-
1
-
-
0035164243
-
The molecular biology of Huntington's disease
-
Ho LW, Carmichael J, Swart J, Wyttenbach A, Rankin J, Rubinsztein DC. The molecular biology of Huntington's disease. Psychol Med 2001; 31: 3-14.
-
(2001)
Psychol Med
, vol.31
, pp. 3-14
-
-
Ho, L.W.1
Carmichael, J.2
Swart, J.3
Wyttenbach, A.4
Rankin, J.5
Rubinsztein, D.C.6
-
2
-
-
0027480960
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
13
-
-
0021722327
-
Clinical correlates of dementia and disability in Huntington's disease
-
Brandt J, Strauss ME, Larus J, Jensen B, Folstein SE, Folstein MF. Clinical correlates of dementia and disability in Huntington's disease. J Clin Neuropsychol 1984; 6(4): 401-12.
-
(1984)
J Clin Neuropsychol
, vol.6
, Issue.4
, pp. 401-412
-
-
Brandt, J.1
Strauss, M.E.2
Larus, J.3
Jensen, B.4
Folstein, S.E.5
Folstein, M.F.6
-
14
-
-
0020954957
-
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
-
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
-
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
-
17
-
-
0342980401
-
Bradykinesia in Huntington's disease
-
Garcia-Ruiz PJ, Gomez-Tortosa E, Sanchez-Bernados V, Rojo A, Fontán A, Garcia de Yebenes J. Bradykinesia in Huntington's disease. Clin Neuropharmacol 2000; 23(1): 50-2.
-
(2000)
Clin Neuropharmacol
, vol.23
, Issue.1
, pp. 50-52
-
-
Garcia-Ruiz, P.J.1
Gomez-Tortosa, E.2
Sanchez-Bernados, V.3
Rojo, A.4
Fontán, A.5
de Yebenes Garcia, J.6
-
18
-
-
77955446151
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
-
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
-
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
-
27
-
-
0035297107
-
Impact of Huntington's disease on quality of life
-
Helder DI, Kaptein AA, van Kempen GM, van Houwelingen JC, Roos RA. Impact of Huntington's disease on quality of life. Mov Disord 2001; 16(2): 325-30.
-
(2001)
Mov Disord
, vol.16
, Issue.2
, pp. 325-330
-
-
Helder, D.I.1
Kaptein, A.A.2
van Kempen, G.M.3
van Houwelingen, J.C.4
Roos, R.A.5
-
28
-
-
79951974420
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
38
-
-
0020084322
-
Tiapride in the treatment of Huntington's chorea
-
Roos RA, Buruma OJ, Bruyn GW, Kemp B, van der Velde EA. Tiapride in the treatment of Huntington's chorea. Acta Neurol Scand 1982; 65: 45-50.
-
(1982)
Acta Neurol Scand
, vol.65
, pp. 45-50
-
-
Roos, R.A.1
Buruma, O.J.2
Bruyn, G.W.3
Kemp, B.4
van der Velde, E.A.5
-
39
-
-
33745759912
-
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
-
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
-
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
-
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
-
43
-
-
0030790234
-
Clozapine versus placebo in Huntington's disease: A double blind randomised comparative study
-
van Vugt JP, Siesling S, Vergeer M, van der Velde EA, Roos RA. Clozapine versus placebo in Huntington's disease: a double blind randomised comparative study. J Neurol Neurosurg Psychiatry 1997; 63: 35-9.
-
(1997)
J Neurol Neurosurg Psychiatry
, vol.63
, pp. 35-39
-
-
van Vugt, J.P.1
Siesling, S.2
Vergeer, M.3
van der Velde, E.A.4
Roos, R.A.5
-
45
-
-
1842854595
-
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
-
46
-
-
39749165003
-
Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease
-
Duff K, Beglinger LJ, O'Rourke ME, Nopoulos P, Paulson HL, Paulsen JS. Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Ann Clin Psychiatry 2008; 20: 1-3
-
(2008)
Ann Clin Psychiatry
, vol.20
, pp. 1-3
-
-
Duff, K.1
Beglinger, L.J.2
O'Rourke, M.E.3
Nopoulos, P.4
Paulson, H.L.5
Paulsen, J.S.6
-
47
-
-
0347285447
-
Ziprasidone in Huntington's disease: The first case reports
-
Bonelli RM, Mayr BM, Niederwieser G, Reisecker F, Kapfhammer HP. Ziprasidone in Huntington's disease: the first case reports. J Psychopharmacol 2003; 17: 459-60.
-
(2003)
J Psychopharmacol
, vol.17
, pp. 459-460
-
-
Bonelli, R.M.1
Mayr, B.M.2
Niederwieser, G.3
Reisecker, F.4
Kapfhammer, H.P.5
-
48
-
-
29544445190
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
57
-
-
0035118773
-
Short-term effects of olanzapine in Huntington's disease
-
Squitieri F, Cannella M, Piorcellini A, Brusa L, Simonelli M, Ruggieri S. Short-term effects of olanzapine in Huntington's disease. Neuropsychiatry Neuropsychol Behav Neurol 2001; 14: 69-72.
-
(2001)
Neuropsychiatry Neuropsychol Behav Neurol
, vol.14
, pp. 69-72
-
-
Squitieri, F.1
Cannella, M.2
Piorcellini, A.3
Brusa, L.4
Simonelli, M.5
Ruggieri, S.6
-
58
-
-
27644524469
-
Cognitive disorders and neurogenesis deficits in Huntington's disease mice are rescued by fluoxetine
-
Grote HE, Bull ND, Howard ML, van Dellen A, Blakemore C, Bartlett PF, Hannan AJ. Cognitive disorders and neurogenesis deficits in Huntington's disease mice are rescued by fluoxetine. Eur J Neurosci 2005; 22(8): 2081-8.
-
(2005)
Eur J Neurosci
, vol.22
, Issue.8
, pp. 2081-2088
-
-
Grote, H.E.1
Bull, N.D.2
Howard, M.L.3
van Dellen, A.4
Blakemore, C.5
Bartlett, P.F.6
Hannan, A.J.7
-
59
-
-
16044373842
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
87
-
-
38949185264
-
Modulation of sirtuins: New targets for antiageing
-
Pallàs M, Verdaguer E, Tajes M, Gutierrez-Cuesta J, Camins A. Modulation of sirtuins: new targets for antiageing. Recent Pat CNS Drug Discov 2008; 3: 61-9.
-
(2008)
Recent Pat CNS Drug Discov
, vol.3
, pp. 61-69
-
-
Pallàs, M.1
Verdaguer, E.2
Tajes, M.3
Gutierrez-Cuesta, J.4
Camins, A.5
-
88
-
-
77952413052
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
117
-
-
84902535945
-
IGF-1 Intranasal Administration Rescues Huntington's Disease Phenotypes in YAC128 Mice
-
[E-pub ahead of print]
-
Lopes C, Ribeiro M, Duarte A, Humbert S, Saudou F, Pereira de Almeida L, Hayden M, Rego AC. IGF-1 Intranasal Administration Rescues Huntington's Disease Phenotypes in YAC128 Mice. Mol Neurobiol 2013; [E-pub ahead of print].
-
(2013)
Mol Neurobiol
-
-
Lopes, C.1
Ribeiro, M.2
Duarte, A.3
Humbert, S.4
Saudou, F.5
Pereira de Almeida, L.6
Hayden, M.7
Rego, A.C.8
-
118
-
-
6344269065
-
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
-
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
-
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
-
122
-
-
0036294859
-
Direct antioxidant properties of creatine
-
Lawler JM, Barnes WS, Wu G, Song W, Demaree S. Direct antioxidant properties of creatine. Biochem Biophys Res Commun 2002; 290: 47-52.
-
(2002)
Biochem Biophys Res Commun
, vol.290
, pp. 47-52
-
-
Lawler, J.M.1
Barnes, W.S.2
Wu, G.3
Song, W.4
Demaree, S.5
-
123
-
-
0034660457
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
-
77649219699
-
Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice
-
Zheng S, Clabough EB, Sarkar S, Futter M, Rubinsztein DC, Zeitlin SO. Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice. PLoS Genet 2010; 6: e1000838.
-
(2010)
PLoS Genet
, vol.6
-
-
Zheng, S.1
Clabough, E.B.2
Sarkar, S.3
Futter, M.4
Rubinsztein, D.C.5
Zeitlin, S.O.6
-
154
-
-
2642586352
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
|