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Volumn 15, Issue 3, 2014, Pages 313-334

Current therapeutic advances in patients and experimental models of Huntington's disease

Author keywords

Huntington disease; Mutant huntingtin; Neuroprotective therapy; Symptomatic treatment

Indexed keywords

ATOMOXETINE; BRAIN DERIVED NEUROTROPHIC FACTOR; CEP 1347; CILIARY NEUROTROPHIC FACTOR; CITALOPRAM; CLONIDINE; FLUPHENAZINE; GENE THERAPY AGENT; GLIAL CELL LINE DERIVED NEUROTROPHIC FACTOR; HALOPERIDOL; ICOSAPENTAENOIC ACID ETHYL ESTER; LEVODOPA; MERCAPTAMINE; METHAZOLAMIDE; MINOCYCLINE; MINOXIDIL; PIMOZIDE; PRAMIPEXOLE; PRIDOPIDINE; RAPAMYCIN; RESERPINE; SHORT HAIRPIN RNA; SMALL INTERFERING RNA; SOMATOMEDIN C; SULPIRIDE; TETRABENAZINE; THIORIDAZINE; TIAPRIDE; TREHALOSE; UBIDECARENONE; UNINDEXED DRUG; VERAPAMIL;

EID: 84896718561     PISSN: 13894501     EISSN: 18735592     Source Type: Journal    
DOI: 10.2174/1389450114666131124140704     Document Type: Review
Times cited : (10)

References (192)
  • 2
    • 0027480960 scopus 로고
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
    • The Huntington's Disease Collaborative Research Group
    • The 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): 971-83.
    • (1993) Cell , vol.72 , Issue.6 , pp. 971-983
  • 3
    • 45149107487 scopus 로고    scopus 로고
    • Mechanisms of neurodegeneration in Huntington's disease
    • Gil JM, Rego AC. Mechanisms of neurodegeneration in Huntington's disease. Eur J Neurosci 2008; 7: 2803-20.
    • (2008) Eur J Neurosci , vol.7 , pp. 2803-2820
    • Gil, J.M.1    Rego, A.C.2
  • 4
    • 77957993152 scopus 로고    scopus 로고
    • Mitochondrial-associated metabolic changes and neurodegeneration in Huntington's disease-from clinical features to the bench
    • Rosenstock TR, Duarte AI, Rego AC. Mitochondrial-associated metabolic changes and neurodegeneration in Huntington's disease-from clinical features to the bench. Curr Drug Targets 2010; 11(10): 1218-36.
    • (2010) Curr Drug Targets , vol.11 , Issue.10 , pp. 1218-1236
    • Rosenstock, T.R.1    Duarte, A.I.2    Rego, A.C.3
  • 5
    • 0038476184 scopus 로고    scopus 로고
    • Evidence for more widespread cerebral pathology in early HD: An MRI-based morphometric analysis
    • Rosas HD, Koroshetz WJ, Chen YI, et al. Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis. Neurology 2003; 60(10): 1615-20.
    • (2003) Neurology , vol.60 , Issue.10 , pp. 1615-1620
    • Rosas, H.D.1    Koroshetz, W.J.2    Chen, Y.I.3
  • 6
    • 78651085932 scopus 로고    scopus 로고
    • Changes in key hypothalamic neuropeptide populations in Huntington disease revealed by neuropathologicalanalyses
    • Gabery S, Murphy K, Schultz K, et al. Changes in key hypothalamic neuropeptide populations in Huntington disease revealed by neuropathologicalanalyses. Acta Neuropathol 2010; 120(6): 777-88
    • (2010) Acta Neuropathol , vol.120 , Issue.6 , pp. 777-788
    • Gabery, S.1    Murphy, K.2    Schultz, K.3
  • 7
    • 0027276977 scopus 로고
    • Neuronal loss in the hippocampus in Huntington's disease: A comparison with HIV infection
    • Spargo E, Everall IP, Lantos PL. Neuronal loss in the hippocampus in Huntington's disease: a comparison with HIV infection. J Neurol Neurosurg Psychiatry 1993; 56(5): 487-91.
    • (1993) J Neurol Neurosurg Psychiatry , vol.56 , Issue.5 , pp. 487-491
    • Spargo, E.1    Everall, I.P.2    Lantos, P.L.3
  • 10
    • 31544446034 scopus 로고    scopus 로고
    • Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation
    • Squitieri F, Cannella M, Sgarbi G, et al. Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation. Mech Ageing Dev 2006; 127: 217-20.
    • (2006) Mech Ageing Dev , vol.127 , pp. 217-220
    • Squitieri, F.1    Cannella, M.2    Sgarbi, G.3
  • 11
    • 0027078587 scopus 로고
    • Causes of death in patients with Huntington's disease and in unaffected first degree relatives
    • Sørensen SA, Fenger K. Causes of death in patients with Huntington's disease and in unaffected first degree relatives. J Med Genet 1992; 29(12): 911-4.
    • (1992) J Med Genet , vol.29 , Issue.12 , pp. 911-914
    • Sørensen, S.A.1    Fenger, K.2
  • 14
    • 0020954957 scopus 로고
    • The association of affective disorder with Huntington's disease in a case series and in families
    • Folstein S, Abbott MH, Chase GA, Jensen BA, Folstein MF. The association of affective disorder with Huntington's disease in a case series and in families. Psychol Med 1983; 13: 537-42.
    • (1983) Psychol Med , vol.13 , pp. 537-542
    • Folstein, S.1    Abbott, M.H.2    Chase, G.A.3    Jensen, B.A.4    Folstein, M.F.5
  • 15
    • 0025290717 scopus 로고
    • Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals
    • Penney JB Jr, Young AB, Shoulson I, et al. Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals. Mov Disord 1990; 5(2): 93-9.
    • (1990) Mov Disord , vol.5 , Issue.2 , pp. 93-99
    • Penney Jr., J.B.1    Young, A.B.2    Shoulson, I.3
  • 16
    • 0023880409 scopus 로고    scopus 로고
    • The coexistence of bradykinesia and chorea in Huntington's disease and its implications for theories of basal ganglia control of movement
    • Thompson PD, Berardelli A, Rothwell JC, et al. The coexistence of bradykinesia and chorea in Huntington's disease and its implications for theories of basal ganglia control of movement. Brain 1998; 111: 223-44.
    • (1998) Brain , vol.111 , pp. 223-244
    • Thompson, P.D.1    Berardelli, A.2    Rothwell, J.C.3
  • 18
    • 77955446151 scopus 로고    scopus 로고
    • Mild cognitive impairment in prediagnosed Huntington disease
    • Duff K, Paulsen J, Mills J, et al. Mild cognitive impairment in prediagnosed Huntington disease. Neurology 2010; 75(6): 500-7.
    • (2010) Neurology , vol.75 , Issue.6 , pp. 500-507
    • Duff, K.1    Paulsen, J.2    Mills, J.3
  • 19
    • 23944461062 scopus 로고    scopus 로고
    • Use of oral nutritional supplements in patients with Huntington's disease
    • Trejo A, Boll MC, Alonso ME, Ochoa A, Velásquez L. Use of oral nutritional supplements in patients with Huntington's disease. Nutrition 2005; 21: 889-94.
    • (2005) Nutrition , vol.21 , pp. 889-894
    • Trejo, A.1    Boll, M.C.2    Alonso, M.E.3    Ochoa, A.4    Velásquez, L.5
  • 20
    • 51249102726 scopus 로고    scopus 로고
    • Hypocretin and melanin-concentrating hormone in patients with Huntington disease
    • Aziz A, Fronczek R, Maat-Schieman M, et al. Hypocretin and melanin-concentrating hormone in patients with Huntington disease. Brain Pathol 2008; 18: 474-83.
    • (2008) Brain Pathol , vol.18 , pp. 474-483
    • Aziz, A.1    Fronczek, R.2    Maat-Schieman, M.3
  • 21
    • 77952379669 scopus 로고    scopus 로고
    • Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression
    • Pouladi MA, Xie Y, Skotte NH, et al. Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet 2010; 19: 1528-38.
    • (2010) Hum Mol Genet , vol.19 , pp. 1528-1538
    • Pouladi, M.A.1    Xie, Y.2    Skotte, N.H.3
  • 22
    • 77954650864 scopus 로고    scopus 로고
    • High insulinlike growth factor I is associated with cognitive decline in Huntington disease. Huntington French Speaking Group
    • Saleh N, Moutereau S, Azulay JP, et al. High insulinlike growth factor I is associated with cognitive decline in Huntington disease. Huntington French Speaking Group. Neurology 2010; 75(1): 57-63.
    • (2010) Neurology , vol.75 , Issue.1 , pp. 57-63
    • Saleh, N.1    Moutereau, S.2    Azulay, J.P.3
  • 23
    • 36248935287 scopus 로고    scopus 로고
    • Early energy deficit in Huntington disease: Identification of a plasma biomarker traceable during disease progression
    • Mochel F, Charles P, Seguin F, et al. Early energy deficit in Huntington disease: identification of a plasma biomarker traceable during disease progression. PLoS One 2007; 2: e647.
    • (2007) PLoS One , vol.2
    • Mochel, F.1    Charles, P.2    Seguin, F.3
  • 24
    • 85009332113 scopus 로고    scopus 로고
    • The Unified Huntington's Disease Rating Scale: Reliability and Consistency
    • Huntington Study Group
    • Huntington Study Group. The Unified Huntington's Disease Rating Scale: Reliability and Consistency. Mov Dis 1996; 11: 136-42
    • (1996) Mov Dis , vol.11 , pp. 136-142
  • 25
    • 0003412410 scopus 로고
    • In: Washington DC: U.S. Department of Health, Education and Welfare
    • Guy W. ECDEU Assessment Manual for Psychopharmacology. In: Washington DC: U.S. Department of Health, Education and Welfare, 1976: 534-7
    • (1976) ECDEU Assessment Manual for Psychopharmacology. , pp. 534-537
    • Guy, W.1
  • 26
    • 0031780369 scopus 로고    scopus 로고
    • Awareness of involuntary movements in Huntington disease
    • Snowden JS, Craufurd D, Griffiths HL, Neary D. Awareness of involuntary movements in Huntington disease. Arch Neurol 1998; 55(6): 801-5.
    • (1998) Arch Neurol , vol.55 , Issue.6 , pp. 801-805
    • Snowden, J.S.1    Craufurd, D.2    Griffiths, H.L.3    Neary, D.4
  • 28
    • 79951974420 scopus 로고    scopus 로고
    • Tetrabenazine: The first approved drug for the treatment of chorea in US patients with Huntington disease
    • Frank S. Tetrabenazine: the first approved drug for the treatment of chorea in US patients with Huntington disease. Neuropsychiatr Dis Treat 2010; 6: 657-65.
    • (2010) Neuropsychiatr Dis Treat , vol.6 , pp. 657-665
    • Frank, S.1
  • 29
    • 33645872123 scopus 로고    scopus 로고
    • Tetrabenazine in the treatment of hyperkinetic movement disorders
    • Kenney C, Jankovic J. Tetrabenazine in the treatment of hyperkinetic movement disorders. Expert Rev Neurother 2006; 6: 7-17.
    • (2006) Expert Rev Neurother , vol.6 , pp. 7-17
    • Kenney, C.1    Jankovic, J.2
  • 30
    • 33645798913 scopus 로고    scopus 로고
    • Tetrabenazine as antichorea therapy in Huntington disease: A randomized controlled trial
    • Huntington Study Group
    • Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology 2006; 66: 366-72.
    • (2006) Neurology , vol.66 , pp. 366-372
  • 31
    • 74549128476 scopus 로고    scopus 로고
    • Tetrabenazine as anti-chorea therapy in Huntington disease: An open-label continuation study. Huntington Study Group/TETRA-HD Investigators
    • Frank S. Tetrabenazine as anti-chorea therapy in Huntington disease: an open-label continuation study. Huntington Study Group/TETRA-HD Investigators. BMC Neurol 2009; 9: 62.
    • (2009) BMC Neurol , vol.9 , pp. 62
    • Frank, S.1
  • 32
    • 81255195330 scopus 로고    scopus 로고
    • Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): A phase 3, randomised, double-blind, placebo-controlled trial
    • de Yebenes JG, Landwehrmeyer B, Squitieri F, et al. Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2011; 10(12): 1049-57.
    • (2011) Lancet Neurol , vol.10 , Issue.12 , pp. 1049-1057
    • de Yebenes, J.G.1    Landwehrmeyer, B.2    Squitieri, F.3
  • 33
    • 47349132531 scopus 로고    scopus 로고
    • Juvenile Huntington disease exacerbated by methylphenidate: Case report
    • Waugh JL, Miller VS, Chudnow RS, Dowling MM. Juvenile Huntington disease exacerbated by methylphenidate: case report. J Child Neurol 2008; 23: 807-9.
    • (2008) J Child Neurol , vol.23 , pp. 807-809
    • Waugh, J.L.1    Miller, V.S.2    Chudnow, R.S.3    Dowling, M.M.4
  • 34
    • 41249096422 scopus 로고    scopus 로고
    • Symptomatic treatment of Huntington disease
    • Adam OR, Jankovic J. Symptomatic treatment of Huntington disease. Neurotherapeutics 2008; 5: 181-97.
    • (2008) Neurotherapeutics , vol.5 , pp. 181-197
    • Adam, O.R.1    Jankovic, J.2
  • 35
    • 0023868584 scopus 로고
    • Serum haloperidol concentration and choreiform movements in Huntington's disease
    • Barr AN, Fischer JH, Koller WC, Spunt AL, Singhal A. Serum haloperidol concentration and choreiform movements in Huntington's disease. Neurology 1988; 38: 84-8.
    • (1988) Neurology , vol.38 , pp. 84-88
    • Barr, A.N.1    Fischer, J.H.2    Koller, W.C.3    Spunt, A.L.4    Singhal, A.5
  • 36
    • 0021175970 scopus 로고
    • A double blind trial of sulpiride in Huntington's disease and tardive dyskinesia
    • Quinn N, Marsden CD. A double blind trial of sulpiride in Huntington's disease and tardive dyskinesia. J Neurol Neurosurg Psychiatry 1984; 47: 844-7.
    • (1984) J Neurol Neurosurg Psychiatry , vol.47 , pp. 844-847
    • Quinn, N.1    Marsden, C.D.2
  • 37
    • 0021733679 scopus 로고
    • Tiapride versus placebo: A double-blind comparative study in the management of Huntington's chorea
    • Deroover J, Baro F, Bourguignon RP, Smets P. Tiapride versus placebo: a double-blind comparative study in the management of Huntington's chorea. Curr Med Res Opin 1984; 9: 329-38.
    • (1984) Curr Med Res Opin , vol.9 , pp. 329-338
    • Deroover, J.1    Baro, F.2    Bourguignon, R.P.3    Smets, P.4
  • 39
    • 33745759912 scopus 로고    scopus 로고
    • Pharmacological management of Huntington's disease: An evidence-based review
    • Bonelli RM, Wenning GK. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des 2006; 12: 2701-20.
    • (2006) Curr Pharm Des , vol.12 , pp. 2701-2720
    • Bonelli, R.M.1    Wenning, G.K.2
  • 40
    • 0028147689 scopus 로고
    • MPP(+)-like neurotoxicity of a pyridinium metabolite derived from haloperidol: In vivo microdialysis and In vitro mitochondrial studies
    • Rollema H, Skolnik M, D'Engelbronner J, Igarashi K, Usuki E, Castagnoli NJr. MPP(+)-like neurotoxicity of a pyridinium metabolite derived from haloperidol: In vivo microdialysis and In vitro mitochondrial studies. J Pharmacol Exp Ther 1994; 268(1): 380-7.
    • (1994) J Pharmacol Exp Ther , vol.268 , Issue.1 , pp. 380-387
    • Rollema, H.1    Skolnik, M.2    D'Engelbronner, J.3    Igarashi, K.4    Usuki, E.5    Castagnoli Jr., N.6
  • 41
    • 0035741972 scopus 로고    scopus 로고
    • Analysis of the metabolism of haloperidol and its neurotoxic pyridinium metabolite in patients with drug-induced parkinsonism
    • Iwahashi K, Anemo K, Nakamura K, Fukunishi I, Igarashi K. Analysis of the metabolism of haloperidol and its neurotoxic pyridinium metabolite in patients with drug-induced parkinsonism. Neuropsychobiology 2001; 44(3): 126-8.
    • (2001) Neuropsychobiology , vol.44 , Issue.3 , pp. 126-128
    • Iwahashi, K.1    Anemo, K.2    Nakamura, K.3    Fukunishi, I.4    Igarashi, K.5
  • 42
    • 21244487457 scopus 로고    scopus 로고
    • Modern antipsychotic drugs: A critical overview
    • Gardner DM, Baldessarini RJ, Waraich P. Modern antipsychotic drugs: a critical overview. CMAJ 2005; 172: 1703-11.
    • (2005) CMAJ , vol.172 , pp. 1703-1711
    • Gardner, D.M.1    Baldessarini, R.J.2    Waraich, P.3
  • 44
    • 0036709253 scopus 로고    scopus 로고
    • Olanzapine for Huntington's disease: An open label study
    • Bonelli RM, Mahnert FA, Niederwieser G. Olanzapine for Huntington's disease: an open label study. Clin Neuropharmacol 2002; 25(5): 263-65.
    • (2002) Clin Neuropharmacol , vol.25 , Issue.5 , pp. 263-265
    • Bonelli, R.M.1    Mahnert, F.A.2    Niederwieser, G.3
  • 45
    • 1842854595 scopus 로고    scopus 로고
    • A review of the treatment options for Huntington's disease
    • Bonelli RM, Hofmann P. A review of the treatment options for Huntington's disease. Expert Opin Pharmacother 2004; 5: 767-76.
    • (2004) Expert Opin Pharmacother , vol.5 , pp. 767-776
    • Bonelli, R.M.1    Hofmann, P.2
  • 48
    • 29544445190 scopus 로고    scopus 로고
    • Quetiapine in the treatment of behavioral disturbances in patients with Huntington's disease
    • Alpay M, Koroshetz WJ. Quetiapine in the treatment of behavioral disturbances in patients with Huntington's disease. Psychosomatics 2006; 47: 70-2.
    • (2006) Psychosomatics , vol.47 , pp. 70-72
    • Alpay, M.1    Koroshetz, W.J.2
  • 49
    • 0037056392 scopus 로고    scopus 로고
    • Huntington's disease: A randomized, controlled trial using the NMDA-antagonist amantadine
    • Verhagen ML, Morris MJ, Farmer C, et al. Huntington's disease: a randomized, controlled trial using the NMDA-antagonist amantadine. Neurology 2002; 59: 694-9.
    • (2002) Neurology , vol.59 , pp. 694-699
    • Verhagen, M.L.1    Morris, M.J.2    Farmer, C.3
  • 50
    • 0345600893 scopus 로고    scopus 로고
    • Dosage effects of riluzole in Huntington's disease: A multicenter placebo-controlled study
    • Huntington Study Group
    • Huntington Study Group. Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 2003; 61: 1551-6.
    • (2003) Neurology , vol.61 , pp. 1551-1556
  • 51
    • 34948838383 scopus 로고    scopus 로고
    • Riluzole in Huntington's disease: A 3-year, randomized controlled study
    • Landwehrmeyer GB, Dubois B, de Yébenes JG. Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol 2007; 62: 262-72.
    • (2007) Ann Neurol , vol.62 , pp. 262-272
    • Landwehrmeyer, G.B.1    Dubois, B.2    de Yébenes, J.G.3
  • 52
    • 61449148438 scopus 로고    scopus 로고
    • Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease
    • Joshi PR, Wu NP, André VM, et al. Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J Neurosci 2009; 29: 2414-27.
    • (2009) J Neurosci , vol.29 , pp. 2414-2427
    • Joshi, P.R.1    Wu, N.P.2    André, V.M.3
  • 53
    • 35248894933 scopus 로고    scopus 로고
    • The mechanisms of action of valproate in neuropsychiatric disorders: Can we see the forest for the trees?
    • Rosenberg G. The mechanisms of action of valproate in neuropsychiatric disorders: can we see the forest for the trees? Cell Mol Life Sci 2007; 64: 2090-103.
    • (2007) Cell Mol Life Sci , vol.64 , pp. 2090-2103
    • Rosenberg, G.1
  • 54
    • 33645216173 scopus 로고    scopus 로고
    • Dosedependent improvement of myoclonic hyperkinesia due to valproic acid in eight Huntington's disease patients: A case series
    • Saft C, Lauter T, Kraus PH, Przuntek H, Andrich J. Dosedependent improvement of myoclonic hyperkinesia due to valproic acid in eight Huntington's disease patients: a case series. BMC Neurol 2006; 6: 11.
    • (2006) BMC Neurol , vol.6 , pp. 11
    • Saft, C.1    Lauter, T.2    Kraus, P.H.3    Przuntek, H.4    Andrich, J.5
  • 55
    • 70349655241 scopus 로고    scopus 로고
    • Randomized controlled trial of atomoxetine for cognitive dysfunction in early Huntington disease
    • Beglinger LJ, Adams WH, Paulson H, et al. Randomized controlled trial of atomoxetine for cognitive dysfunction in early Huntington disease. J Clin Psychopharmacol 2009; 29: 484-7.
    • (2009) J Clin Psychopharmacol , vol.29 , pp. 484-487
    • Beglinger, L.J.1    Adams, W.H.2    Paulson, H.3
  • 56
    • 33846809051 scopus 로고    scopus 로고
    • A systematic review of the treatment studies in Huntington's disease since 1990
    • Bonelli RM, Hofmann P. A systematic review of the treatment studies in Huntington's disease since 1990. Expert Opin Pharmacother 2007; 8: 141-53.
    • (2007) Expert Opin Pharmacother , vol.8 , pp. 141-153
    • Bonelli, R.M.1    Hofmann, P.2
  • 59
    • 16044373842 scopus 로고    scopus 로고
    • Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
    • Mangiarini L, Sathasivam K, Seller M, 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: 493-506.
    • (1996) Cell , vol.87 , pp. 493-506
    • Mangiarini, L.1    Sathasivam, K.2    Seller, M.3
  • 60
    • 10744227174 scopus 로고    scopus 로고
    • Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
    • Slow EJ, van Raamsdonk J, Rogers D, et al. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 2003; 12: 1555-67.
    • (2003) Hum Mol Genet , vol.12 , pp. 1555-1567
    • Slow, E.J.1    van Raamsdonk, J.2    Rogers, D.3
  • 61
    • 65249132310 scopus 로고    scopus 로고
    • Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin
    • Pouladi MA, Graham RK, Karasinska JM, Xie Y, Santos RD, Petersén A, Hayden MR. Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin. Brain 2009; 132(Pt 4): 919-32.
    • (2009) Brain , vol.132 , Issue.PART 4 , pp. 919-932
    • Pouladi, M.A.1    Graham, R.K.2    Karasinska, J.M.3    Xie, Y.4    Santos, R.D.5    Petersén, A.6    Hayden, M.R.7
  • 63
    • 58949085531 scopus 로고    scopus 로고
    • Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease
    • Pang TY, Du X, Zajac MS, Howard ML, Hannan AJ. 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):753-66.
    • (2009) Hum Mol Genet , vol.18 , Issue.4 , pp. 753-766
    • Pang, T.Y.1    Du, X.2    Zajac, M.S.3    Howard, M.L.4    Hannan, A.J.5
  • 64
    • 43649106471 scopus 로고    scopus 로고
    • Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's Disease
    • Duan W, Peng Q, Masuda N, et al. Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's Disease. Neurobiol Dis 2008; 30: 312-22.
    • (2008) Neurobiol Dis , vol.30 , pp. 312-322
    • Duan, W.1    Peng, Q.2    Masuda, N.3
  • 65
    • 0033054555 scopus 로고    scopus 로고
    • Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant Nterminal fragment of huntingtin
    • Schilling G, Becher MW, Sharp AH, et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant Nterminal fragment of huntingtin. Hum Mol Genet 1999; 8: 397-407.
    • (1999) Hum Mol Genet , vol.8 , pp. 397-407
    • Schilling, G.1    Becher, M.W.2    Sharp, A.H.3
  • 66
    • 3843077481 scopus 로고    scopus 로고
    • Bilateral globus pallidus stimulation for Huntington's disease
    • Moro E, Lang AE, Strafella AP, et al. Bilateral globus pallidus stimulation for Huntington's disease. Ann Neurol 2004; 56(2): 290-4.
    • (2004) Ann Neurol , vol.56 , Issue.2 , pp. 290-294
    • Moro, E.1    Lang, A.E.2    Strafella, A.P.3
  • 67
    • 18144380962 scopus 로고    scopus 로고
    • Pallidal deep brain stimulation influences both reflexive and voluntary saccades in Huntington's disease
    • Fawcett AP, Moro E, Lang AE, Lozano AM, Hutchison WD. Pallidal deep brain stimulation influences both reflexive and voluntary saccades in Huntington's disease. Mov Disord 2005; 20(3): 371-7.
    • (2005) Mov Disord , vol.20 , Issue.3 , pp. 371-377
    • Fawcett, A.P.1    Moro, E.2    Lang, A.E.3    Lozano, A.M.4    Hutchison, W.D.5
  • 68
    • 32244442568 scopus 로고    scopus 로고
    • Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: Technical case report
    • Hebb MO, Garcia R, Gaudet P, Mendez IM. Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: technical case report. Neurosurgery 2006; 58(2): E383.
    • (2006) Neurosurgery , vol.58 , Issue.2
    • Hebb, M.O.1    Garcia, R.2    Gaudet, P.3    Mendez, I.M.4
  • 69
    • 33747760663 scopus 로고    scopus 로고
    • Motor and cognitive improvement by deep brain stimulation in a transgenic rat model of Huntington's disease
    • Temel Y, Cao C, Vlamings R, et al. Motor and cognitive improvement by deep brain stimulation in a transgenic rat model of Huntington's disease. Neurosci Lett 2006; 406(1-2): 138-41.
    • (2006) Neurosci Lett , vol.406 , Issue.1-2 , pp. 138-141
    • Temel, Y.1    Cao, C.2    Vlamings, R.3
  • 70
    • 0035909330 scopus 로고    scopus 로고
    • Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
    • Steffan JS, Bodai L, Pallos J, et al. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 2001; 413: 739-43.
    • (2001) Nature , vol.413 , pp. 739-743
    • Steffan, J.S.1    Bodai, L.2    Pallos, J.3
  • 71
    • 0037452775 scopus 로고    scopus 로고
    • Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
    • Hockly E, Richon VM, Woodman B, 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: 2041-6.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 2041-2046
    • Hockly, E.1    Richon, V.M.2    Woodman, B.3
  • 72
    • 82155182012 scopus 로고    scopus 로고
    • SAHA decreases HDAC 2 and 4 levels In vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington'sdisease
    • Mielcarek M, Benn CL, Franklin SA, et al. SAHA decreases HDAC 2 and 4 levels In vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington'sdisease. PLoS One 2011; 6(11): e27746.
    • (2011) PLoS One , vol.6 , Issue.11
    • Mielcarek, M.1    Benn, C.L.2    Franklin, S.A.3
  • 73
    • 0142157600 scopus 로고    scopus 로고
    • Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice
    • Ferrante RJ, Kubilus JK, Lee J, et al. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. J Neurosci 2003; 23: 9418-27.
    • (2003) J Neurosci , vol.23 , pp. 9418-9427
    • Ferrante, R.J.1    Kubilus, J.K.2    Lee, J.3
  • 74
    • 0037386621 scopus 로고    scopus 로고
    • Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway
    • Ryu H, Lee J, Olofsson BA, et al. Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway. Proc Natl Acad Sci USA 2003; 100: 4281-6.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 4281-4286
    • Ryu, H.1    Lee, J.2    Olofsson, B.A.3
  • 75
    • 33751106144 scopus 로고    scopus 로고
    • Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: Effect of histone deacetylase inhibitors
    • Oliveira JM, Chen S, Almeida S, et al. Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. J Neurosci 2006; 26: 11174-86.
    • (2006) J Neurosci , vol.26 , pp. 11174-11186
    • Oliveira, J.M.1    Chen, S.2    Almeida, S.3
  • 76
    • 19944431703 scopus 로고    scopus 로고
    • Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease
    • Gardian G, Browne SE, Choi DK, et al. Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease. J Biol Chem 2005; 280: 556-63.
    • (2005) J Biol Chem , vol.280 , pp. 556-563
    • Gardian, G.1    Browne, S.E.2    Choi, D.K.3
  • 77
    • 55749103407 scopus 로고    scopus 로고
    • The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice
    • Thomas EA, Coppola G, Desplats PA, et al. The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice. Proc Natl Acad Sci USA 2008; 105: 15564-9.
    • (2008) Proc Natl Acad Sci USA , vol.105 , pp. 15564-15569
    • Thomas, E.A.1    Coppola, G.2    Desplats, P.A.3
  • 78
    • 34047175919 scopus 로고    scopus 로고
    • Histone deacetylase 6 inhibition compensates for the transport deficit in Huntington's disease by increasing tubulin acetylation
    • Dompierre JP, Godin JD, Charrin BC, et al. Histone deacetylase 6 inhibition compensates for the transport deficit in Huntington's disease by increasing tubulin acetylation. J Neurosci 2007; 27: 3571-83.
    • (2007) J Neurosci , vol.27 , pp. 3571-3583
    • Dompierre, J.P.1    Godin, J.D.2    Charrin, B.C.3
  • 79
    • 36248970599 scopus 로고    scopus 로고
    • Sodium phenylbutyrate in Huntington's disease: A dose-finding study
    • Hogarth P, Lovrecic L, Krainc D. Sodium phenylbutyrate in Huntington's disease: a dose-finding study. Mov Disord 2007; 22: 1962-4.
    • (2007) Mov Disord , vol.22 , pp. 1962-1964
    • Hogarth, P.1    Lovrecic, L.2    Krainc, D.3
  • 80
    • 42949125477 scopus 로고    scopus 로고
    • SIRT1 and neuronal diseases
    • Tang BL, Chua CE. SIRT1 and neuronal diseases. Mol Aspects Med 2008; 29: 187-200.
    • (2008) Mol Aspects Med , vol.29 , pp. 187-200
    • Tang, B.L.1    Chua, C.E.2
  • 81
    • 33750437278 scopus 로고    scopus 로고
    • Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1α in Huntington's disease neurodegeneration
    • Weydt P, Pineda VV, Torrence AE, et al. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1α in Huntington's disease neurodegeneration. Cell Metab 2006; 4: 349-62.
    • (2006) Cell Metab , vol.4 , pp. 349-362
    • Weydt, P.1    Pineda, V.V.2    Torrence, A.E.3
  • 82
    • 33749042331 scopus 로고    scopus 로고
    • Transcriptional repression of PGC-1TTTby mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
    • Cui L, Jeong H, Borovecki F, Parkhurst CN, Tanese N, Krainc D. Transcriptional repression of PGC-1TTTby mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006; 127: 59-69.
    • (2006) Cell , vol.127 , pp. 59-69
    • Cui, L.1    Jeong, H.2    Borovecki, F.3    Parkhurst, C.N.4    Tanese, N.5    Krainc, D.6
  • 83
    • 16844375290 scopus 로고    scopus 로고
    • Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons
    • Parker JA, Arango M, Abderrahmane S, et al. Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons. Nat Genet 2005; 37: 349-50.
    • (2005) Nat Genet , vol.37 , pp. 349-350
    • Parker, J.A.1    Arango, M.2    Abderrahmane, S.3
  • 84
    • 84872672238 scopus 로고    scopus 로고
    • Resveratrol potently reduces prostaglandin E2 production and free radical formation in lipopolysaccharide-activated primary rat microglia
    • Candelario-Jalil E, de Oliveira AC, Gräf S, et al. Resveratrol potently reduces prostaglandin E2 production and free radical formation in lipopolysaccharide-activated primary rat microglia. J Neuroinflammation 2007; 10: 4-25.
    • (2007) J Neuroinflammation , vol.10 , pp. 4-25
    • Candelario-Jalil, E.1    de Oliveira, A.C.2    Gräf, S.3
  • 85
    • 78851468921 scopus 로고    scopus 로고
    • Neuroprotective properties of resveratrol and derivatives
    • Richard T, Pawlus AD, Iglésias ML, et al. Neuroprotective properties of resveratrol and derivatives. Ann NY Acad Sci 2011; 1215: 103-8.
    • (2011) Ann NY Acad Sci , vol.1215 , pp. 103-108
    • Richard, T.1    Pawlus, A.D.2    Iglésias, M.L.3
  • 86
    • 0035919661 scopus 로고    scopus 로고
    • Resveratrol reduction of infarct size in Long-Evans rats subjected to focal cerebral ischemia
    • Huang SS, Tsai MC, Chih CL, Hung LM, Tsai SK. Resveratrol reduction of infarct size in Long-Evans rats subjected to focal cerebral ischemia. Life Sci 2001; 69: 1057-65.
    • (2001) Life Sci , vol.69 , pp. 1057-1065
    • Huang, S.S.1    Tsai, M.C.2    Chih, C.L.3    Hung, L.M.4    Tsai, S.K.5
  • 88
    • 77952413052 scopus 로고    scopus 로고
    • SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis
    • Luthi-Carter R, Taylor DM, Pallos J, et al. SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc Natl Acad Sci USA 2010; 107: 7927-32.
    • (2010) Proc Natl Acad Sci USA , vol.107 , pp. 7927-7932
    • Luthi-Carter, R.1    Taylor, D.M.2    Pallos, J.3
  • 89
    • 0041353535 scopus 로고    scopus 로고
    • Huntingtin interacts with REST NRSF to modulate the transcription of NRSE-controlled neuronal genes
    • Zuccato C, Tartari M, Crotti A, et al. Huntingtin interacts with REST NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet 2003; 35: 76-83.
    • (2003) Nat Genet , vol.35 , pp. 76-83
    • Zuccato, C.1    Tartari, M.2    Crotti, A.3
  • 90
    • 3142636768 scopus 로고    scopus 로고
    • Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
    • Gauthier LR, Charrin BC, Borrell-Pagès M, et al. Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell 2004; 118: 127-38.
    • (2004) Cell , vol.118 , pp. 127-138
    • Gauthier, L.R.1    Charrin, B.C.2    Borrell-Pagès, M.3
  • 91
    • 4444350918 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease
    • Canals JM, Pineda JR, Torres-Peraza JF, et al. Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease. J Neurosci 2004; 24: 7727-39.
    • (2004) J Neurosci , vol.24 , pp. 7727-7739
    • Canals, J.M.1    Pineda, J.R.2    Torres-Peraza, J.F.3
  • 92
    • 41749083721 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor over-expressionin the forebrain ameliorates Huntington's disease phenotypes in mice
    • Gharami K, Xie Y, An JJ, Tonegawa S, Xu B. Brain-derived neurotrophic factor over-expressionin the forebrain ameliorates Huntington's disease phenotypes in mice. J Neurochem 2008; 105: 369-79.
    • (2008) J Neurochem , vol.105 , pp. 369-379
    • Gharami, K.1    Xie, Y.2    An, J.J.3    Tonegawa, S.4    Xu, B.5
  • 94
    • 68149169019 scopus 로고    scopus 로고
    • BDNF regulates BIM expression levels in 3-nitropropionic acidtreated cortical neurons
    • Almeida S, Laço M, Cunha-Oliveira T, Oliveira CR, Rego AC. BDNF regulates BIM expression levels in 3-nitropropionic acidtreated cortical neurons. Neurobiol Dis 2009; 35: 448-56.
    • (2009) Neurobiol Dis , vol.35 , pp. 448-456
    • Almeida, S.1    Laço, M.2    Cunha-Oliveira, T.3    Oliveira, C.R.4    Rego, A.C.5
  • 95
    • 77953951286 scopus 로고    scopus 로고
    • Dysregulation of CREB activation and histone acetylation in 3-nitropropionic acid-treated cortical neurons: Prevention by BDNF and NGF
    • Almeida S, Cunha-Oliveira T, Laço M, Oliveira CR, Rego AC. Dysregulation of CREB activation and histone acetylation in 3-nitropropionic acid-treated cortical neurons: prevention by BDNF and NGF. Neurotox Res 2010; 17: 399-405.
    • (2010) Neurotox Res , vol.17 , pp. 399-405
    • Almeida, S.1    Cunha-Oliveira, T.2    Laço, M.3    Oliveira, C.R.4    Rego, A.C.5
  • 96
    • 34948855498 scopus 로고    scopus 로고
    • Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease
    • Cho SR, Benraiss A, Chmielnicki E, Samdani A, Economides A, Goldman SA. Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease. J Clin Invest 2007; 117: 2889-902.
    • (2007) J Clin Invest , vol.117 , pp. 2889-2902
    • Cho, S.R.1    Benraiss, A.2    Chmielnicki, E.3    Samdani, A.4    Economides, A.5    Goldman, S.A.6
  • 97
    • 78049525220 scopus 로고    scopus 로고
    • BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice
    • Xie Y, Hayden MR, Xu B. BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice. J Neurosci 2010; 30(44): 14708-18.
    • (2010) J Neurosci , vol.30 , Issue.44 , pp. 14708-14718
    • Xie, Y.1    Hayden, M.R.2    Xu, B.3
  • 98
    • 1542286877 scopus 로고    scopus 로고
    • Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism
    • Spires TL, Grote HE, Varshney NK, et al. Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism. J Neurosci 2004; 24: 2270-66.
    • (2004) J Neurosci , vol.24 , pp. 2266-2270
    • Spires, T.L.1    Grote, H.E.2    Varshney, N.K.3
  • 99
    • 46749109164 scopus 로고    scopus 로고
    • PYM50028, a novel, orally active, nonpeptide neurotrophic factor inducer, prevents and reverses neuronal damage induced by MPP+ in mesencephalic neurons and by MPTP in a mouse model of Parkinson's disease
    • Visanji NP, Orsi A, Johnston TH, et al. PYM50028, a novel, orally active, nonpeptide neurotrophic factor inducer, prevents and reverses neuronal damage induced by MPP+ in mesencephalic neurons and by MPTP in a mouse model of Parkinson's disease. FASEB J 2008; 22: 2488-97.
    • (2008) FASEB J , vol.22 , pp. 2488-2497
    • Visanji, N.P.1    Orsi, A.2    Johnston, T.H.3
  • 100
    • 63849151219 scopus 로고    scopus 로고
    • Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice
    • Simmons DA, Rex CS, Palmer L, et al. Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice. Proc Natl Acad Sci USA 2009; 106: 4906-11.
    • (2009) Proc Natl Acad Sci USA , vol.106 , pp. 4906-4911
    • Simmons, D.A.1    Rex, C.S.2    Palmer, L.3
  • 101
    • 49549088134 scopus 로고    scopus 로고
    • CEP-1347 reduces mutant huntingtin associated neurotoxicity and restores BDNF levels in R6/2 mice
    • Apostol BL, Simmons DA, Zuccato C, et al. CEP-1347 reduces mutant huntingtin associated neurotoxicity and restores BDNF levels in R6/2 mice. Mol Cell Neurosci 2008; 39: 8-20.
    • (2008) Mol Cell Neurosci , vol.39 , pp. 8-20
    • Apostol, B.L.1    Simmons, D.A.2    Zuccato, C.3
  • 102
    • 33644956205 scopus 로고    scopus 로고
    • Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain
    • Ginés S, Bosch M, Marco S, et al. Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain. Eur J Neurosci 2006; 23: 649-58.
    • (2006) Eur J Neurosci , vol.23 , pp. 649-658
    • Ginés, S.1    Bosch, M.2    Marco, S.3
  • 103
    • 77954362528 scopus 로고    scopus 로고
    • Impaired TrkB-mediated ERK1/2 activation in Huntington's disease knock-in striatal cells involves reduced p52/p46 Shc expression
    • Ginés S, Paoletti P, Alberch J. Impaired TrkB-mediated ERK1/2 activation in Huntington's disease knock-in striatal cells involves reduced p52/p46 Shc expression. J Biol Chem 2010; 285: 21537-48.
    • (2010) J Biol Chem , vol.285 , pp. 21537-21548
    • Ginés, S.1    Paoletti, P.2    Alberch, J.3
  • 104
    • 0037762555 scopus 로고    scopus 로고
    • Structural and functional neuroprotection in a rat model of Huntington's disease by viral gene transfer of GDNF
    • McBride JL, During MJ, Wuu J, Chen EY, Leurgans SE, Kordower JH. Structural and functional neuroprotection in a rat model of Huntington's disease by viral gene transfer of GDNF. Exp Neurol 2003; 181(2): 213-23.
    • (2003) Exp Neurol , vol.181 , Issue.2 , pp. 213-223
    • McBride, J.L.1    During, M.J.2    Wuu, J.3    Chen, E.Y.4    Leurgans, S.E.5    Kordower, J.H.6
  • 105
    • 2442702838 scopus 로고    scopus 로고
    • AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease
    • Kells AP, Fong DM, Dragunow M, During MJ, Young D, Connor B. AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease. Mol Ther 2004; 9(5): 682-8.
    • (2004) Mol Ther , vol.9 , Issue.5 , pp. 682-688
    • Kells, A.P.1    Fong, D.M.2    Dragunow, M.3    During, M.J.4    Young, D.5    Connor, B.6
  • 106
    • 16244373680 scopus 로고    scopus 로고
    • Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes
    • Popovic N, Maingay M, Kirik D, Brundin P. Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes. Exp Neurol 2005; 193: 65-74.
    • (2005) Exp Neurol , vol.193 , pp. 65-74
    • Popovic, N.1    Maingay, M.2    Kirik, D.3    Brundin, P.4
  • 107
    • 33745178085 scopus 로고    scopus 로고
    • Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington's disease
    • McBride JL, Ramaswamy S, Gasmi M, et al. Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington's disease. Proc Natl Acad Sci USA 2006; 103(24): 9345-50.
    • (2006) Proc Natl Acad Sci USA , vol.103 , Issue.24 , pp. 9345-9350
    • McBride, J.L.1    Ramaswamy, S.2    Gasmi, M.3
  • 108
    • 77953701829 scopus 로고    scopus 로고
    • Ex vivo delivery of GDNF maintains motor function and prevents neuronal loss in a transgenic mouse model of Huntington's disease
    • Ebert AD, Barber AE, Heins BM, Svendsen CN. Ex vivo delivery of GDNF maintains motor function and prevents neuronal loss in a transgenic mouse model of Huntington's disease. Exp Neurol 2010; 224(1): 155-62.
    • (2010) Exp Neurol , vol.224 , Issue.1 , pp. 155-162
    • Ebert, A.D.1    Barber, A.E.2    Heins, B.M.3    Svendsen, C.N.4
  • 109
    • 33845976361 scopus 로고    scopus 로고
    • Neuroprotection by GDNFsecreting stem cells in a Huntington's disease model: Optical neuroimage tracking of brain-grafted cells
    • Pineda JR, Rubio N, Akerud P, et al. Neuroprotection by GDNFsecreting stem cells in a Huntington's disease model: optical neuroimage tracking of brain-grafted cells. Gene Ther 2007; 14: 118-28.
    • (2007) Gene Ther , vol.14 , pp. 118-128
    • Pineda, J.R.1    Rubio, N.2    Akerud, P.3
  • 110
    • 1842868523 scopus 로고    scopus 로고
    • Dose-dependent neuroprotective effect of ciliary neurotrophic factor delivered via tetracycline-regulated lentiviral vectors in the quinolinic acid rat model of Huntington's disease
    • Régulier E, Pereira de Almeida L, Sommer B, Aebischer P, Déglon N. Dose-dependent neuroprotective effect of ciliary neurotrophic factor delivered via tetracycline-regulated lentiviral vectors in the quinolinic acid rat model of Huntington's disease. Hum Gene Ther 2002; 13: 1981-90.
    • (2002) Hum Gene Ther , vol.13 , pp. 1981-1990
    • Régulier, E.1    Pereira de Almeida, L.2    Sommer, B.3    Aebischer, P.4    Déglon, N.5
  • 111
    • 0346993604 scopus 로고    scopus 로고
    • Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice
    • Zala D, Bensadoun JC, Pereira de Almeida L, et al. Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice. Exp Neurol 2004; 185: 26-35.
    • (2004) Exp Neurol , vol.185 , pp. 26-35
    • Zala, D.1    Bensadoun, J.C.2    Pereira de Almeida, L.3
  • 112
    • 27744443276 scopus 로고    scopus 로고
    • Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment
    • Zala D, Benchoua A, Brouillet E, et al. Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol Dis 2005; 20: 785-98.
    • (2005) Neurobiol Dis , vol.20 , pp. 785-798
    • Zala, D.1    Benchoua, A.2    Brouillet, E.3
  • 113
    • 2442529846 scopus 로고    scopus 로고
    • Neuroprotective effects of encapsulated CNTF-producing cells in a rodent model of Huntington's disease are dependent on the proximity of the implant to the lesioned striatum
    • Emerich DF, Winn SR. Neuroprotective effects of encapsulated CNTF-producing cells in a rodent model of Huntington's disease are dependent on the proximity of the implant to the lesioned striatum. Cell Transplant 2004; 13: 253-9.
    • (2004) Cell Transplant , vol.13 , pp. 253-259
    • Emerich, D.F.1    Winn, S.R.2
  • 114
    • 5644295321 scopus 로고    scopus 로고
    • Neuroprotective gene therapy for Huntington's disease, using polymer-encapsulated cells engineered to secrete human ciliary neurotrophic factor: Results of a phase I study
    • Bloch J, Bachoud-Lévi AC, Déglon N, et al. Neuroprotective gene therapy for Huntington's disease, using polymer-encapsulated cells engineered to secrete human ciliary neurotrophic factor: results of a phase I study. Hum Gene Ther 2004; 15: 968-75.
    • (2004) Hum Gene Ther , vol.15 , pp. 968-975
    • Bloch, J.1    Bachoud-Lévi, A.C.2    Déglon, N.3
  • 115
    • 80053424337 scopus 로고    scopus 로고
    • IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease
    • Sadagurski M, Cheng Z, Rozzo A, et al. IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease. J Clin Invest 2011; 121(10): 4070-81.
    • (2011) J Clin Invest , vol.121 , Issue.10 , pp. 4070-4081
    • Sadagurski, M.1    Cheng, Z.2    Rozzo, A.3
  • 116
    • 80052520685 scopus 로고    scopus 로고
    • IGF-1 protects against diabetic features in an In vivo model of Huntington's disease
    • Duarte AI, Petit GH, Ranganathan S, et al. IGF-1 protects against diabetic features in an In vivo model of Huntington's disease. Exp Neurol 2011; 231(2): 314-9.
    • (2011) Exp Neurol , vol.231 , Issue.2 , pp. 314-319
    • Duarte, A.I.1    Petit, G.H.2    Ranganathan, S.3
  • 118
    • 6344269065 scopus 로고    scopus 로고
    • Insulin growth factor-1 protects against excitotoxicity in the rat striatum
    • Escartin C, Boyer F, Bemelmans AP, Hantraye P, Brouillet E. Insulin growth factor-1 protects against excitotoxicity in the rat striatum. Neuroreport 2004; 15: 2251-54.
    • (2004) Neuroreport , vol.15 , pp. 2251-2254
    • Escartin, C.1    Boyer, F.2    Bemelmans, A.P.3    Hantraye, P.4    Brouillet, E.5
  • 119
    • 0036083379 scopus 로고    scopus 로고
    • The IGF-1/Akt Pathway is neuroprotective in Huntington's Disease and involves huntingtin phosphorylation by Akt
    • Humbert S, Bryson EA, Cordelières FP, et al. The IGF-1/Akt Pathway is neuroprotective in Huntington's Disease and involves huntingtin phosphorylation by Akt. Dev Cell 2002; 2: 831-7.
    • (2002) Dev Cell , vol.2 , pp. 831-837
    • Humbert, S.1    Bryson, E.A.2    Cordelières, F.P.3
  • 120
    • 33644540193 scopus 로고    scopus 로고
    • Autophagy-mediated clearance of huntingtin aggregates triggered by the insulinsignalling pathway
    • Yamamoto A, Cremona ML, Rothman JE. Autophagy-mediated clearance of huntingtin aggregates triggered by the insulinsignalling pathway. J Cell Biol 2006; 172: 719-31.
    • (2006) J Cell Biol , vol.172 , pp. 719-731
    • Yamamoto, A.1    Cremona, M.L.2    Rothman, J.E.3
  • 121
    • 27344447235 scopus 로고    scopus 로고
    • The therapeutic role of creatine in Huntington's disease
    • Ryu H, Rosas HD, Hersch SM, Ferrante RJ. The therapeutic role of creatine in Huntington's disease. Pharmacol Ther 2005; 108: 193-207.
    • (2005) Pharmacol Ther , vol.108 , pp. 193-207
    • Ryu, H.1    Rosas, H.D.2    Hersch, S.M.3    Ferrante, R.J.4
  • 123
    • 0034660457 scopus 로고    scopus 로고
    • Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
    • Ferrante RJ, Andreassen OA, Jenkins BG, et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci 2000; 20: 4389-97.
    • (2000) J Neurosci , vol.20 , pp. 4389-4397
    • Ferrante, R.J.1    Andreassen, O.A.2    Jenkins, B.G.3
  • 124
    • 0038115294 scopus 로고    scopus 로고
    • Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice
    • Dedeoglu A, Kubilus JK, Yang L, et al. Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice. J Neurochem 2003; 85: 1359-67.
    • (2003) J Neurochem , vol.85 , pp. 1359-1367
    • Dedeoglu, A.1    Kubilus, J.K.2    Yang, L.3
  • 125
    • 0141959170 scopus 로고    scopus 로고
    • Creatine supplementation in Huntington's disease: A placebo-controlled pilot trial
    • Verbessem P, Lemiere J, Eijnde BO, et al. Creatine supplementation in Huntington's disease: a placebo-controlled pilot trial. Neurology 2003; 61: 925-30.
    • (2003) Neurology , vol.61 , pp. 925-930
    • Verbessem, P.1    Lemiere, J.2    Eijnde, B.O.3
  • 126
    • 77956250930 scopus 로고    scopus 로고
    • Coenzyme Q and mitochondrial disease
    • Quinzii CM, Hirano M. Coenzyme Q and mitochondrial disease. Dev Disabil Res Rev 2010; 16(2):183-8
    • (2010) Dev Disabil Res Rev , vol.16 , Issue.2 , pp. 183-188
    • Quinzii, C.M.1    Hirano, M.2
  • 127
    • 33745120560 scopus 로고    scopus 로고
    • Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice
    • Smith KM, Matson S, Matson WR, et al. Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice. Biochim Biophys Acta 2006; 1762: 616-26.
    • (2006) Biochim Biophys Acta , vol.1762 , pp. 616-626
    • Smith, K.M.1    Matson, S.2    Matson, W.R.3
  • 128
    • 0036523110 scopus 로고    scopus 로고
    • Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease
    • Ferrante RJ, Andreassen OA, Dedeoglu A, et al. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci 2002; 22: 1592-1599.
    • (2002) J Neurosci , vol.22 , pp. 1592-1599
    • Ferrante, R.J.1    Andreassen, O.A.2    Dedeoglu, A.3
  • 129
    • 85009226418 scopus 로고    scopus 로고
    • A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease
    • Huntington Study Group
    • Huntington Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001; 57: 397-404.
    • (2001) Neurology , vol.57 , pp. 397-404
  • 130
    • 31644439986 scopus 로고    scopus 로고
    • Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease mice
    • Stack EC, Smith KM, Ryu H, et al. Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease mice. Biochim Biophys Acta 2006; 1762: 373-80.
    • (2006) Biochim Biophys Acta , vol.1762 , pp. 373-380
    • Stack, E.C.1    Smith, K.M.2    Ryu, H.3
  • 131
    • 65549091910 scopus 로고    scopus 로고
    • Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases
    • Yang L, Calingasan NY, Wille EJ, et al. Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases. J Neurochem 2009; 109: 1427-39.
    • (2009) J Neurochem , vol.109 , pp. 1427-1439
    • Yang, L.1    Calingasan, N.Y.2    Wille, E.J.3
  • 132
    • 77956802394 scopus 로고    scopus 로고
    • Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects
    • Hyson HC, Kieburtz K, Shoulson I, et al. Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects. Mov Disord 2010; 25(12): 1924-28.
    • (2010) Mov Disord , vol.25 , Issue.12 , pp. 1924-1928
    • Hyson, H.C.1    Kieburtz, K.2    Shoulson, I.3
  • 133
    • 40349100270 scopus 로고    scopus 로고
    • Endogenous proresolving and antiinflammatory lipid mediators: A new pharmacologic genus
    • Serhan CN, Chiang N. Endogenous proresolving and antiinflammatory lipid mediators: a new pharmacologic genus. Br J Pharmacol 2008; 153: S200-15.
    • (2008) Br J Pharmacol , vol.153
    • Serhan, C.N.1    Chiang, N.2
  • 134
    • 33847622176 scopus 로고    scopus 로고
    • Ethyl-EPA in Huntington disease: Potentially relevant mechanism of action
    • Murck H, Manku M. Ethyl-EPA in Huntington disease: potentially relevant mechanism of action. Brain Res Bull 2007; 72: 159-64.
    • (2007) Brain Res Bull , vol.72 , pp. 159-164
    • Murck, H.1    Manku, M.2
  • 135
    • 27744478499 scopus 로고    scopus 로고
    • Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease
    • Van Raamsdonk JM, Pearson J, Rogers DA, et al. Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease. Exp Neurol 2005; 196(2): 266-72.
    • (2005) Exp Neurol , vol.196 , Issue.2 , pp. 266-272
    • Van Raamsdonk, J.M.1    Pearson, J.2    Rogers, D.A.3
  • 136
    • 54449085055 scopus 로고    scopus 로고
    • Reduction in cerebral atrophy associated with ethyleicosapentaenoic acid treatment in patients with Huntington's disease
    • Puri BK, Bydder GM, Manku MS, Clarke A, Waldman AD, Beckmann CF. Reduction in cerebral atrophy associated with ethyleicosapentaenoic acid treatment in patients with Huntington's disease. J Int Med Res 2008; 36(5): 896-905.
    • (2008) J Int Med Res , vol.36 , Issue.5 , pp. 896-905
    • Puri, B.K.1    Bydder, G.M.2    Manku, M.S.3    Clarke, A.4    Waldman, A.D.5    Beckmann, C.F.6
  • 137
    • 61449249687 scopus 로고    scopus 로고
    • Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: The TREND-HD study
    • Huntington Study Group TREND-HD Investigators
    • Huntington Study Group TREND-HD Investigators. Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study. Arch Neurol 2008; 65: 1582-9.
    • (2008) Arch Neurol , vol.65 , pp. 1582-1589
  • 138
    • 0037087771 scopus 로고    scopus 로고
    • Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
    • Wheeler VC, Gutekunst CA, Vrbanac V, et al. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet 2002; 11: 633-40.
    • (2002) Hum Mol Genet , vol.11 , pp. 633-640
    • Wheeler, V.C.1    Gutekunst, C.A.2    Vrbanac, V.3
  • 139
    • 0033912716 scopus 로고    scopus 로고
    • Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
    • Chen M, Ona VO, Li M, et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med 2000; 6: 797-801.
    • (2000) Nat Med , vol.6 , pp. 797-801
    • Chen, M.1    Ona, V.O.2    Li, M.3
  • 140
    • 0041335559 scopus 로고    scopus 로고
    • Minocycline inhibits caspaseindependent and-dependent mitochondrial cell death pathways in models of Huntington's disease
    • Wang X, Zhu S, Drozda M, et al. Minocycline inhibits caspaseindependent and-dependent mitochondrial cell death pathways in models of Huntington's disease. Proc Natl Acad Sci USA 2003; 100: 10483-7.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 10483-10487
    • Wang, X.1    Zhu, S.2    Drozda, M.3
  • 141
    • 0042845880 scopus 로고    scopus 로고
    • Minocycline and doxycycline are not beneficial in a model of Huntington's disease
    • Smith DL, Woodman B, Mahal A, et al. Minocycline and doxycycline are not beneficial in a model of Huntington's disease. Ann Neurol 2003; 54: 186-96.
    • (2003) Ann Neurol , vol.54 , pp. 186-196
    • Smith, D.L.1    Woodman, B.2    Mahal, A.3
  • 142
    • 3543092682 scopus 로고    scopus 로고
    • Minocycline safety and tolerability in Huntington disease
    • Huntington Study Group
    • Huntington Study Group. Minocycline safety and tolerability in Huntington disease. Neurology 2004; 63: 547-9.
    • (2004) Neurology , vol.63 , pp. 547-549
  • 143
    • 4444233214 scopus 로고    scopus 로고
    • Minocycline in Huntington's disease: A pilot study
    • Thomas M, Ashizawa T, Jankovic J. Minocycline in Huntington's disease: a pilot study. Mov Disord 2004; 19: 692-5.
    • (2004) Mov Disord , vol.19 , pp. 692-695
    • Thomas, M.1    Ashizawa, T.2    Jankovic, J.3
  • 145
    • 78649369122 scopus 로고    scopus 로고
    • A futility study of minocycline in Huntington's disease
    • Huntington Sudy Group DOMINO Investigators
    • Huntington Sudy Group DOMINO Investigators. A futility study of minocycline in Huntington's disease. Mov Disord 2010; 25(13): 2219-24.
    • (2010) Mov Disord , vol.25 , Issue.13 , pp. 2219-2224
  • 146
    • 0036677435 scopus 로고    scopus 로고
    • Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease
    • Keene CD, Rodrigues CM, Eich T, Chhabra MS, Steer CJ, Low WC. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc Natl Acad Sci USA 2002; 99: 10671-6.
    • (2002) Proc Natl Acad Sci USA , vol.99 , pp. 10671-10676
    • Keene, C.D.1    Rodrigues, C.M.2    Eich, T.3    Chhabra, M.S.4    Steer, C.J.5    Low, W.C.6
  • 147
    • 58149343340 scopus 로고    scopus 로고
    • Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease
    • Wang X, Zhu S, Pei Z, et al. Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease. J Neurosci 2008; 28: 9473-85.
    • (2008) J Neurosci , vol.28 , pp. 9473-9485
    • Wang, X.1    Zhu, S.2    Pei, Z.3
  • 148
    • 33745003424 scopus 로고    scopus 로고
    • Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin
    • Graham RK, Deng Y, Slow EJ, et al. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 2006; 125: 1179-91.
    • (2006) Cell , vol.125 , pp. 1179-1191
    • Graham, R.K.1    Deng, Y.2    Slow, E.J.3
  • 149
    • 84859244583 scopus 로고    scopus 로고
    • Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice
    • Uribe V, Wong BK, Graham RK, et al. Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice. Hum Mol Genet 2012; 21(9): 1954-67.
    • (2012) Hum Mol Genet , vol.21 , Issue.9 , pp. 1954-1967
    • Uribe, V.1    Wong, B.K.2    Graham, R.K.3
  • 150
    • 84867459264 scopus 로고    scopus 로고
    • Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease
    • e4fd085bfc9973
    • Landles C, Weiss A, Franklin S, Howland D, Bates G. Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS Curr 2012; 4: e4fd085bfc9973.
    • (2012) PLoS Curr , vol.4
    • Landles, C.1    Weiss, A.2    Franklin, S.3    Howland, D.4    Bates, G.5
  • 151
    • 84861630493 scopus 로고    scopus 로고
    • Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment
    • Gafni J, Papanikolaou T, Degiacomo F, et al. Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment. J Neurosci 2012; 32(22): 7454-65.
    • (2012) J Neurosci , vol.32 , Issue.22 , pp. 7454-7465
    • Gafni, J.1    Papanikolaou, T.2    Degiacomo, F.3
  • 152
    • 65249096525 scopus 로고    scopus 로고
    • Synthesis and In vitro evaluation of sulfonamide isatin Michael acceptors as small molecule inhibitors of caspase-6
    • Chu W, Rothfuss J, Chu Y, Zhou D, Mach RH. Synthesis and In vitro evaluation of sulfonamide isatin Michael acceptors as small molecule inhibitors of caspase-6. J Med Chem 2009; 52: 2188-91.
    • (2009) J Med Chem , vol.52 , pp. 2188-2191
    • Chu, W.1    Rothfuss, J.2    Chu, Y.3    Zhou, D.4    Mach, R.H.5
  • 153
  • 154
    • 2642586352 scopus 로고    scopus 로고
    • Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
    • Ravikumar B, Vacher C, Berger Z, 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: 585-95.
    • (2004) Nat Genet , vol.36 , pp. 585-595
    • Ravikumar, B.1    Vacher, C.2    Berger, Z.3
  • 155
    • 77951665859 scopus 로고    scopus 로고
    • Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
    • Martinez-Vicente M, Talloczy Z, Wong E, et al. Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease. Nat Neurosci 2010; 13(5): 567-76.
    • (2010) Nat Neurosci , vol.13 , Issue.5 , pp. 567-576
    • Martinez-Vicente, M.1    Talloczy, Z.2    Wong, E.3
  • 156
    • 42249106042 scopus 로고    scopus 로고
    • Novel targets for Huntington's disease in an mTOR-independent autophagy pathway
    • Williams A, Sarkar S, Cuddon P, et al. Novel targets for Huntington's disease in an mTOR-independent autophagy pathway. Nat Chem Biol 2008; 4: 295-305.
    • (2008) Nat Chem Biol , vol.4 , pp. 295-305
    • Williams, A.1    Sarkar, S.2    Cuddon, P.3
  • 157
    • 34247161367 scopus 로고    scopus 로고
    • Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein
    • Sarkar S, Davies JE, Huang Z, Tunnacliffe A, Rubinsztein DC. Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein. J Biol Chem 2007; 282: 5641-52.
    • (2007) J Biol Chem , vol.282 , pp. 5641-5652
    • Sarkar, S.1    Davies, J.E.2    Huang, Z.3    Tunnacliffe, A.4    Rubinsztein, D.C.5
  • 158
    • 1642633757 scopus 로고    scopus 로고
    • Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
    • Tanaka M, Machida Y, Niu S, et al. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat Med 2004; 10: 148-54.
    • (2004) Nat Med , vol.10 , pp. 148-154
    • Tanaka, M.1    McHida, Y.2    Niu, S.3
  • 159
    • 0032014092 scopus 로고    scopus 로고
    • Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine
    • Kahlem P, Green H, Djian P. Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine. Mol Cell 1998; 1: 595-601.
    • (1998) Mol Cell , vol.1 , pp. 595-601
    • Kahlem, P.1    Green, H.2    Djian, P.3
  • 160
    • 0037109665 scopus 로고    scopus 로고
    • Therapeutic effects of cystamine in a murine model of Huntington's disease
    • Dedeoglu A, Kubilus JK, Jeitner TM, et al. Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 2002; 22: 8942-50.
    • (2002) J Neurosci , vol.22 , pp. 8942-8950
    • Dedeoglu, A.1    Kubilus, J.K.2    Jeitner, T.M.3
  • 161
    • 15944409947 scopus 로고    scopus 로고
    • Cerebral PET imaging and histological evidence of transglutaminase inhibitor cystamine induced neuroprotection in transgenic R6/2 mouse model of Huntington's disease
    • Wang X, Sarkar A, Cicchetti F, et al. Cerebral PET imaging and histological evidence of transglutaminase inhibitor cystamine induced neuroprotection in transgenic R6/2 mouse model of Huntington's disease. J Neurol Sci 2005; 231(1-2): 57-66.
    • (2005) J Neurol Sci , vol.231 , Issue.1-2 , pp. 57-66
    • Wang, X.1    Sarkar, A.2    Cicchetti, F.3
  • 162
    • 25644445315 scopus 로고    scopus 로고
    • Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease
    • van Raamsdonk JM, Pearson J, Bailey CD, et al. Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease. J Neurochem 2005; 95(1): 210-20.
    • (2005) J Neurochem , vol.95 , Issue.1 , pp. 210-220
    • van Raamsdonk, J.M.1    Pearson, J.2    Bailey, C.D.3
  • 163
    • 33645450264 scopus 로고    scopus 로고
    • Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells
    • Mao Z, Choo YS, Lesort M. Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells. Eur J Neurosci 2006; 23(7): 1701-10.
    • (2006) Eur J Neurosci , vol.23 , Issue.7 , pp. 1701-1710
    • Mao, Z.1    Choo, Y.S.2    Lesort, M.3
  • 164
    • 33646421164 scopus 로고    scopus 로고
    • Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase
    • Borrell-Pagès M, Canals JM, Cordelières FP, et al. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J Clin Invest 2006; 116: 1410-24.
    • (2006) J Clin Invest , vol.116 , pp. 1410-1424
    • Borrell-Pagès, M.1    Canals, J.M.2    Cordelières, F.P.3
  • 165
    • 44449131447 scopus 로고    scopus 로고
    • Huntington's disease: From pathology and genetics to potential therapies
    • Imarisio S, Carmichael J, Korolchuk V, et al. Huntington's disease: from pathology and genetics to potential therapies. Biochem J 2008; 412: 191-209.
    • (2008) Biochem J , vol.412 , pp. 191-209
    • Imarisio, S.1    Carmichael, J.2    Korolchuk, V.3
  • 166
    • 33747069946 scopus 로고    scopus 로고
    • New targets for pharmacological intervention in the glutamatergic synapse
    • Gardoni F, Di Luca M. New targets for pharmacological intervention in the glutamatergic synapse. Eur J Pharmacol 2006; 545: 2-10.
    • (2006) Eur J Pharmacol , vol.545 , pp. 2-10
    • Gardoni, F.1    Di Luca, M.2
  • 167
    • 20844460621 scopus 로고    scopus 로고
    • The molecular basis of memantine action in Alzheimer's disease and other neurologic disorders: Low-affinity, uncompetitive antagonism
    • Lipton SA. The molecular basis of memantine action in Alzheimer's disease and other neurologic disorders: low-affinity, uncompetitive antagonism. Curr Alzheimer Res 2005; 2: 155-65.
    • (2005) Curr Alzheimer Res , vol.2 , pp. 155-165
    • Lipton, S.A.1
  • 168
    • 33750378660 scopus 로고    scopus 로고
    • Memantine reduces striatal cell death with decreasing calpain level in 3-nitropropionic model of Huntington's disease
    • Lee ST, Chu K, Park JE. Memantine reduces striatal cell death with decreasing calpain level in 3-nitropropionic model of Huntington's disease. Brain Res 2006; 1118: 199-207.
    • (2006) Brain Res , vol.1118 , pp. 199-207
    • Lee, S.T.1    Chu, K.2    Park, J.E.3
  • 169
    • 74549181538 scopus 로고    scopus 로고
    • Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice
    • Milnerwood AJ, Gladding CM, Pouladi MA, et al. Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron 2010; 65: 178-90.
    • (2010) Neuron , vol.65 , pp. 178-190
    • Milnerwood, A.J.1    Gladding, C.M.2    Pouladi, M.A.3
  • 170
    • 71549143207 scopus 로고    scopus 로고
    • Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin
    • Okamoto S, Pouladi MA, Talantova M, et al. Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med 2009; 15: 1407-13.
    • (2009) Nat Med , vol.15 , pp. 1407-1413
    • Okamoto, S.1    Pouladi, M.A.2    Talantova, M.3
  • 171
    • 34548814154 scopus 로고    scopus 로고
    • A pilot study of the clinical efficacy and safety of memantine for Huntington's disease
    • Ondo WG, Mejia NI, Hunter CB. A pilot study of the clinical efficacy and safety of memantine for Huntington's disease. Parkinsonism Relat Disord 2007; 13: 453-4.
    • (2007) Parkinsonism Relat Disord , vol.13 , pp. 453-454
    • Ondo, W.G.1    Mejia, N.I.2    Hunter, C.B.3
  • 172
    • 34047161070 scopus 로고    scopus 로고
    • Functions, dysfunctions and possible therapeutic relevance of adenosine A2A receptors in Huntington's disease
    • Popoli P, Blum D, Martire A, Ledent C, Ceruti S, Abbracchio MP. Functions, dysfunctions and possible therapeutic relevance of adenosine A2A receptors in Huntington's disease. Prog Neurobiol 2007; 81: 331-48.
    • (2007) Prog Neurobiol , vol.81 , pp. 331-348
    • Popoli, P.1    Blum, D.2    Martire, A.3    Ledent, C.4    Ceruti, S.5    Abbracchio, M.P.6
  • 173
    • 20244362093 scopus 로고    scopus 로고
    • CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model
    • Chou SY, Lee YC, Chen HM, et al. CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model. J Neurochem 2005; 93: 310-20.
    • (2005) J Neurochem , vol.93 , pp. 310-320
    • Chou, S.Y.1    Lee, Y.C.2    Chen, H.M.3
  • 174
    • 35348983910 scopus 로고    scopus 로고
    • Behavioral and electrophysiological effects of the adenosine A2A receptor antagonist SCH 58261 in R6/2 Huntington's disease mice
    • Domenici MR, Scattoni ML, Martire 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: 197-205.
    • (2007) Neurobiol Dis , vol.28 , pp. 197-205
    • Domenici, M.R.1    Scattoni, M.L.2    Martire, A.3
  • 175
    • 36249024154 scopus 로고    scopus 로고
    • Adenosine A(2A) receptors modulate BDNF both in normal conditions and in experimental models of Huntington's disease
    • Potenza RL, Tebano MT, Martire A, et al. Adenosine A(2A) receptors modulate BDNF both in normal conditions and in experimental models of Huntington's disease. Purinergic Signal 2007; 3: 333-8.
    • (2007) Purinergic Signal , vol.3 , pp. 333-338
    • Potenza, R.L.1    Tebano, M.T.2    Martire, A.3
  • 176
    • 76149084493 scopus 로고    scopus 로고
    • A Randomized, Placebo-Controlled Trial of Latrepirdine in Huntington Disease
    • Kieburtz K, McDermott MP, Voss TS, et al. A Randomized, Placebo-Controlled Trial of Latrepirdine in Huntington Disease. Arch Neurol 2010; 67: 154-60.
    • (2010) Arch Neurol , vol.67 , pp. 154-160
    • Kieburtz, K.1    McDermott, M.P.2    Voss, T.S.3
  • 177
    • 55349083365 scopus 로고    scopus 로고
    • Evaluation of Dimebon in cellular model of Huntington's disease
    • Wu J, Li Q, Bezprozvanny I. Evaluation of Dimebon in cellular model of Huntington's disease. Mol Neurodegener 2008; 3: 15.
    • (2008) Mol Neurodegener , vol.3 , pp. 15
    • Wu, J.1    Li, Q.2    Bezprozvanny, I.3
  • 178
    • 0034944798 scopus 로고    scopus 로고
    • Antihistamine agent Dimebon as a novel neuroprotector and a cognition enhancer
    • Bachurin S, Bukatina E, Lermontova N, et al. Antihistamine agent Dimebon as a novel neuroprotector and a cognition enhancer. Ann NY Acad Sci 2001; 939: 425-35.
    • (2001) Ann NY Acad Sci , vol.939 , pp. 425-435
    • Bachurin, S.1    Bukatina, E.2    Lermontova, N.3
  • 179
    • 60549103857 scopus 로고    scopus 로고
    • The R6 lines of transgenic mice: A model for screening new therapies for Huntington's disease
    • Gil JM, Rego AC. The R6 lines of transgenic mice: A model for screening new therapies for Huntington's disease. Brain Res Rev 2009; 59: 410-31.
    • (2009) Brain Res Rev , vol.59 , pp. 410-431
    • Gil, J.M.1    Rego, A.C.2
  • 180
    • 26844494370 scopus 로고    scopus 로고
    • Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA
    • Wang YL, Liu W, Wada E, Murata M, Wada K, Kanazawa I. Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA. Neurosci Res 2005; 53: 241-9.
    • (2005) Neurosci Res , vol.53 , pp. 241-249
    • Wang, Y.L.1    Liu, W.2    Wada, E.3    Murata, M.4    Wada, K.5    Kanazawa, I.6
  • 181
    • 36749033738 scopus 로고    scopus 로고
    • Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
    • DiFiglia M, Sena-Esteves M, Chase K, et al. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc Natl Acad Sci USA 2007; 104: 17204-9.
    • (2007) Proc Natl Acad Sci USA , vol.104 , pp. 17204-17209
    • DiFiglia, M.1    Sena-Esteves, M.2    Chase, K.3
  • 182
    • 34248146697 scopus 로고    scopus 로고
    • High-capacity adenoviral vector-mediated reduction of huntingtin aggregate load In vitro and In vivo
    • Huang B, Schiefer J, Sass C, Landwehrmeyer GB, Kosinski CM, Kochanek S. High-capacity adenoviral vector-mediated reduction of huntingtin aggregate load In vitro and In vivo. Hum Gene Ther 2007; 18: 303-11.
    • (2007) Hum Gene Ther , vol.18 , pp. 303-311
    • Huang, B.1    Schiefer, J.2    Sass, C.3    Landwehrmeyer, G.B.4    Kosinski, C.M.5    Kochanek, S.6
  • 183
    • 28644433087 scopus 로고    scopus 로고
    • Normal huntingtin function: An alternative approach to Huntington's disease
    • Cattaneo E, Zuccato C, Tartari M. Normal huntingtin function: an alternative approach to Huntington's disease. Nat Rev Neurosci 2005; 6: 919-30.
    • (2005) Nat Rev Neurosci , vol.6 , pp. 919-930
    • Cattaneo, E.1    Zuccato, C.2    Tartari, M.3
  • 184
    • 84860192454 scopus 로고    scopus 로고
    • Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum
    • Grondin R, Kaytor MD, Ai Y, et al. Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum. Brain 2012; 135(Pt 4): 1197-209.
    • (2012) Brain , vol.135 , Issue.PART 4 , pp. 1197-1209
    • Grondin, R.1    Kaytor, M.D.2    Ai, Y.3
  • 185
    • 0038132996 scopus 로고    scopus 로고
    • Allele-specific silencing of dominant disease genes
    • Miller VM, Xia H, Marrs GL, et al. Allele-specific silencing of dominant disease genes. Proc Natl Acad Sci USA 2003; 100: 7195-200.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 7195-7200
    • Miller, V.M.1    Xia, H.2    Marrs, G.L.3
  • 186
    • 56149120246 scopus 로고    scopus 로고
    • Linking SNPs to CAG repeat length in Huntington's disease patients
    • Liu W, Kennington LA, Rosas HD, et al. Linking SNPs to CAG repeat length in Huntington's disease patients. Nat Methods 2008; 5: 951-3.
    • (2008) Nat Methods , vol.5 , pp. 951-953
    • Liu, W.1    Kennington, L.A.2    Rosas, H.D.3
  • 187
    • 47549105506 scopus 로고    scopus 로고
    • Identification and allele-specific silencing of the mutant huntingtin allele in Huntington's disease patient-derived fibroblasts
    • Van Bilsen PH, Jaspers L, Lombardi MS, Odekerken JC, Burright EN, Kaemmerer WF. Identification and allele-specific silencing of the mutant huntingtin allele in Huntington's disease patient-derived fibroblasts. Hum Gene Ther 2008; 19: 710-9.
    • (2008) Hum Gene Ther , vol.19 , pp. 710-719
    • Van Bilsen, P.H.1    Jaspers, L.2    Lombardi, M.S.3    Odekerken, J.C.4    Burright, E.N.5    Kaemmerer, W.F.6
  • 188
    • 67349263503 scopus 로고    scopus 로고
    • A majority of Huntington's disease patients may be treatable by individualized allele-specific RNA interference
    • Lombardi MS, Jaspers L, Spronkmans C, et al. A majority of Huntington's disease patients may be treatable by individualized allele-specific RNA interference. Exp Neurol 2009; 217: 312-9.
    • (2009) Exp Neurol , vol.217 , pp. 312-319
    • Lombardi, M.S.1    Jaspers, L.2    Spronkmans, C.3
  • 189
    • 67349159137 scopus 로고    scopus 로고
    • Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients
    • Pfister EL, Kennington L, Straubhaar J, et al. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol 2009; 19: 774-8.
    • (2009) Curr Biol , vol.19 , pp. 774-778
    • Pfister, E.L.1    Kennington, L.2    Straubhaar, J.3
  • 190
    • 66149181896 scopus 로고    scopus 로고
    • Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs
    • Hu J, Matsui M, Gagnon KT, et al. Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs. Nat Biotechnol 2009; 27: 478-84.
    • (2009) Nat Biotechnol , vol.27 , pp. 478-484
    • Hu, J.1    Matsui, M.2    Gagnon, K.T.3
  • 191
    • 84887018742 scopus 로고    scopus 로고
    • Mitochondrial respiratory chain complex activity and bioenergetic alterations in human platelets derived from pre-symptomatic and symptomatic Huntington's disease carriers
    • Silva AC, Almeida S, Laço M, et al. Mitochondrial respiratory chain complex activity and bioenergetic alterations in human platelets derived from pre-symptomatic and symptomatic Huntington's disease carriers. Mitochondrion 2013; 13(6): 801-9.
    • (2013) Mitochondrion , vol.13 , Issue.6 , pp. 801-809
    • Silva, A.C.1    Almeida, S.2    Laço, M.3
  • 192
    • 77953810571 scopus 로고    scopus 로고
    • Earliest functional declines in Huntington disease
    • Beglinger LJ, O'Rourke JJ, Wang C, et al. Earliest functional declines in Huntington disease. Psychiatry Res 2010; 178: 414-8
    • (2010) Psychiatry Res , vol.178 , pp. 414-418
    • Beglinger, L.J.1    O'Rourke, J.J.2    Wang, C.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.