., Hopkins P.C., Davies W.E., Barnes N.M. Reduced levels of 5-HT3 receptor recognition sites in the putamen of patients with Huntington's disease. Eur J Pharmacol 1993, 242(2):137-143.
276. Subramaniam S., Unsicker K. ERK and cell death: ERK1/2 in neuronal death. FEBS J 2010, 277(1):22-29.
277. Sugars K.L., Brown R., Cook L.J., Swartz J., Rubinsztein D.C. Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis. J Biol Chem 2004, 279(6):4988-4999.
278. Suhr S.T., Senut M.C., Whitelegge J.P., Faull K.F., Cuizon D.B., Gage F.H. Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression. J Cell Biol 2001, 153(2):283-294.
279. Suzuki M., Desmond T.J., Albin R.L., Frey K.A. Vesicular neurotransmitter transporters in Huntington's disease: initial observations and comparison with traditional synaptic markers. Synapse 2001, 41(4):329-336.
280. Tagawa K., Hoshino M., Okuda T., Ueda H., Hayashi H., Engemann S., et al. Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein. J Neurochem 2004, 89(4):974-987.
281. Tang T.-S., Chen X., Liu J., Bezprozvanny I. Dopaminergic signaling and striatal neurodegeneration in Huntington's disease. J Neurosci 2007, 27(30):7899-7910.
282. Tang T.-S., Slow E., Lupu V., Stavrovskaya I.G., Sugimori M., Llinas R., et al. Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci USA 2005, 102(7):2602-2607.
283. Tang T.-S., Tu H., Chan E.Y.W., Maximov A., Wang Z., Wellington C.L., et al. Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1, 4, 5) triphosphate receptor type 1. Neuron 2003, 39(2):227-239.
284. Tarsy D., Bralower M. Deanol acetamidobenzoate treatment in choreiform movement disorders. Arch Neurol 1977, 34(12):756-758.
285. Tebano M.T., Pintor A., Frank C., Domenici M.R., Martire A., Pepponi R., et al. Adenosine A2A receptor blockade differentially influences excitotoxic mechanisms at pre- and postsynaptic sites in the rat striatum. J Neurosci Res 2004, 77(1):100-107.
286. Tedroff J., Ekesbo A., Sonesson C., Waters N., Carlsson A. Long-lasting improvement following (-)-OSU6162 in a patient with Huntington's disease. Neurology 1999, 53(7):1605-1606.
287. Testa C.M., Friberg I.K., Weiss S.W., Standaert D.G. Immunohistochemical localization of metabotropic glutamate receptors mGluR1a and mGluR2/3 in the rat basal ganglia. J Comp Neurol 1998, 390(1):5-19.
288. Thu D.C., Oorschot D.E., Tippett L.J., Nana A.L., Hogg V.M., Synek B.J., et al. Cell loss in the motor and cingulate cortex correlates with symptomatology in Huntington's disease. Brain 2010, 133(Pt 4):1094-1110.
289. Tippett L.J., Waldvogel H.J., Thomas S.J., Hogg V.M., van Roon-Mom W., Synek B.J., et al. Striosomes and mood dysfunction in Huntington's disease. Brain 2007, 130(Pt 1):206-221.
290. van Dellen A., Blakemore C., Deacon R., York D., Hannan A.J. Delaying the onset of Huntington's in mice. Nature 2000, 404(6779):721-722.
291. Varani K., Abbracchio M.P., Cannella M., Cislaghi G., Giallonardo P., Mariotti C., et al. Aberrant A2A receptor function in peripheral blood cells in Huntington's disease. FASEB J 2003, 17(14):2148-2150.
292. Varani K., Rigamonti D., Sipione S., Camurri A., Borea P.A., Cattabeni F., et al. Aberrant amplification of A(2A) receptor signaling in striatal cells expressing mutant huntingtin. FASEB J 2001, 15(7):1245-1247.
293. Varma H., Cheng R., Voisine C., Hart A.C., Stockwell B.R. Inhibitors of metabolism rescue cell death in Huntington's disease models. Proc Natl Acad Sci USA 2007, 104(36):14525-14530.
294. Vecsei L., Widerlov E. Preclinical and clinical studies with cysteamine and pantethine related to the central nervous system. Prog Neuropsychopharmacol Biol Psychiatry 1990, 14(6):835-862.
295. Vetter J.M., Jehle T., Heinemeyer J., Franz P., Behrens P.F., Jackisch R., et al. Mice transgenic for exon 1 of Huntington's disease: properties of cholinergic and dopaminergic pre-synaptic function in the striatum. J Neurochem 2003, 85(4):1054-1063.
296. Vitale C., Marconi S., Di Maio L., De Michele G., Longo K., Bonavita V., et al. Short-term continuous infusion of apomorphine hydrochloride for treatment of Huntington's chorea: a double blind, randomized cross-over trial. Mov Disord 2007, 22(16):2359-2364.
297. von Horsten S., Schmitt I., Nguyen H.P., Holzmann C., Schmidt T., Walther T., et al. Transgenic rat model of Huntington's disease. Hum Mol Genet 2003, 12(6):617-624.
298. Vonsattel J.P., Myers R.H., Stevens T.J., Ferrante R.J., Bird E.D., Richardson E.P. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 1985, 44(6):559-577.
299. Waeber C., Palacios J.M. Serotonin-1 receptor binding sites in the human basal ganglia are decreased in Huntington's chorea but not in Parkinson's disease: a quantitative in vitro autoradiography study. Neuroscience 1989, 32(2):337-347.
300. Waeber C., Rigo M., Chinaglia G., Probst A., Palacios J.M. Beta-adrenergic receptor subtypes in the basal ganglia of patients with Huntington's chorea and Parkinson's disease. Synapse 1991, 8(4):270-280.
301. Wexler N.S., Lorimer J., Porter J., Gomez F., Moskowitz C., Shackell E., et al. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci USA 2004, 101(10):3498-3503.
302. Wheeler V.C., White J.K., Gutekunst C.A., Vrbanac V., Weaver M., Li X.J., et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet 2000, 9(4):503-513.
303. Williams A., Sarkar S., Cuddon P., Ttofi E.K., Saiki S., Siddiqi F.H., et al. Novel targets for Huntington's disease in an mTOR-independent autophagy pathway. Nat Chem Biol 2008, 4(5):295-305.
304. Wong E.H., Reynolds G.P., Bonhaus D.W., Hsu S., Eglen R.M. Characterization of [3H]GR 113808 binding to 5-HT4 receptors in brain tissues from patients with neurodegenerative disorders. Behav Brain Res 1996, 73(1-2):249-252.
305. Wong L.F., Goodhead L., Prat C., Mitrophanous K.A., Kingsman S.M., Mazarakis N.D. Lentivirus-mediated gene transfer to the central nervous system: therapeutic and research applications. Hum Gene Ther 2006, 17(1):1-9.
306. Woodruff T.M., Crane J.W., Proctor L.M., Buller K.M., Shek A.B., de Vos K., et al. Therapeutic activity of C5a receptor antagonists in a rat model of neurodegeneration. FASEB J 2006, 20(9):1407-1417.
307. Wu J., Li Q., Bezprozvanny I. Evaluation of Dimebon in cellular model of Huntington's disease. Mol Neurodegener 2008, 3:15.
308. Wyttenbach A., Swartz J., Kita H., Thykjaer T., Carmichael J., Bradley J., et al. Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease. Hum Mol Genet 2001, 10(17):1829-1845.
309. Yang S.H., Cheng P.H., Banta H., Piotrowska-Nitsche K., Yang J.J., Cheng E.C., et al. Towards a transgenic model of Huntington's disease in a non-human primate. Nature 2008, 453(7197):921-924.
310. Yang W., Dunlap J.R., Andrews R.B., Wetzel R. Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells. Hum Mol Genet 2002, 11(23):2905-2917.
311. Yanpallewar S.U., Fernandes K., Marathe S.V., Vadodaria K.C., Jhaveri D., Rommelfanger K., et al. Alpha2-adrenoceptor blockade accelerates the neurogenic, neurotrophic, and behavioral effects of chronic antidepressant treatment. J Neurosci 2010, 30(3):1096-1109.
312. Zhai W., Jeong H., Cui L., Krainc D., Tjian R. In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Cell 2005, 123(7):1241-1253.
313. Zhang W., Liu H.T. MAPK signal pathways in the regulation of cell proliferation in mammalian cells. Cell Res 2002, 12(1):9-18.
314. Zoghbi H.Y., Orr H.T. Glutamine repeats and neurodegeneration. Annu Rev Neurosci 2000, 23:217-247.
315. Zuccato C., Cattaneo E. Brain-derived neurotrophic factor in neurodegenerative diseases. Nat Rev Neurol 2009, 5(6):311-322.
316. Zuccato C., Ciammola A., Rigamonti D., Leavitt B.R., Goffredo D., Conti L., et al. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 2001, 293(5529):493-498.
317. Zuccato C., Marullo M., Conforti P., MacDonald M.E., Tartari M., Cattaneo E. Systematic assessment of BDNF and its receptor levels in human cortices affected by Huntington's disease. Brain Pathol 2008, 18(2):225-238.