-
2
-
-
0013218415
-
-
Oxford University Press, Oxford
-
Bates G : Huntington's disease. Oxford University Press, Oxford, 2002
-
(2002)
Huntington's disease
-
-
Bates, G.1
-
3
-
-
0026928944
-
New insights into the clinical features, pathogenesis and molecular genetics of Huntington disease
-
Kremer B, Weber B, Hayden MR : New insights into the clinical features, pathogenesis and molecular genetics of Huntington disease. Brain Pathol 1992 ; 2 : 321-35
-
(1992)
Brain Pathol
, vol.2
, pp. 321-335
-
-
Kremer, B.1
Weber, B.2
Hayden, M.R.3
-
4
-
-
0029906665
-
Executive and mnemonic functions in early Huntington's disease
-
Lawrence AD, Sahakian BJ, Hodges JR, Rosser AE, Lange KW, Robbins TW : Executive and mnemonic functions in early Huntington's disease. Brain 1996 ; 119 : 1633-45
-
(1996)
Brain
, vol.119
, pp. 1633-1645
-
-
Lawrence, A.D.1
Sahakian, B.J.2
Hodges, J.R.3
Rosser, A.E.4
Lange, K.W.5
Robbins, T.W.6
-
5
-
-
0022395922
-
Neuropathological classification of Huntington's disease
-
Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP Jr : Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol : 1985 ; 44 : 559-77
-
(1985)
J Neuropathol Exp Neurol
, vol.44
, pp. 559-577
-
-
Vonsattel, J.P.1
Myers, R.H.2
Stevens, T.J.3
Ferrante, R.J.4
Bird, E.D.5
Richardson Jr, E.P.6
-
6
-
-
0022343684
-
Selective sparing of a class of striatal neurons in Huntington's disease
-
Ferrante RJ, Kowall NW, Beal MF, Richardson EP Jr, Bird ED, Martin JB : Selective sparing of a class of striatal neurons in Huntington's disease. Science 1985 ; 230 : 561-3
-
(1985)
Science
, vol.230
, pp. 561-563
-
-
Ferrante, R.J.1
Kowall, N.W.2
Beal, M.F.3
Richardson Jr, E.P.4
Bird, E.D.5
Martin, J.B.6
-
7
-
-
0023227209
-
Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease
-
Ferrante RJ, Beal MF, Kowall NW, Richardson EP Jr, Martin JB : Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease. Brain Res 1987 ; 411 : 162-6
-
(1987)
Brain Res
, vol.411
, pp. 162-166
-
-
Ferrante, R.J.1
Beal, M.F.2
Kowall, N.W.3
Richardson Jr, E.P.4
Martin, J.B.5
-
8
-
-
0023080626
-
Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease
-
Ferrante RJ, Kowall NW, Beal MF, Martin JB, Bird ED, Richardson EP Jr : Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease. J Neuropathol Exp Neurol 1987 ; 46 : 12-27
-
(1987)
J Neuropathol Exp Neurol
, vol.46
, pp. 12-27
-
-
Ferrante, R.J.1
Kowall, N.W.2
Beal, M.F.3
Martin, J.B.4
Bird, E.D.5
Richardson Jr, E.P.6
-
10
-
-
4644245086
-
In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease
-
Fennema-Notestine C, Archibald SL, Jacobson MW et al. : In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease. Neurology 2004 ; 63 : 989-95
-
(2004)
Neurology
, vol.63
, pp. 989-995
-
-
Fennema-Notestine, C.1
Archibald, S.L.2
Jacobson, M.W.3
-
11
-
-
1042276562
-
Evidence for more widespread cerebral pathology in early HD : An MRI-based morphometry analysis
-
Kassubek J, Gaus W, Landwehrmeyer GB : Evidence for more widespread cerebral pathology in early HD : an MRI-based morphometry analysis. Neurology 2004 ; 62 : 523-4
-
(2004)
Neurology
, vol.62
, pp. 523-524
-
-
Kassubek, J.1
Gaus, W.2
Landwehrmeyer, G.B.3
-
12
-
-
33747602312
-
Hypothalamic-endocrine aspects in Huntington's disease
-
Petersen A, Bjorkqvist M : Hypothalamic-endocrine aspects in Huntington's disease. Eur J Neurosci 2006 ; 24 : 961-7
-
(2006)
Eur J Neurosci
, vol.24
, pp. 961-967
-
-
Petersen, A.1
Bjorkqvist, M.2
-
13
-
-
0035111235
-
Early and progressive accumulation of reactive microglia in the Huntington disease brain
-
Sapp E, Kegel KB, Aronin N et al. : Early and progressive accumulation of reactive microglia in the Huntington disease brain. J Neuropathol Exp Neurol 2001 ; 60 : 161-72
-
(2001)
J Neuropathol Exp Neurol
, vol.60
, pp. 161-172
-
-
Sapp, E.1
Kegel, K.B.2
Aronin, N.3
-
14
-
-
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
-
The Huntington's disease collaborative research group : A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell 1993 ; 72 : 971-83
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
15
-
-
8144228406
-
Trinucleotide repeats and neurodegenerative disease
-
Everett CM, Wood NW : Trinucleotide repeats and neurodegenerative disease. Brain 2004 ; 127 : 2385-405
-
(2004)
Brain
, vol.127
, pp. 2385-2405
-
-
Everett, C.M.1
Wood, N.W.2
-
17
-
-
0034329159
-
Molecular genetics : Unmasking polyglutamine triggers in neurodegenerative disease
-
Gusella JF, McDonald ME : Molecular genetics : unmasking polyglutamine triggers in neurodegenerative disease. Nat Rev Neurosci 2000 ; 1 : 109-15
-
(2000)
Nat Rev Neurosci
, vol.1
, pp. 109-115
-
-
Gusella, J.F.1
McDonald, M.E.2
-
18
-
-
0030919726
-
CAG repeat number governs the development rate of pathology in Huntington's disease
-
Penney JB Jr, Vonsattel JP, MacDonald ME, Gusella JF, Myers RH : CAG repeat number governs the development rate of pathology in Huntington's disease. Ann Neurol 1997 ; 41: 689-92
-
(1997)
Ann Neurol
, vol.41
, pp. 689-692
-
-
Penney Jr, J.B.1
Vonsattel, J.P.2
MacDonald, M.E.3
Gusella, J.F.4
Myers, R.H.5
-
19
-
-
0027261537
-
Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease
-
Snell RG, MacMillan JC, Cheadle JP et al. : Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease. Nat Genet 1993 ; 4 : 393-97
-
(1993)
Nat Genet
, vol.4
, pp. 393-397
-
-
Snell, R.G.1
MacMillan, J.C.2
Cheadle, J.P.3
-
20
-
-
12144288251
-
-
Wexler NS, Lorimer J, Porter J et al. : Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A 2004 ; 101 : 3498-503
-
Wexler NS, Lorimer J, Porter J et al. : Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A 2004 ; 101 : 3498-503
-
-
-
-
21
-
-
0942298741
-
Different phenotypic expression in monozygotic twins with Huntington disease
-
Anca MH, Gazit E, Loewenthal R, Ostrovsky O, Frydman M, Giladi N : Different phenotypic expression in monozygotic twins with Huntington disease. Am J Med Genet A 2004 ; 124 : 89-91
-
(2004)
Am J Med Genet A
, vol.124
, pp. 89-91
-
-
Anca, M.H.1
Gazit, E.2
Loewenthal, R.3
Ostrovsky, O.4
Frydman, M.5
Giladi, N.6
-
23
-
-
15444363304
-
NR2A and NR2B receptor gene variations modify age at onset in Huntington disease
-
Arning L, Kraus PH, Valentin S, Saft C, Andrich J, Epplen JT : NR2A and NR2B receptor gene variations modify age at onset in Huntington disease. Neurogenetics 2005 ; 6 : 25-8
-
(2005)
Neurogenetics
, vol.6
, pp. 25-28
-
-
Arning, L.1
Kraus, P.H.2
Valentin, S.3
Saft, C.4
Andrich, J.5
Epplen, J.T.6
-
24
-
-
0032867615
-
Evidence for the GluR6 gene associated with younger onset age of Huntington's disease
-
MacDonald ME, Vonsattel JP, Shrinidhi J et al. : Evidence for the GluR6 gene associated with younger onset age of Huntington's disease. Neurology 199 ; 53 : 1330-2
-
Neurology
, vol.199
, Issue.53
, pp. 1330-1332
-
-
MacDonald, M.E.1
Vonsattel, J.P.2
Shrinidhi, J.3
-
25
-
-
25444453003
-
Association between BDNF Val66Met polymorphism and age at onset in Huntington disease
-
Alberch J, Lopez M, Badenas C et al. : Association between BDNF Val66Met polymorphism and age at onset in Huntington disease. Neurology 2005 : 964-6
-
(2005)
Neurology
, pp. 964-966
-
-
Alberch, J.1
Lopez, M.2
Badenas, C.3
-
26
-
-
33750209146
-
No evidence of association between BDNF gene variants and age-at-onset of Huntington's disease
-
Di Maria E, Marasco A, Tartari M et al. : No evidence of association between BDNF gene variants and age-at-onset of Huntington's disease. Neurobiol Dis 2006 ; 24 : 274-9
-
(2006)
Neurobiol Dis
, vol.24
, pp. 274-279
-
-
Di Maria, E.1
Marasco, A.2
Tartari, M.3
-
27
-
-
33749988016
-
Brain-derived neurotrophic factor does not influence age at neurologic onset of Huntington's disease
-
Kishikawa S, Li JL, Gillis T et al. : Brain-derived neurotrophic factor does not influence age at neurologic onset of Huntington's disease. Neurobiol Dis 2006 ; 24 : 280-5
-
(2006)
Neurobiol Dis
, vol.24
, pp. 280-285
-
-
Kishikawa, S.1
Li, J.L.2
Gillis, T.3
-
28
-
-
0038042172
-
Modulation of age at onset in Huntington's disease and spinocerebellar ataxia type 2 patients originated from eastern India
-
Chattopadhyay B, Ghosh S, Gangopadhyay PK et al. : Modulation of age at onset in Huntington's disease and spinocerebellar ataxia type 2 patients originated from eastern India. Neurosci Lett 2003 ; 345 : 93-6
-
(2003)
Neurosci Lett
, vol.345
, pp. 93-96
-
-
Chattopadhyay, B.1
Ghosh, S.2
Gangopadhyay, P.K.3
-
29
-
-
33644658207
-
The S18Y polymorphism in the UCHL1 gene is a genetic modifier in Huntington's disease
-
Metzger S, Bauer R Tomiuk J et al. : The S18Y polymorphism in the UCHL1 gene is a genetic modifier in Huntington's disease. Neurogenetics 2006 : 27-30
-
(2006)
Neurogenetics
, pp. 27-30
-
-
Metzger, S.1
Bauer, R.2
Tomiuk, J.3
-
30
-
-
11844250016
-
Modulation of age at onset of Huntington disease patients by variations in TP53 and human caspase activated DNase (hCAD) genes
-
Chattopadhyay B, Baksi K, Mukhopadhyay S, Bhattacharyya NP : Modulation of age at onset of Huntington disease patients by variations in TP53 and human caspase activated DNase (hCAD) genes. Neurosci Lett 2005 ; 374 : 81-6
-
(2005)
Neurosci Lett
, vol.374
, pp. 81-86
-
-
Chattopadhyay, B.1
Baksi, K.2
Mukhopadhyay, S.3
Bhattacharyya, N.P.4
-
31
-
-
0029055601
-
Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form
-
Trottier Y, Devys D, Imbert G et al. : Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form. Nat Genet 1995 ; 10 : 104-10
-
(1995)
Nat Genet
, vol.10
, pp. 104-110
-
-
Trottier, Y.1
Devys, D.2
Imbert, G.3
-
32
-
-
0030950980
-
Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum
-
Ferrante RJ, Gutekunst CA, Persichetti F et al. : Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum. J Neurosci 1997 ; 17 : 3052-63
-
(1997)
J Neurosci
, vol.17
, pp. 3052-3063
-
-
Ferrante, R.J.1
Gutekunst, C.A.2
Persichetti, F.3
-
33
-
-
15144351709
-
Differential distribution of the normal and mutated forms of huntingtin in the human brain
-
Gourfinkel-An I, Cancel G, Trottier Y et al. : Differential distribution of the normal and mutated forms of huntingtin in the human brain. Ann Neurol 1997 ; 42 : 712-9
-
(1997)
Ann Neurol
, vol.42
, pp. 712-719
-
-
Gourfinkel-An, I.1
Cancel, G.2
Trottier, Y.3
-
34
-
-
0028829596
-
CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain
-
Aronin N, Chase K, Young C et al. : CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain. Neuron 1995 ; 15 : 1193-201
-
(1995)
Neuron
, vol.15
, pp. 1193-1201
-
-
Aronin, N.1
Chase, K.2
Young, C.3
-
35
-
-
0028891145
-
Huntington's disease gene : Regional and cellular expression in brain of normal and affected individuals
-
Landwehrmeyer GB, McNeil SM, Dure LS et al. : Huntington's disease gene : regional and cellular expression in brain of normal and affected individuals. Ann Neurol 1995 ; 37 : 218-30
-
(1995)
Ann Neurol
, vol.37
, pp. 218-230
-
-
Landwehrmeyer, G.B.1
McNeil, S.M.2
Dure, L.S.3
-
36
-
-
0041656292
-
The hunt for huntingtin function : Interaction partners tell many different stories
-
Harjes P, Wanker EE : The hunt for huntingtin function : interaction partners tell many different stories. Trends Biochem Sci 2003 ; 28 : 425-33
-
(2003)
Trends Biochem Sci
, vol.28
, pp. 425-433
-
-
Harjes, P.1
Wanker, E.E.2
-
37
-
-
0035816627
-
Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95
-
Sun Y, Savanenin A, Reddy PH, Liu YF : Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem 2001 ; 276 : 24713-8
-
(2001)
J Biol Chem
, vol.276
, pp. 24713-24718
-
-
Sun, Y.1
Savanenin, A.2
Reddy, P.H.3
Liu, Y.F.4
-
38
-
-
3142636768
-
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
-
Gauthier LR, Charrin BC, Borrell-Pages 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-Pages, M.3
-
39
-
-
0034657112
-
Wild-type huntingtin protects from apoptosis upstream of caspase-3
-
Rigamonti D, Bauer JH, De Fraja C et al. : Wild-type huntingtin protects from apoptosis upstream of caspase-3. J Neurosci 2000 ; 20 : 3705-13
-
(2000)
J Neurosci
, vol.20
, pp. 3705-3713
-
-
Rigamonti, D.1
Bauer, J.H.2
De Fraja, C.3
-
40
-
-
0035805504
-
Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing
-
Rigamonti D, Sipione S, Goffredo D, Zuccato C, Fossale E, Cattaneo E : Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing. J Biol Chem 2001 ; 276 : 14545-8
-
(2001)
J Biol Chem
, vol.276
, pp. 14545-14548
-
-
Rigamonti, D.1
Sipione, S.2
Goffredo, D.3
Zuccato, C.4
Fossale, E.5
Cattaneo, E.6
-
41
-
-
33845720388
-
-
Zhang Y, Leavitt BR, van Raamsdonk JM et al. : Huntingtin inhibits caspase-3 activation. EMBO J 2006 ; sous presse
-
Zhang Y, Leavitt BR, van Raamsdonk JM et al. : Huntingtin inhibits caspase-3 activation. EMBO J 2006 ; sous presse
-
-
-
-
42
-
-
0029082383
-
-
Duyao MP, Auerbach AB, Ryan A et al. : Inactivation of the mouse Huntington's disease gene homolog Hdh. Science 1995 ; 269 : 407-10
-
Duyao MP, Auerbach AB, Ryan A et al. : Inactivation of the mouse Huntington's disease gene homolog Hdh. Science 1995 ; 269 : 407-10
-
-
-
-
43
-
-
0029055717
-
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
-
Nasir J, Floresco SB, O'Kusky JR et al. : Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 1995 ; 81 : 811-23
-
(1995)
Cell
, vol.81
, pp. 811-823
-
-
Nasir, J.1
Floresco, S.B.2
O'Kusky, J.R.3
-
44
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
-
Zeitlin S, Liu JP, Chapman DL, Papaioannou VE, Efstratiadis A : Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nat Genet 1995 ; 11 : 155-63
-
(1995)
Nat Genet
, vol.11
, pp. 155-163
-
-
Zeitlin, S.1
Liu, J.P.2
Chapman, D.L.3
Papaioannou, V.E.4
Efstratiadis, A.5
-
45
-
-
0033757718
-
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
-
Dragatsis I, Levine MS, Zeitlin S : Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet 2000 ; 26 : 300-6
-
(2000)
Nat Genet
, vol.26
, pp. 300-306
-
-
Dragatsis, I.1
Levine, M.S.2
Zeitlin, S.3
-
46
-
-
0035919701
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
-
Zuccato C, Ciammola A, Rigamonti D et al. : Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 2001 ; 293 : 493-8
-
(2001)
Science
, vol.293
, pp. 493-498
-
-
Zuccato, C.1
Ciammola, A.2
Rigamonti, D.3
-
47
-
-
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
-
48
-
-
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
-
49
-
-
0037292586
-
Dysfunction of wild-type huntingtin in Huntington disease
-
Cattaneo E : Dysfunction of wild-type huntingtin in Huntington disease. News Physiol Sci 2003 ; 18 : 34-7
-
(2003)
News Physiol Sci
, vol.18
, pp. 34-37
-
-
Cattaneo, E.1
-
50
-
-
29644433445
-
Selective Degeneration and Nuclear Localization of Mutant Huntingtin in the YAC128 Mouse Model of Huntington Disease
-
Van Raamsdonk JM, Murphy Z, Slow EJ, Leavitt BR, Hayden MR : Selective Degeneration and Nuclear Localization of Mutant Huntingtin in the YAC128 Mouse Model of Huntington Disease. Hum Mol Genet 2005 ; 14 : 3823-35
-
(2005)
Hum Mol Genet
, vol.14
, pp. 3823-3835
-
-
Van Raamsdonk, J.M.1
Murphy, Z.2
Slow, E.J.3
Leavitt, B.R.4
Hayden, M.R.5
-
51
-
-
1242338856
-
-
Li SH, Li XJ : Huntingtin-protein interactions and the pathogenesis of Huntington's disease. Trends Genet 2004 ; 3 : 146-54
-
Li SH, Li XJ : Huntingtin-protein interactions and the pathogenesis of Huntington's disease. Trends Genet 2004 ; 3 : 146-54
-
-
-
-
52
-
-
0032912386
-
Recent advances on the pathogensis of Huntington's disease
-
Petersen A, Mani K, Brundin P : Recent advances on the pathogensis of Huntington's disease. Exp Neurol 1999 ; 157 : 1-18
-
(1999)
Exp Neurol
, vol.157
, pp. 1-18
-
-
Petersen, A.1
Mani, K.2
Brundin, P.3
-
53
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
Saudou F, Finkbeiner S, Devys D, Greenberg ME : Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998 ; 95 : 55-66
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
54
-
-
0032248397
-
Pathological mechanisms in Huntingtion's disease and other polyglutamine expansion diseases
-
Lunkes A, Trottier Y, Mandel JL : Pathological mechanisms in Huntingtion's disease and other polyglutamine expansion diseases. Essays Biochem 1998 ; 33 : 149-63
-
(1998)
Essays Biochem
, vol.33
, pp. 149-163
-
-
Lunkes, A.1
Trottier, Y.2
Mandel, J.L.3
-
55
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia M, Sapp E, Chase KO et al. : Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997 ; 277 : 1990-3
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
-
56
-
-
0032987513
-
Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease
-
Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, DiFiglia M : Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. J Neuropathol Exp Neurol 1999 ; 58 : 165-73
-
(1999)
J Neuropathol Exp Neurol
, vol.58
, pp. 165-173
-
-
Sapp, E.1
Penney, J.2
Young, A.3
Aronin, N.4
Vonsattel, J.P.5
DiFiglia, M.6
-
58
-
-
85177152626
-
-
Peruttz MF, Johnson T, Suzuki M, Fich JT : Glutamine repeats as polar zippers : their possible role in inherited neurodegenerative diseases. Proc Natl Acad Sci U S A 1994 ; 91 : 5355-8
-
Peruttz MF, Johnson T, Suzuki M, Fich JT : Glutamine repeats as polar zippers : their possible role in inherited neurodegenerative diseases. Proc Natl Acad Sci U S A 1994 ; 91 : 5355-8
-
-
-
-
59
-
-
0029059477
-
Incorporation of glutamine repeats makes protein oligomerize : Implications for neurodegenerative diseases
-
Stott K, Blackburn JM, Butler PJG, Perutz M : Incorporation of glutamine repeats makes protein oligomerize : implications for neurodegenerative diseases. Proc Natl Acad Sci U S A 1995 ; 92 : 6509-13
-
(1995)
Proc Natl Acad Sci U S A
, vol.92
, pp. 6509-6513
-
-
Stott, K.1
Blackburn, J.M.2
Butler, P.J.G.3
Perutz, M.4
-
60
-
-
0036172346
-
Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine
-
Karpuj MV, Becher MW, Springer JE et al. : Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat Med 2002 ; 8 : 143-9
-
(2002)
Nat Med
, vol.8
, pp. 143-149
-
-
Karpuj, M.V.1
Becher, M.W.2
Springer, J.E.3
-
61
-
-
0141493679
-
Huntingtin proteolysis in Huntington's disease
-
Wellington CL, Ellerby LM, Leavitt B, Roy S, Nicholson DW, Hayden MR : Huntingtin proteolysis in Huntington's disease. Clin Neurosci Res 2003 ; 3 : 129-39
-
(2003)
Clin Neurosci Res
, vol.3
, pp. 129-139
-
-
Wellington, C.L.1
Ellerby, L.M.2
Leavitt, B.3
Roy, S.4
Nicholson, D.W.5
Hayden, M.R.6
-
62
-
-
13444273499
-
Development of novel therapies for Huntington's disease : Hope and challenge
-
Qin ZH, Wang J, Gu ZL : Development of novel therapies for Huntington's disease : hope and challenge. Acta Pharmacol Sin 2005 ; 26 : 129-42
-
(2005)
Acta Pharmacol Sin
, vol.26
, pp. 129-142
-
-
Qin, Z.H.1
Wang, J.2
Gu, Z.L.3
-
63
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease : Relationship to neuropathology
-
Gutekunst CA, Li SH, Yi H et al. : Nuclear and neuropil aggregates in Huntington's disease : relationship to neuropathology. J Neurosci 1999 ; 19 : 2522-34
-
(1999)
J Neurosci
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
-
64
-
-
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
-
65
-
-
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
-
66
-
-
3042717240
-
Cellular toxicity of polyglutamine expansion proteins : Mechanism of transcription factor deactivation
-
Schaffar G, Breuer P, Boteva R et al. : Cellular toxicity of polyglutamine expansion proteins : mechanism of transcription factor deactivation. Mol Cell 2004 ; 15 : 95-105
-
(2004)
Mol Cell
, vol.15
, pp. 95-105
-
-
Schaffar, G.1
Breuer, P.2
Boteva, R.3
-
67
-
-
0032590053
-
Huntington aggregates may not predict neuronal death in Huntington's disease
-
Kuemmerle S, Gutekunst CA, Klein AM et al. : Huntington aggregates may not predict neuronal death in Huntington's disease. Ann Neurol 1999 ; 46 : 842-9
-
(1999)
Ann Neurol
, vol.46
, pp. 842-849
-
-
Kuemmerle, S.1
Gutekunst, C.A.2
Klein, A.M.3
-
68
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S : Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004 ; 431 : 805-10
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
69
-
-
23844472610
-
-
Slow EJ, Graham RK, Osmand AP et al. : Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A 2005 ; 102 : 11402-7
-
Slow EJ, Graham RK, Osmand AP et al. : Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A 2005 ; 102 : 11402-7
-
-
-
-
70
-
-
0029926199
-
Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease : A detailed cellular in situ hybridization study
-
Augood SJ, Faull RL, Love DR, Emson PC : Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease : a detailed cellular in situ hybridization study. Neuroscience 1996 ; 72 : 1023-36
-
(1996)
Neuroscience
, vol.72
, pp. 1023-1036
-
-
Augood, S.J.1
Faull, R.L.2
Love, D.R.3
Emson, P.C.4
-
71
-
-
0029895292
-
Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease
-
Weeks RA, Piccini P, Harding AE, Brooks DJ : Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease. Ann Neurol 1996 ; 40 : 49-54
-
(1996)
Ann Neurol
, vol.40
, pp. 49-54
-
-
Weeks, R.A.1
Piccini, P.2
Harding, A.E.3
Brooks, D.J.4
-
72
-
-
0030612119
-
Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease
-
Augood SJ, Faull RL, Emson PC : Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease. Ann Neurol 1997 ; 42 : 215-21
-
(1997)
Ann Neurol
, vol.42
, pp. 215-221
-
-
Augood, S.J.1
Faull, R.L.2
Emson, P.C.3
-
73
-
-
0032568517
-
-
Cha JH, Kosinski CM, Kerner JA et al. : Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc Natl Acad Sci U S A 1998 ; 95 : 6480-5
-
Cha JH, Kosinski CM, Kerner JA et al. : Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc Natl Acad Sci U S A 1998 ; 95 : 6480-5
-
-
-
-
74
-
-
0033614761
-
Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease
-
Cha JH, Frey AS, Alsdorf SA et al. : Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 1999 ; 354 : 981-9
-
(1999)
Philos Trans R Soc Lond B Biol Sci
, vol.354
, pp. 981-989
-
-
Cha, J.H.1
Frey, A.S.2
Alsdorf, S.A.3
-
75
-
-
0034612255
-
-
Bibb JA, Yan Z, Svenningsson P et al. : Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice. Proc Natl Acad Sci U S A 2000 ; 97 : 6809-14
-
Bibb JA, Yan Z, Svenningsson P et al. : Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice. Proc Natl Acad Sci U S A 2000 ; 97 : 6809-14
-
-
-
-
76
-
-
0034047444
-
Cannabinoid receptor messenger RNA levels decrease in a subset of neurons of the lateral striatum, cortex and hippocampus of transgenic Huntington's disease mice
-
Denovan-Wright EM, Robertson HA : Cannabinoid receptor messenger RNA levels decrease in a subset of neurons of the lateral striatum, cortex and hippocampus of transgenic Huntington's disease mice. Neuroscience 2000 ; 98 : 705-13
-
(2000)
Neuroscience
, vol.98
, pp. 705-713
-
-
Denovan-Wright, E.M.1
Robertson, H.A.2
-
77
-
-
0037101835
-
Dysregulation of gene expression in the R6/2 model of polyglutamine disease : Parallel changes in muscle and brain
-
Luthi-Carter R, Hanson SA, Strand AD et al. : Dysregulation of gene expression in the R6/2 model of polyglutamine disease : parallel changes in muscle and brain. Hum Mol Genet 2002 ; 11 : 1911-26
-
(2002)
Hum Mol Genet
, vol.11
, pp. 1911-1926
-
-
Luthi-Carter, R.1
Hanson, S.A.2
Strand, A.D.3
-
78
-
-
0037097287
-
differential D1 and D2 receptor-mediated effects on immediate early gene induction in a transgenic mouse model of Huntington's disease
-
Spektor BS, Miller DW, Hollingsworth ZR et al. : differential D1 and D2 receptor-mediated effects on immediate early gene induction in a transgenic mouse model of Huntington's disease. Brain Res Mol Brain Res 2002 ; 102 : 118-28
-
(2002)
Brain Res Mol Brain Res
, vol.102
, pp. 118-128
-
-
Spektor, B.S.1
Miller, D.W.2
Hollingsworth, Z.R.3
-
79
-
-
0037101837
-
Polyglutamine and transcription : Gene expression changes shared by DRPLA and Huntington's disease mouse model reveal context-dependent independent-effects
-
Luthi-Carter R, Strand AD, Hanson SA et al. : Polyglutamine and transcription : gene expression changes shared by DRPLA and Huntington's disease mouse model reveal context-dependent independent-effects. Hum Mol Genet 2002 ; 11 : 1927-37
-
(2002)
Hum Mol Genet
, vol.11
, pp. 1927-1937
-
-
Luthi-Carter, R.1
Strand, A.D.2
Hanson, S.A.3
-
80
-
-
0141828353
-
Mechanisms of transcriptional dysregulation in Huntington's disease
-
Luthi-Carter R, Cha JHJ : Mechanisms of transcriptional dysregulation in Huntington's disease. Clinical Neuroscience Research 2003 ; 3 : 165-77
-
(2003)
Clinical Neuroscience Research
, vol.3
, pp. 165-177
-
-
Luthi-Carter, R.1
Cha, J.H.J.2
-
81
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity
-
Nucifora FC Jr, Sasaki M, Peters MF et al. : Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 2001 ; 291 : 2423-8
-
(2001)
Science
, vol.291
, pp. 2423-2428
-
-
Nucifora Jr, F.C.1
Sasaki, M.2
Peters, M.F.3
-
82
-
-
12244298155
-
Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein
-
Jiang H, Nucifora FC Jr, Ross CA, DeFranco DB : Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein. Hum Mol Genet 2003 ; 12 : 1-12
-
(2003)
Hum Mol Genet
, vol.12
, pp. 1-12
-
-
Jiang, H.1
Nucifora Jr, F.C.2
Ross, C.A.3
DeFranco, D.B.4
-
83
-
-
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
-
84
-
-
0036578784
-
Disruption of CREB function in brain leads to neurodegeneration
-
Mantamadiotis T, Lemberger T, Bleckmann SC et al. : Disruption of CREB function in brain leads to neurodegeneration. Nat Genet 2002 ; 31 : 47-54
-
(2002)
Nat Genet
, vol.31
, pp. 47-54
-
-
Mantamadiotis, T.1
Lemberger, T.2
Bleckmann, S.C.3
-
85
-
-
29244462838
-
In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets
-
Zhai W, Jeong H, Cui L, Krainc D, Tjian R : In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Cell 2005 ; 123 : 1241-53
-
(2005)
Cell
, vol.123
, pp. 1241-1253
-
-
Zhai, W.1
Jeong, H.2
Cui, L.3
Krainc, D.4
Tjian, R.5
-
86
-
-
0037198698
-
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration
-
LaMonte BH, Wallace KE, Holloway BA et al. : Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration. Neuron 2002 ; 34 : 715-27
-
(2002)
Neuron
, vol.34
, pp. 715-727
-
-
LaMonte, B.H.1
Wallace, K.E.2
Holloway, B.A.3
-
87
-
-
0042512291
-
Disruption of cellular transport : A common cause of neurodegeneration ?
-
Crosby AH : Disruption of cellular transport : a common cause of neurodegeneration ? Lancet Neurol 2003 ; 2 : 311-6
-
(2003)
Lancet Neurol
, vol.2
, pp. 311-316
-
-
Crosby, A.H.1
-
88
-
-
17844378486
-
Axonal transport failure in neurodegenerative disorders : The case of Huntington's disease
-
Charrin BC, Saudou F, Humbert S : Axonal transport failure in neurodegenerative disorders : the case of Huntington's disease. Pathol Biol (Paris) 2005 ; 53 : 189-92
-
(2005)
Pathol Biol (Paris)
, vol.53
, pp. 189-192
-
-
Charrin, B.C.1
Saudou, F.2
Humbert, S.3
-
89
-
-
0028989602
-
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
-
DiFiglia M, Sapp E, Chase K et al. : Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 1995 ; 14 : 1075-81
-
(1995)
Neuron
, vol.14
, pp. 1075-1081
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.3
-
90
-
-
0030878098
-
Fast transport and retrograde movement of huntingtin and HAP1 in axons
-
Block-Galarza J, Chase KO, Sapp E et al. : Fast transport and retrograde movement of huntingtin and HAP1 in axons. Neuroreport 1997 ; 8 : 2247-51
-
(1997)
Neuroreport
, vol.8
, pp. 2247-2251
-
-
Block-Galarza, J.1
Chase, K.O.2
Sapp, E.3
-
91
-
-
85177155795
-
-
Gutekunst CA, Levey AI, Heilman CJ et al. : Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies. Proc Natl Acad Sci U S A 1995 ; 92 : 8710-4
-
Gutekunst CA, Levey AI, Heilman CJ et al. : Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies. Proc Natl Acad Sci U S A 1995 ; 92 : 8710-4
-
-
-
-
92
-
-
0030986659
-
HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain
-
Kalchman MA, Koide HB, McCutcheon K et al. : HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain. Nat Genet 1997 ; 16 : 44-53
-
(1997)
Nat Genet
, vol.16
, pp. 44-53
-
-
Kalchman, M.A.1
Koide, H.B.2
McCutcheon, K.3
-
93
-
-
0031056478
-
HIP-I : A huntingtin interacting protein isolated by the yeast two-hybrid system
-
Wanker EE, Rovira C, Scherzinger E et al. : HIP-I : a huntingtin interacting protein isolated by the yeast two-hybrid system. Hum Mol Genet 1997 ; 6 : 487-95
-
(1997)
Hum Mol Genet
, vol.6
, pp. 487-495
-
-
Wanker, E.E.1
Rovira, C.2
Scherzinger, E.3
-
94
-
-
0036500862
-
Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin : Relevance to Huntington's disease
-
Hoffner G, Kahlem P, Djian P : Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin : relevance to Huntington's disease. J Cell Sci 2002 ; 115 : 941-8
-
(2002)
J Cell Sci
, vol.115
, pp. 941-948
-
-
Hoffner, G.1
Kahlem, P.2
Djian, P.3
-
95
-
-
0036796261
-
PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
-
Modregger J, DiProspero NA, Charles V, Tagle DA, Plomann M : PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains. Hum Mol Genet 2002 ; 11 : 2547-58
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2547-2558
-
-
Modregger, J.1
DiProspero, N.A.2
Charles, V.3
Tagle, D.A.4
Plomann, M.5
-
96
-
-
0036850524
-
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis
-
Singaraja RR, Hadano S, Metzler M et al. : HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet 2002 ; 11 : 2815-28
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2815-2828
-
-
Singaraja, R.R.1
Hadano, S.2
Metzler, M.3
-
97
-
-
0141750470
-
Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila
-
Gunawardena S, Her LS, Brusch RG et al. : Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. Neuron 2003 ; 40 : 25-40
-
(2003)
Neuron
, vol.40
, pp. 25-40
-
-
Gunawardena, S.1
Her, L.S.2
Brusch, R.G.3
-
98
-
-
0842269058
-
Cargo-carrying motor vehicles on the neuronal highway : Transport pathways and neurodegenerative disease
-
Gunawardena S, Goldstein LS : Cargo-carrying motor vehicles on the neuronal highway : transport pathways and neurodegenerative disease. J Neurobiol 2004 ; 58 : 258-71
-
(2004)
J Neurobiol
, vol.58
, pp. 258-271
-
-
Gunawardena, S.1
Goldstein, L.S.2
-
99
-
-
0034426013
-
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
-
Li H, Li SH, Johnston H, Shelbourne PF, Li XJ : Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nat Genet 2000 ; 25 : 385-9
-
(2000)
Nat Genet
, vol.25
, pp. 385-389
-
-
Li, H.1
Li, S.H.2
Johnston, H.3
Shelbourne, P.F.4
Li, X.J.5
-
100
-
-
10744224530
-
Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport
-
Szebenyi G, Morfini GA, Babcock A et al. : Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport. Neuron 2003 ; 40 : 41-52
-
(2003)
Neuron
, vol.40
, pp. 41-52
-
-
Szebenyi, G.1
Morfini, G.A.2
Babcock, A.3
-
101
-
-
1542267796
-
-
Lee WC, Yoshihara M, Littleton JT : Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc Natl Acad Sci U S A 2004 ; 101 : 3224-9
-
Lee WC, Yoshihara M, Littleton JT : Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc Natl Acad Sci U S A 2004 ; 101 : 3224-9
-
-
-
-
102
-
-
4444316194
-
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro
-
Trushina E, Dyer RB, Badger JD et al. : Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol 2004 ; 24 : 8195-209
-
(2004)
Mol Cell Biol
, vol.24
, pp. 8195-8209
-
-
Trushina, E.1
Dyer, R.B.2
Badger, J.D.3
-
103
-
-
0344413576
-
Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington disease
-
Li L, Fan M, Icton CD et al. : Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington disease. Neurobiol Aging 2003 ; 24 : 1113-21
-
(2003)
Neurobiol Aging
, vol.24
, pp. 1113-1121
-
-
Li, L.1
Fan, M.2
Icton, C.D.3
-
104
-
-
0035158533
-
Progressive depletion of complexin II in a transgenic mouse model of Huntington's disease
-
Morton AJ, Edwardson JM : Progressive depletion of complexin II in a transgenic mouse model of Huntington's disease. J Neurochem 2001 ; 76 : 166-72
-
(2001)
J Neurochem
, vol.76
, pp. 166-172
-
-
Morton, A.J.1
Edwardson, J.M.2
-
105
-
-
0036173770
-
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi
-
Gervais FG, Singaraja R, Xanthoudakis S et al. : Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol 2002 ; 4 : 95-105
-
(2002)
Nat Cell Biol
, vol.4
, pp. 95-105
-
-
Gervais, F.G.1
Singaraja, R.2
Xanthoudakis, S.3
-
106
-
-
0037150687
-
Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease
-
Dunah AW, Jeong H, Griffin A, Kim YM, Standaert DG, Hersch SM : Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 2002 ; 296 : 2238-43
-
(2002)
Science
, vol.296
, pp. 2238-2243
-
-
Dunah, A.W.1
Jeong, H.2
Griffin, A.3
Kim, Y.M.4
Standaert, D.G.5
Hersch, S.M.6
-
107
-
-
0642334395
-
Abberant A2A receptor function in peripheral blood cells in Huntington's disease
-
Varani K, Abbracchio MP, Cannella M et al. : Abberant A2A receptor function in peripheral blood cells in Huntington's disease. FASEB J 2003 ; 17 : 2148-50
-
(2003)
FASEB J
, vol.17
, pp. 2148-2150
-
-
Varani, K.1
Abbracchio, M.P.2
Cannella, M.3
-
108
-
-
33745134073
-
Eraly and transient alteration of A2A receptor signalling in a mouse mode of Huntington's disease
-
Tarditi A, Camurri A, Varani K et al. : Eraly and transient alteration of A2A receptor signalling in a mouse mode of Huntington's disease. Neurobiol Dis 2006 ; 23 : 44-53
-
(2006)
Neurobiol Dis
, vol.23
, pp. 44-53
-
-
Tarditi, A.1
Camurri, A.2
Varani, K.3
-
109
-
-
0035816627
-
Polyglutamine-expanded huntingtin promotes sensitization of NMDA receptors via post-synaptic density 95
-
Sun Y, Savanenin A, Reddy PH, Liu YF : Polyglutamine-expanded huntingtin promotes sensitization of NMDA receptors via post-synaptic density 95. J Biol Chem 2001 ; 276 : 24713-8
-
(2001)
J Biol Chem
, vol.276
, pp. 24713-24718
-
-
Sun, Y.1
Savanenin, A.2
Reddy, P.H.3
Liu, Y.F.4
-
110
-
-
0019991784
-
Cerebral metabolism and atrophy in Huntington's disease determined by 18FDG and computed tomographic scan
-
Kuhl DE, Phelps ME, Markham CH, Metter EJ, Riege WH, Winter J : Cerebral metabolism and atrophy in Huntington's disease determined by 18FDG and computed tomographic scan. Ann Neurol 1982 ; 12 : 425-34
-
(1982)
Ann Neurol
, vol.12
, pp. 425-434
-
-
Kuhl, D.E.1
Phelps, M.E.2
Markham, C.H.3
Metter, E.J.4
Riege, W.H.5
Winter, J.6
-
111
-
-
0022641113
-
Positron emission tomography in the early diagnosis of Huntington's disease
-
Hayden MR, Martin WR, Stoessl AJ et al. : Positron emission tomography in the early diagnosis of Huntington's disease. Neurology 1986 ; 36 : 888-94
-
(1986)
Neurology
, vol.36
, pp. 888-894
-
-
Hayden, M.R.1
Martin, W.R.2
Stoessl, A.J.3
-
112
-
-
0023147905
-
Reduced cerebral glucose metabolism in asymptomatic subjects at risk for Huntington's disease
-
Mazziotta JC, Phelps ME, Pahl JJ et al. : Reduced cerebral glucose metabolism in asymptomatic subjects at risk for Huntington's disease. N Engl J Med 1987 ; 316 : 357-62
-
(1987)
N Engl J Med
, vol.316
, pp. 357-362
-
-
Mazziotta, J.C.1
Phelps, M.E.2
Pahl, J.J.3
-
113
-
-
0025009430
-
A comparison of neurological, metabolic, structural, and genetic evaluations in persons at risk for Huntington's disease
-
Grafton ST, Mazziotta JC, Pahl JJ et al. : A comparison of neurological, metabolic, structural, and genetic evaluations in persons at risk for Huntington's disease. Ann Neurol 1990 ; 28 : 614-21
-
(1990)
Ann Neurol
, vol.28
, pp. 614-621
-
-
Grafton, S.T.1
Mazziotta, J.C.2
Pahl, J.J.3
-
114
-
-
0027741301
-
Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy
-
Jenkins BG, Koroshetz WJ, Beal MF, Rosen BR : Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy. Neurology 1993 ; 43 : 2689-95
-
(1993)
Neurology
, vol.43
, pp. 2689-2695
-
-
Jenkins, B.G.1
Koroshetz, W.J.2
Beal, M.F.3
Rosen, B.R.4
-
115
-
-
0031044805
-
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
-
Koroshetz WJ, Jenkins BG, Rosen BR, Beal MF : Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol 1997 ; 41 : 160-5
-
(1997)
Ann Neurol
, vol.41
, pp. 160-165
-
-
Koroshetz, W.J.1
Jenkins, B.G.2
Rosen, B.R.3
Beal, M.F.4
-
116
-
-
0031596998
-
1H NMR spectroscopy studies of Huntington's disease : Correlations with CAG repeat numbers
-
Jenkins BG, Rosas HD, Chen YC et al. : 1H NMR spectroscopy studies of Huntington's disease : correlations with CAG repeat numbers. Neurology 1998 ; 50 : 1357-65
-
(1998)
Neurology
, vol.50
, pp. 1357-1365
-
-
Jenkins, B.G.1
Rosas, H.D.2
Chen, Y.C.3
-
117
-
-
0033914747
-
Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropa-llidoluysian atrophy
-
Lodi R, Schapira AH, Manners D et al. : Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropa-llidoluysian atrophy. Ann Neurol 2000 ; 48 : 72-6
-
(2000)
Ann Neurol
, vol.48
, pp. 72-76
-
-
Lodi, R.1
Schapira, A.H.2
Manners, D.3
-
118
-
-
22844440902
-
Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease
-
Saft C, Zange J, Andrich J et al. : Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease. Mov Disord 2005 ; 20 : 674-9
-
(2005)
Mov Disord
, vol.20
, pp. 674-679
-
-
Saft, C.1
Zange, J.2
Andrich, J.3
-
119
-
-
0029875381
-
Mitochondrial defect in Huntington's disease caudate nucleus
-
Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AH : Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol 1996 ; 39 : 385-9
-
(1996)
Ann Neurol
, vol.39
, pp. 385-389
-
-
Gu, M.1
Gash, M.T.2
Mann, V.M.3
Javoy-Agid, F.4
Cooper, J.M.5
Schapira, A.H.6
-
120
-
-
0030919567
-
Oxidative damage and metabolic dysfunction in Huntington's disease : Selective vulnerability of the basal ganglia
-
Browne SE, Bowling AC, MacGarvey U et al. : Oxidative damage and metabolic dysfunction in Huntington's disease : selective vulnerability of the basal ganglia. Ann Neurol 1997 ; 41 : 646-53
-
(1997)
Ann Neurol
, vol.41
, pp. 646-653
-
-
Browne, S.E.1
Bowling, A.C.2
MacGarvey, U.3
-
121
-
-
0032900574
-
Biochemical abnormalities and excitotoxicity in Huntington's disease brain
-
Tabrizi SJ, Cleeter MW, Xuereb J, Taanman JW, Cooper JM, Schapira AH : Biochemical abnormalities and excitotoxicity in Huntington's disease brain. Ann Neurol 1999 ; 45 : 25-32
-
(1999)
Ann Neurol
, vol.45
, pp. 25-32
-
-
Tabrizi, S.J.1
Cleeter, M.W.2
Xuereb, J.3
Taanman, J.W.4
Cooper, J.M.5
Schapira, A.H.6
-
122
-
-
33745392939
-
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin
-
Benchoua A, Trioulier Y, Zala D et al. : Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell 2006 ; 17 : 1652-63
-
(2006)
Mol Biol Cell
, vol.17
, pp. 1652-1663
-
-
Benchoua, A.1
Trioulier, Y.2
Zala, D.3
-
123
-
-
29144468251
-
3-Nitropropionic acid : A mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease
-
Brouillet E, Jacquard C, Bizat N, Blum D : 3-Nitropropionic acid : a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease. J Neurochem 2005 ; 95 : 1521-40
-
(2005)
J Neurochem
, vol.95
, pp. 1521-1540
-
-
Brouillet, E.1
Jacquard, C.2
Bizat, N.3
Blum, D.4
-
124
-
-
0032851595
-
Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization
-
Sawa A, Wiegand GW, Cooper J et al. : Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization. Nat Med 1999 ; 5 : 1194-8
-
(1999)
Nat Med
, vol.5
, pp. 1194-1198
-
-
Sawa, A.1
Wiegand, G.W.2
Cooper, J.3
-
125
-
-
0036327065
-
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines
-
Panov AV, Gutekunst CA, Leavitt BR et al. : Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci 2002 ; 5 : 731-6
-
(2002)
Nat Neurosci
, vol.5
, pp. 731-736
-
-
Panov, A.V.1
Gutekunst, C.A.2
Leavitt, B.R.3
-
126
-
-
3543141113
-
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
-
Choo YS, Johnson GV, MacDonald M, Detloff PJ, Lesort M : Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet 2004 ; 13 : 1407-20
-
(2004)
Hum Mol Genet
, vol.13
, pp. 1407-1420
-
-
Choo, Y.S.1
Johnson, G.V.2
MacDonald, M.3
Detloff, P.J.4
Lesort, M.5
-
127
-
-
1842533573
-
Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway
-
Ruan Q, Lesort M, MacDonald ME, Johnson GV : Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway. Hum Mol Genet 2004 ; 13 : 669-81
-
(2004)
Hum Mol Genet
, vol.13
, pp. 669-681
-
-
Ruan, Q.1
Lesort, M.2
MacDonald, M.E.3
Johnson, G.V.4
-
128
-
-
21544450545
-
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
-
Bae BI, Xu H, Igarashi S et al. : p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron 2005 ; 47 : 29-41
-
(2005)
Neuron
, vol.47
, pp. 29-41
-
-
Bae, B.I.1
Xu, H.2
Igarashi, S.3
-
129
-
-
0014682523
-
Brain lesions in an infant rhesus monkey treated with monsodium glutamate
-
Olney JW, Sharpe LG : Brain lesions in an infant rhesus monkey treated with monsodium glutamate. Science 1969 ; 166 : 386-8
-
(1969)
Science
, vol.166
, pp. 386-388
-
-
Olney, J.W.1
Sharpe, L.G.2
-
130
-
-
0022446150
-
Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
-
Beal MF, Kowall NW, Ellison DW, Mazurek MF, Swartz KJ, Martin JB : Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature 1986 ; 321 : 168-71
-
(1986)
Nature
, vol.321
, pp. 168-171
-
-
Beal, M.F.1
Kowall, N.W.2
Ellison, D.W.3
Mazurek, M.F.4
Swartz, K.J.5
Martin, J.B.6
-
131
-
-
0024533140
-
Differential sparing of somatostatin-neuropeptide Y and cholinergic neurons following striatal excitotoxin lesions
-
Beal MF, Kowall NW, Swartz KJ, Ferrante RJ, Martin JB : Differential sparing of somatostatin-neuropeptide Y and cholinergic neurons following striatal excitotoxin lesions. Synapse 1989 ; 3 : 38-47
-
(1989)
Synapse
, vol.3
, pp. 38-47
-
-
Beal, M.F.1
Kowall, N.W.2
Swartz, K.J.3
Ferrante, R.J.4
Martin, J.B.5
-
132
-
-
0026043565
-
Chronic quinolinic acid lesions in rats closely resemble Huntington's disease
-
Beal MF, Ferrante RJ, Swartz KJ, Kowall NW : Chronic quinolinic acid lesions in rats closely resemble Huntington's disease. J Neurosci 1991 ; 11 : 1649-59
-
(1991)
J Neurosci
, vol.11
, pp. 1649-1659
-
-
Beal, M.F.1
Ferrante, R.J.2
Swartz, K.J.3
Kowall, N.W.4
-
133
-
-
0027396021
-
Excitotoxin lesions in primates as a model for Huntington's disease : Histopathologic and neurochemical characterization
-
Ferrante RJ, Kowall NW, Cipolloni PB, Storey E, Beal MF : Excitotoxin lesions in primates as a model for Huntington's disease : histopathologic and neurochemical characterization. Exp Neurol 1993 ; 119 : 46-71
-
(1993)
Exp Neurol
, vol.119
, pp. 46-71
-
-
Ferrante, R.J.1
Kowall, N.W.2
Cipolloni, P.B.3
Storey, E.4
Beal, M.F.5
-
134
-
-
0032815680
-
Replicating Huntington's disease phenotype in experimental animals
-
Brouillet E, Conde F, Beal MF, Hantraye P : Replicating Huntington's disease phenotype in experimental animals. Prog Neurobiol 1999 ; 59 : 427-68
-
(1999)
Prog Neurobiol
, vol.59
, pp. 427-468
-
-
Brouillet, E.1
Conde, F.2
Beal, M.F.3
Hantraye, P.4
-
135
-
-
0030997707
-
Quantifiable bradykinesia, gait abnormalities and Huntington's disease-like striatal lesions in rats chronically treated with 3-nitropropionic acid
-
Guyot MC, Hantraye P, Dolan R, Palfi S, Maziere M, Brouillet E : Quantifiable bradykinesia, gait abnormalities and Huntington's disease-like striatal lesions in rats chronically treated with 3-nitropropionic acid. Neuroscience 1997 ; 79 : 45-56
-
(1997)
Neuroscience
, vol.79
, pp. 45-56
-
-
Guyot, M.C.1
Hantraye, P.2
Dolan, R.3
Palfi, S.4
Maziere, M.5
Brouillet, E.6
-
136
-
-
0037107346
-
The adenosine A1 receptor agonist adenosine amine congener exerts a neuroprotective effect against the development of striatal lesions and motor impairments in the 3-nitropropionic acid model of neurotoxicity
-
Blum D, Gall D, Galas MC, D'Alcantara P, Bantubungi K, Schiffmann SN : The adenosine A1 receptor agonist adenosine amine congener exerts a neuroprotective effect against the development of striatal lesions and motor impairments in the 3-nitropropionic acid model of neurotoxicity. J Neurosci 2002 ; 22 : 9122-33
-
(2002)
J Neurosci
, vol.22
, pp. 9122-9133
-
-
Blum, D.1
Gall, D.2
Galas, M.C.3
D'Alcantara, P.4
Bantubungi, K.5
Schiffmann, S.N.6
-
138
-
-
0026939298
-
Role of excitotoxicity in human neurological disease
-
Beal MF : Role of excitotoxicity in human neurological disease. Curr Opin Neurobiol 1992 ; 2 : 657-62
-
(1992)
Curr Opin Neurobiol
, vol.2
, pp. 657-662
-
-
Beal, M.F.1
-
139
-
-
0027686249
-
Puttfarcken P : Oxidative stress, glutamate, and neurodegenerative disorders
-
Coyle JT, Puttfarcken P : Oxidative stress, glutamate, and neurodegenerative disorders. Science 1993 ; 262 : 689-95
-
(1993)
Science
, vol.262
, pp. 689-695
-
-
Coyle, J.T.1
-
140
-
-
0028762647
-
Excitatory amino acids as a final common pathway for neurologic disorders
-
Lipton SA, Rosenberg PA : Excitatory amino acids as a final common pathway for neurologic disorders. N Engl J Med 1994 ; 330 : 613-22
-
(1994)
N Engl J Med
, vol.330
, pp. 613-622
-
-
Lipton, S.A.1
Rosenberg, P.A.2
-
141
-
-
0033571743
-
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
-
Levine MS, Klapstein GJ, Koppel A et al. : Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res 1999 ; 58 : 515-32
-
(1999)
J Neurosci Res
, vol.58
, pp. 515-532
-
-
Levine, M.S.1
Klapstein, G.J.2
Koppel, A.3
-
142
-
-
0035889535
-
NMDA receptor function in mouse models of Huntington disease
-
Cepeda C, Ariano MA, Calvert CR et al. : NMDA receptor function in mouse models of Huntington disease. J Neurosci Res 2001 ; 66 : 525-39
-
(2001)
J Neurosci Res
, vol.66
, pp. 525-539
-
-
Cepeda, C.1
Ariano, M.A.2
Calvert, C.R.3
-
143
-
-
0035575858
-
Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease
-
Laforet GA, Sapp E, Chase K et al. : Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci 2001 ; 21 : 9112-23
-
(2001)
J Neurosci
, vol.21
, pp. 9112-9123
-
-
Laforet, G.A.1
Sapp, E.2
Chase, K.3
-
144
-
-
5044224807
-
Genetic mouse models of Huntington's and Parkinson's diseases : Illuminating but imperfect
-
Levine MS, Cepeda C, Hickey MA, Fleming SM, Chesselet MF : Genetic mouse models of Huntington's and Parkinson's diseases : illuminating but imperfect. Trends Neurosci 2004 ; 27 : 691-7
-
(2004)
Trends Neurosci
, vol.27
, pp. 691-697
-
-
Levine, M.S.1
Cepeda, C.2
Hickey, M.A.3
Fleming, S.M.4
Chesselet, M.F.5
-
145
-
-
0037075624
-
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease
-
Zeron MM, Hansson O, Chen N et al. : Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 2002 ; 33 : 849-60
-
(2002)
Neuron
, vol.33
, pp. 849-860
-
-
Zeron, M.M.1
Hansson, O.2
Chen, N.3
-
146
-
-
0346749473
-
Enhanced Akt signaling is an early pro-survival response that reflects N-methyl-D-aspartate receptor activation in Huntington's disease knock-in striatal cells
-
Gines S, Ivanova E, Seong IS, Saura CA, MacDonald ME : Enhanced Akt signaling is an early pro-survival response that reflects N-methyl-D-aspartate receptor activation in Huntington's disease knock-in striatal cells. J Biol Chem 2003 ; 278 : 50514-22
-
(2003)
J Biol Chem
, vol.278
, pp. 50514-50522
-
-
Gines, S.1
Ivanova, E.2
Seong, I.S.3
Saura, C.A.4
MacDonald, M.E.5
-
147
-
-
29144460321
-
Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity
-
Shin JY, Fang ZH, Yu ZX, Wang CE, Li SH, Li XJ : Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity. J Cell Biol 2005 ; 171 : 1001-12
-
(2005)
J Cell Biol
, vol.171
, pp. 1001-1012
-
-
Shin, J.Y.1
Fang, Z.H.2
Yu, Z.X.3
Wang, C.E.4
Li, S.H.5
Li, X.J.6
-
148
-
-
0032971311
-
Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin
-
Chen N, Luo T, Wellington C, Metzler M, McCutcheon K, Hayden MR, Raymond LA : Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin. J Neurochem 1999 ; 72 : 1890-8
-
(1999)
J Neurochem
, vol.72
, pp. 1890-1898
-
-
Chen, N.1
Luo, T.2
Wellington, C.3
Metzler, M.4
McCutcheon, K.5
Hayden, M.R.6
Raymond, L.A.7
-
149
-
-
0344413576
-
Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington's disease
-
Li L, Fan M, Icton CD et al. : Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington's disease. Neurobiol Aging 2003 ; 24 : 1113-21
-
(2003)
Neurobiol Aging
, vol.24
, pp. 1113-1121
-
-
Li, L.1
Fan, M.2
Icton, C.D.3
-
150
-
-
9644270316
-
Neostriatal and cortical quinolinate levels are increased in early grade Huntington's disease
-
Guidetti P, Luthi-Carter RE, Augood SJ, Schwarcz R : Neostriatal and cortical quinolinate levels are increased in early grade Huntington's disease. Neurobiol Dis 2004 ; 17 : 455-61
-
(2004)
Neurobiol Dis
, vol.17
, pp. 455-461
-
-
Guidetti, P.1
Luthi-Carter, R.E.2
Augood, S.J.3
Schwarcz, R.4
-
151
-
-
18144406846
-
-
Giorgini F, Guidetti P, Nguyen Q, Bennett SC, Muchowski PJ : A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease. Nat Genet 2005 ; 37 : 526-31
-
Giorgini F, Guidetti P, Nguyen Q, Bennett SC, Muchowski PJ : A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease. Nat Genet 2005 ; 37 : 526-31
-
-
-
-
152
-
-
4444302167
-
Deranged neuronal calcium signaling and Huntington disease
-
Bezprozvanny I, Hayden MR : Deranged neuronal calcium signaling and Huntington disease. Biochem Biophys Res Commun 2004 ; 322 : 1310-7
-
(2004)
Biochem Biophys Res Commun
, vol.322
, pp. 1310-1317
-
-
Bezprozvanny, I.1
Hayden, M.R.2
-
153
-
-
0041963057
-
-
Tang TS, Tu H, Chan EY et al. : Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron 2003 ; 39 : 227-39
-
Tang TS, Tu H, Chan EY et al. : Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron 2003 ; 39 : 227-39
-
-
-
-
154
-
-
0037096376
-
Calpain activation in Huntington's disease
-
Gafni J, Ellerby LM : Calpain activation in Huntington's disease. J Neurosci 2002 ; 22 : 4842-9
-
(2002)
J Neurosci
, vol.22
, pp. 4842-4849
-
-
Gafni, J.1
Ellerby, L.M.2
-
155
-
-
14044264256
-
-
Tang TS, Slow E, Lupu V et al. : Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A 2005 ; 102 : 2602-7
-
Tang TS, Slow E, Lupu V et al. : Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A 2005 ; 102 : 2602-7
-
-
-
-
156
-
-
0030937126
-
Decreases in evoked overflow of dopamine in rat striatum after neurotoxic doses of metamphetamine
-
Cass WA : Decreases in evoked overflow of dopamine in rat striatum after neurotoxic doses of metamphetamine. J Pharmacol Exp Ther 1997 ; 280 : 105-13
-
(1997)
J Pharmacol Exp Ther
, vol.280
, pp. 105-113
-
-
Cass, W.A.1
-
157
-
-
24744434920
-
Unravelling a role for dopamine in Huntington's disease : The dual role of reactive oxygen species and D2 receptor stimulation
-
Charvin D, Vanhoutte P, Pages C, Borrelli E, Caboche J : Unravelling a role for dopamine in Huntington's disease : the dual role of reactive oxygen species and D2 receptor stimulation. Proc Natl Acad Sci U S A 2005 ; 102 : 12218-23
-
(2005)
Proc Natl Acad Sci U S A
, vol.102
, pp. 12218-12223
-
-
Charvin, D.1
Vanhoutte, P.2
Pages, C.3
Borrelli, E.4
Caboche, J.5
-
158
-
-
33845612662
-
Dopamine enhances motor and neuropathological consequences of polyglutamine expanded huntingtin
-
Cyr M, Sotnikova TD, Gainetdinov RR, Caron MG : Dopamine enhances motor and neuropathological consequences of polyglutamine expanded huntingtin. FASEB J 2006 ; 20 : 2541-3
-
(2006)
FASEB J
, vol.20
, pp. 2541-2543
-
-
Cyr, M.1
Sotnikova, T.D.2
Gainetdinov, R.R.3
Caron, M.G.4
-
159
-
-
0032413345
-
6-hydroxydopamine injections into the nigrostriatal pathway attenuate striatal malonate and 3-nitropropionic acid lesions
-
Maragos WF, Jakel RJ, Pang Z, Geddes JW 6-hydroxydopamine injections into the nigrostriatal pathway attenuate striatal malonate and 3-nitropropionic acid lesions. Exp Neurol 1998 ; 154 : 637-44
-
(1998)
Exp Neurol
, vol.154
, pp. 637-644
-
-
Maragos, W.F.1
Jakel, R.J.2
Pang, Z.3
Geddes, J.W.4
-
160
-
-
0032402138
-
Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease
-
Reynolds DS, Carter RJ, Morton AJ : Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease. J Neurosci 1998 ; 18 : 10116-27
-
(1998)
J Neurosci
, vol.18
, pp. 10116-10127
-
-
Reynolds, D.S.1
Carter, R.J.2
Morton, A.J.3
-
161
-
-
0038231381
-
Adenosine receptors and Huntington's disease : Implications for pathogenesis and therapeutics
-
Blum D, Hourez R, Galas MC, Popoli P, Schiffmann SN : Adenosine receptors and Huntington's disease : implications for pathogenesis and therapeutics. Lancet Neurol 2003 ; 2 : 366-74
-
(2003)
Lancet Neurol
, vol.2
, pp. 366-374
-
-
Blum, D.1
Hourez, R.2
Galas, M.C.3
Popoli, P.4
Schiffmann, S.N.5
-
162
-
-
0038113064
-
A dual role of adenosine A2A receptors in 3-nitropropionic acid-induced striatal lesions : Implications for the neuroprotective potential of A2A antagonists
-
Blum D, Galas MC, Pintor A et al. : A dual role of adenosine A2A receptors in 3-nitropropionic acid-induced striatal lesions : implications for the neuroprotective potential of A2A antagonists. J Neurosci 2003 ; 23 : 5361-9
-
(2003)
J Neurosci
, vol.23
, pp. 5361-5369
-
-
Blum, D.1
Galas, M.C.2
Pintor, A.3
-
163
-
-
0036522987
-
Blockade of striatal adenosine A2A receptor reduces, through a presynaptic mechanism, quinolinic acid-induced excitotoxicity : Possible relevance to neuroprotective interventions in neurodegenerative diseases of the striatum
-
Popoli P, Pintor A, Domenici MR et al. : Blockade of striatal adenosine A2A receptor reduces, through a presynaptic mechanism, quinolinic acid-induced excitotoxicity : possible relevance to neuroprotective interventions in neurodegenerative diseases of the striatum. J Neurosci 2002 ; 22 : 1967-75
-
(2002)
J Neurosci
, vol.22
, pp. 1967-1975
-
-
Popoli, P.1
Pintor, A.2
Domenici, M.R.3
-
164
-
-
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
-
165
-
-
37649008929
-
Functions, dysfunctions and possible therapeutic relevance of adenosine A2A receptors in Hutington's disease
-
sous presse
-
Popoli P Blum D, Martire A, Ledent C, Ceruti S, Abbracchio MP : Functions, dysfunctions and possible therapeutic relevance of adenosine A2A receptors in Hutington's disease. Prog Neurobiol 2006 (sous presse)
-
(2006)
Prog Neurobiol
-
-
Popoli, P.1
Blum, D.2
Martire, A.3
Ledent, C.4
Ceruti, S.5
Abbracchio, M.P.6
-
166
-
-
18844441689
-
Emerging chemotherapeutic strategies for Huntington's disease
-
Ryu H, Ferrante RJ : Emerging chemotherapeutic strategies for Huntington's disease. Expert Opin Emerg Drugs 2005 ; 10 : 345-63
-
(2005)
Expert Opin Emerg Drugs
, vol.10
, pp. 345-363
-
-
Ryu, H.1
Ferrante, R.J.2
-
167
-
-
33646248382
-
CYTE-I-HD : Phase I dose finding and tolerability study of systeamine (cystagon) in Hutington's disease
-
Dubinsky R, Gray C : CYTE-I-HD : phase I dose finding and tolerability study of systeamine (cystagon) in Hutington's disease. Mov Disord 2006 ; 21 : 530-3
-
(2006)
Mov Disord
, vol.21
, pp. 530-533
-
-
Dubinsky, R.1
Gray, C.2
-
168
-
-
33646421164
-
Cystamine and cysteamine increase brain levels of BDNF in Huntington's disease via HSJ1b and transglutaminase
-
Borrell-Pages M, Canals JM, Cordelieres FP et al. : Cystamine and cysteamine increase brain levels of BDNF in Huntington's disease via HSJ1b and transglutaminase. J Clin Invest 2006 ; 116 : 140-24
-
(2006)
J Clin Invest
, vol.116
, pp. 140-124
-
-
Borrell-Pages, M.1
Canals, J.M.2
Cordelieres, F.P.3
-
169
-
-
31544454404
-
Rapamycin alleviates toxicity of different aggregate-prone proteins
-
Berger Z, Ravikumar B, Menzies FM et al. : Rapamycin alleviates toxicity of different aggregate-prone proteins. Hum Mol Genet 2006 ; 15 : 433-42
-
(2006)
Hum Mol Genet
, vol.15
, pp. 433-442
-
-
Berger, Z.1
Ravikumar, B.2
Menzies, F.M.3
-
170
-
-
0034537290
-
Autophagy as a regulated pathway of cellular degradation
-
Klionsky DJ, Emr SD : Autophagy as a regulated pathway of cellular degradation. Science 2000 ; 290 : 1717-21
-
(2000)
Science
, vol.290
, pp. 1717-1721
-
-
Klionsky, D.J.1
Emr, S.D.2
-
171
-
-
0036566266
-
Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy
-
Ravikumar B, Duden R, Rubinsztein DC : Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy. Hum Mol Genet 2002 ; 11 : 1107-17
-
(2002)
Hum Mol Genet
, vol.11
, pp. 1107-1117
-
-
Ravikumar, B.1
Duden, R.2
Rubinsztein, D.C.3
-
172
-
-
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
-
173
-
-
25444483066
-
Lithium induces autophagy by inhibiting inositol monophosphatase
-
Sarkar S, Floto RA, Berger Z et al. : Lithium induces autophagy by inhibiting inositol monophosphatase. J Cell Biol 2005 ; 170 : 1101-11
-
(2005)
J Cell Biol
, vol.170
, pp. 1101-1111
-
-
Sarkar, S.1
Floto, R.A.2
Berger, Z.3
-
174
-
-
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
-
175
-
-
34248994604
-
Small molecules enhance autophagy and reduce toxicity in Huntington's disease models
-
Sarkar S, Perlstein EO, Imarisio S et al. : Small molecules enhance autophagy and reduce toxicity in Huntington's disease models. Nat Chem Biol 2007 ; 3 : 331-8
-
(2007)
Nat Chem Biol
, vol.3
, pp. 331-338
-
-
Sarkar, S.1
Perlstein, E.O.2
Imarisio, S.3
-
176
-
-
20044390015
-
-
Zhang X, Smith DL, Meriin AB et al. : A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo. Proc Natl Acad Sci U S A 2005 ; 102 : 892-7
-
Zhang X, Smith DL, Meriin AB et al. : A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo. Proc Natl Acad Sci U S A 2005 ; 102 : 892-7
-
-
-
-
177
-
-
18744369020
-
-
Heiser V, Engemann S, Brocker W et al. : Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay. Proc Natl Acad Sci U S A 2002 ; 99 : 16400-6
-
Heiser V, Engemann S, Brocker W et al. : Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay. Proc Natl Acad Sci U S A 2002 ; 99 : 16400-6
-
-
-
-
178
-
-
33846351654
-
Selective inhibitors of death in mutant huntingtin cells
-
Varma H, Voisine C, DeMarco CT et al. : Selective inhibitors of death in mutant huntingtin cells. Nat Chem Biol 2007 ; 2 : 99-100
-
(2007)
Nat Chem Biol
, vol.2
, pp. 99-100
-
-
Varma, H.1
Voisine, C.2
DeMarco, C.T.3
-
179
-
-
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
-
180
-
-
0037452775
-
-
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 U S A 2003 ; 100 : 2041-6
-
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 U S A 2003 ; 100 : 2041-6
-
-
-
-
181
-
-
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
-
182
-
-
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 : 1592-9
-
(2002)
J Neurosci
, vol.22
, pp. 1592-1599
-
-
Ferrante, R.J.1
Andreassen, O.A.2
Dedeoglu, A.3
-
183
-
-
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
-
-
-
184
-
-
2942527444
-
Integrating fetal neural transplants into a therapeutic strategy : The example of Huntington's disease
-
Peschanski M, Bachoud-Levi AC, Hantraye P : Integrating fetal neural transplants into a therapeutic strategy : the example of Huntington's disease. Brain 2004 ; 127 : 1219-28
-
(2004)
Brain
, vol.127
, pp. 1219-1228
-
-
Peschanski, M.1
Bachoud-Levi, A.C.2
Hantraye, P.3
-
185
-
-
15444378095
-
Cell therapy in Huntington's disease
-
Dunnett SB, Rosser AE : Cell therapy in Huntington's disease. Neurorx 2004 ; 1 : 394-405
-
(2004)
Neurorx
, vol.1
, pp. 394-405
-
-
Dunnett, S.B.1
Rosser, A.E.2
-
186
-
-
0029011778
-
Rationale for intrastriatal grafting of striatal neuroblasts in patients with Huntington's disease
-
Peschanski M, Cesaro P, Hantraye P : Rationale for intrastriatal grafting of striatal neuroblasts in patients with Huntington's disease. Neuroscience 1995 ; 68 : 273-85
-
(1995)
Neuroscience
, vol.68
, pp. 273-285
-
-
Peschanski, M.1
Cesaro, P.2
Hantraye, P.3
-
187
-
-
0031777250
-
Functional integration of striatal allografts in a primate model of Huntington's disease
-
Kendall AL, Rayment FD, Torres EM, Baker HF, Ridley RM, Dunnett SB : Functional integration of striatal allografts in a primate model of Huntington's disease. Nat Med 1998 ; 4 : 727-9
-
(1998)
Nat Med
, vol.4
, pp. 727-729
-
-
Kendall, A.L.1
Rayment, F.D.2
Torres, E.M.3
Baker, H.F.4
Ridley, R.M.5
Dunnett, S.B.6
-
188
-
-
0031902287
-
Fetal striatal allografts reverse cognitive deficits in a primate model of Huntington disease
-
Palfi S, Conde F, Riche D et al. : Fetal striatal allografts reverse cognitive deficits in a primate model of Huntington disease. Nat Med 1998 ; 4 : 963-6
-
(1998)
Nat Med
, vol.4
, pp. 963-966
-
-
Palfi, S.1
Conde, F.2
Riche, D.3
-
190
-
-
0021226160
-
Functional neuronal replacement by grafted striatal neurones in the ibotenic acid-lesioned rat striatum
-
Isacson O, Brundin P, Kelly PA, Gage FH, Bjorklund A : Functional neuronal replacement by grafted striatal neurones in the ibotenic acid-lesioned rat striatum. Nature 1984 ; 311 : 458-60
-
(1984)
Nature
, vol.311
, pp. 458-460
-
-
Isacson, O.1
Brundin, P.2
Kelly, P.A.3
Gage, F.H.4
Bjorklund, A.5
-
191
-
-
0032211289
-
Striatal transplantation in a transgenic mouse model of Huntington's disease
-
Dunnett SB, Carter RJ, Watts C et al. : Striatal transplantation in a transgenic mouse model of Huntington's disease. Exp Neurol 1998 ; 154 : 31-40
-
(1998)
Exp Neurol
, vol.154
, pp. 31-40
-
-
Dunnett, S.B.1
Carter, R.J.2
Watts, C.3
-
192
-
-
0031777250
-
Functional integration of striatal allografts in a primate model of Huntington's disease
-
Kendall AL, Rayment FD, Torres EM, Baker HF, Ridley RM, Dunnett SB : Functional integration of striatal allografts in a primate model of Huntington's disease. Nat Med 1998 ; 4 : 727-9
-
(1998)
Nat Med
, vol.4
, pp. 727-729
-
-
Kendall, A.L.1
Rayment, F.D.2
Torres, E.M.3
Baker, H.F.4
Ridley, R.M.5
Dunnett, S.B.6
-
193
-
-
0033940773
-
Behavioral recovery after transplantation into a rat model of Huntington's disease : Dependence on anatomical connectivity and extensive postoperative training
-
Brasted PJ, Watts C, Torres EM, Robbins TW, Dunnett SB : Behavioral recovery after transplantation into a rat model of Huntington's disease : dependence on anatomical connectivity and extensive postoperative training. Behav Neurosci 2000 ; 114 : 431-6
-
(2000)
Behav Neurosci
, vol.114
, pp. 431-436
-
-
Brasted, P.J.1
Watts, C.2
Torres, E.M.3
Robbins, T.W.4
Dunnett, S.B.5
-
194
-
-
0023900877
-
Striatal grafts in rats with unilateral neostriatal lesions. II. In vivo monitoring of GABA release in globus pallidus and substantia nigra
-
Sirinathsinghji DJ, Dunnett SB, Isacson O, Clarke DJ, Kendrick K, Bjorklund A : Striatal grafts in rats with unilateral neostriatal lesions. II. In vivo monitoring of GABA release in globus pallidus and substantia nigra. Neuroscience 1988 ; 24 : 803-11
-
(1988)
Neuroscience
, vol.24
, pp. 803-811
-
-
Sirinathsinghji, D.J.1
Dunnett, S.B.2
Isacson, O.3
Clarke, D.J.4
Kendrick, K.5
Bjorklund, A.6
-
195
-
-
0026603230
-
Anatomy and connectivity of intrastriatal striatal transplants
-
Wictorin K : Anatomy and connectivity of intrastriatal striatal transplants. Prog Neurobiol 1992 ; 38 : 611-39
-
(1992)
Prog Neurobiol
, vol.38
, pp. 611-639
-
-
Wictorin, K.1
-
196
-
-
0027460630
-
Characterization of GABA release from intrastriatal striatal transplants : Dependence on host-derived afferents
-
Campbell K, Kalen P, Wictorin K, Lundberg C, Mandel RJ, Bjorklund A : Characterization of GABA release from intrastriatal striatal transplants : dependence on host-derived afferents. Neuroscience 1993 ; 53 : 403-15
-
(1993)
Neuroscience
, vol.53
, pp. 403-415
-
-
Campbell, K.1
Kalen, P.2
Wictorin, K.3
Lundberg, C.4
Mandel, R.J.5
Bjorklund, A.6
-
197
-
-
0032890301
-
Embryonic striatal grafts restore neuronal activity of the globus pallidus in a rodent model of Huntington's disease
-
Nakao N, Ogura M, Nakai K, Itakura T : Embryonic striatal grafts restore neuronal activity of the globus pallidus in a rodent model of Huntington's disease. Neuroscience 1999 ; 88 : 469-77
-
(1999)
Neuroscience
, vol.88
, pp. 469-477
-
-
Nakao, N.1
Ogura, M.2
Nakai, K.3
Itakura, T.4
-
198
-
-
0034015127
-
Fetal tissue transplants in animal models of Huntington's disease : The effects on damaged neuronal circuitry and behavioral deficits
-
Nakao N, Itakura T : Fetal tissue transplants in animal models of Huntington's disease : the effects on damaged neuronal circuitry and behavioral deficits. Prog Neurobiol 2000 ; 61 : 313-38
-
(2000)
Prog Neurobiol
, vol.61
, pp. 313-338
-
-
Nakao, N.1
Itakura, T.2
-
199
-
-
0037066096
-
Bilateral human fetal striatal transplantation in Huntington's disease
-
Hauser RA, Furtado S, Cimino CR et al. : Bilateral human fetal striatal transplantation in Huntington's disease. Neurology 2002 ; 58 : 687-95
-
(2002)
Neurology
, vol.58
, pp. 687-695
-
-
Hauser, R.A.1
Furtado, S.2
Cimino, C.R.3
-
200
-
-
0031887047
-
Safety of intrastriatal neurotransplantation for Huntington's disease patients
-
Kopyov OV, Jacques S, Lieberman A, Duma CM, Eagle KS : Safety of intrastriatal neurotransplantation for Huntington's disease patients. Exp Neurol 1998 ; 149 : 97-108
-
(1998)
Exp Neurol
, vol.149
, pp. 97-108
-
-
Kopyov, O.V.1
Jacques, S.2
Lieberman, A.3
Duma, C.M.4
Eagle, K.S.5
-
201
-
-
0034627143
-
Motor and cognitive improvements in patients with Huntington's disease after neural transplantation
-
Bachoud-Levi AC, Remy P, Nguyen JP et al. : Motor and cognitive improvements in patients with Huntington's disease after neural transplantation. Lancet 2000 ; 356 : 1975-9
-
(2000)
Lancet
, vol.356
, pp. 1975-1979
-
-
Bachoud-Levi, A.C.1
Remy, P.2
Nguyen, J.P.3
-
202
-
-
0034105443
-
Porcine xenografts in Parkinson's disease and Huntington's disease patients : Preliminary results
-
Fink JS, Schumacher JM, Ellias SL et al. : Porcine xenografts in Parkinson's disease and Huntington's disease patients : preliminary results. Cell Transplant 2000 ; 9 : 273-8.
-
(2000)
Cell Transplant
, vol.9
, pp. 273-278
-
-
Fink, J.S.1
Schumacher, J.M.2
Ellias, S.L.3
-
203
-
-
0036904801
-
Unilateral transplantation of human primary fetal tissue in four patients with Huntington's disease : NEST-UK safety report ISRCTN no 36485475
-
Rosser AE, Barker RA, Harrower T et al. : Unilateral transplantation of human primary fetal tissue in four patients with Huntington's disease : NEST-UK safety report ISRCTN no 36485475. J Neurol Neurosurg Psychiatry 2002 ; 73 : 678-85
-
(2002)
J Neurol Neurosurg Psychiatry
, vol.73
, pp. 678-685
-
-
Rosser, A.E.1
Barker, R.A.2
Harrower, T.3
-
204
-
-
9144221004
-
Striatal neural grafting improves cortical metabolism in Huntington's disease patients
-
Gaura V, Bachoud-Levi AC, Ribeiro MJ et al. : Striatal neural grafting improves cortical metabolism in Huntington's disease patients. Brain 2004 ; 127 : 65-72
-
(2004)
Brain
, vol.127
, pp. 65-72
-
-
Gaura, V.1
Bachoud-Levi, A.C.2
Ribeiro, M.J.3
-
205
-
-
0033595502
-
Influence of lamotrigine on progression of early Huntington disease : A randomized clinical trial
-
Kremer B, Clark CM, Almqvist EW et al. : Influence of lamotrigine on progression of early Huntington disease : a randomized clinical trial. Neurology 1999 ; 53 : 1000-11
-
(1999)
Neurology
, vol.53
, pp. 1000-1011
-
-
Kremer, B.1
Clark, C.M.2
Almqvist, E.W.3
-
206
-
-
33644967927
-
Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery : A long-term follow-up study
-
Bachoud-Levi AC, Gaura V, Brugieres P et al. : Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery : a long-term follow-up study. Lancet Neurol 2006 ; 5 : 303-9
-
(2006)
Lancet Neurol
, vol.5
, pp. 303-309
-
-
Bachoud-Levi, A.C.1
Gaura, V.2
Brugieres, P.3
-
208
-
-
0032854992
-
The IGF-I amino-terminal tripeptide glycine-proline-glutamate (GPE) is neuroprotective to striatum in the quinolinic acid lesion animal model of Huntington's disease
-
Alexi T, Hughes PE, Roon-Mom WM et al. : The IGF-I amino-terminal tripeptide glycine-proline-glutamate (GPE) is neuroprotective to striatum in the quinolinic acid lesion animal model of Huntington's disease. Exp Neurol 1999 ; 159 : 84-97
-
(1999)
Exp Neurol
, vol.159
, pp. 84-97
-
-
Alexi, T.1
Hughes, P.E.2
Roon-Mom, W.M.3
-
209
-
-
0033789409
-
Brain-derived neurotrophic factor, neurotrophin-3, and neurotrophin-4/5 prevent the death of striatal projection neurons in a rodent model of Huntington's disease
-
Perez-Navarro E, Canudas AM, Akerund P, Alberch J, Arenas E : Brain-derived neurotrophic factor, neurotrophin-3, and neurotrophin-4/5 prevent the death of striatal projection neurons in a rodent model of Huntington's disease. J Neurochem 2000 ; 75 : 2190-9
-
(2000)
J Neurochem
, vol.75
, pp. 2190-2199
-
-
Perez-Navarro, E.1
Canudas, A.M.2
Akerund, P.3
Alberch, J.4
Arenas, E.5
-
210
-
-
0343091556
-
Neurturin protects striatal projection neurons but not interneurons in a rat model of Huntington's disease
-
Perez Navaro E, Akerud P, Marco S et al. : Neurturin protects striatal projection neurons but not interneurons in a rat model of Huntington's disease. Neuroscience 2000 ; 98 : 89-96
-
(2000)
Neuroscience
, vol.98
, pp. 89-96
-
-
Perez Navaro, E.1
Akerud, P.2
Marco, S.3
-
211
-
-
0036094145
-
Neuroprotection by neurotrophins and GDNF family members in the excitotoxic model of Huntington's disease
-
Alberch J, Perez-Navarro E, Canals JM : Neuroprotection by neurotrophins and GDNF family members in the excitotoxic model of Huntington's disease. Brain Res Bull 2002 ; 57 : 817-22
-
(2002)
Brain Res Bull
, vol.57
, pp. 817-822
-
-
Alberch, J.1
Perez-Navarro, E.2
Canals, J.M.3
-
212
-
-
0029900762
-
Ciliary neurotrophic factor protects striatal output neurons in an animal model of Huntington disease
-
Anderson KD, Panayotatos N, Corcoran TL, Lindsay RM, Wiegand SJ : Ciliary neurotrophic factor protects striatal output neurons in an animal model of Huntington disease. Proc Natl Acad Sci U S A 1996 ; 93 : 7346-51
-
(1996)
Proc Natl Acad Sci U S A
, vol.93
, pp. 7346-7351
-
-
Anderson, K.D.1
Panayotatos, N.2
Corcoran, T.L.3
Lindsay, R.M.4
Wiegand, S.J.5
-
213
-
-
0034690615
-
Restauration of cognitive and motor functions by ciliary neurotrophic factor in a primate model of Huntington's disease
-
Mittoux V, Joseph JM, Conde F et al. : Restauration of cognitive and motor functions by ciliary neurotrophic factor in a primate model of Huntington's disease. Hum Gene Ther 2000 ; 11 : 1177-87
-
(2000)
Hum Gene Ther
, vol.11
, pp. 1177-1187
-
-
Mittoux, V.1
Joseph, J.M.2
Conde, F.3
-
214
-
-
0034979217
-
Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease
-
Almeida LP, Zala D, Aebischer P, Deglon N : Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease. Neurobiol Dis 2001 ; 8 : 433-46
-
(2001)
Neurobiol Dis
, vol.8
, pp. 433-446
-
-
Almeida, L.P.1
Zala, D.2
Aebischer, P.3
Deglon, N.4
-
215
-
-
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
-
Regulier E, Pereira A, Sommer B, Aebischer P, Deglon 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
-
-
Regulier, E.1
Pereira, A.2
Sommer, B.3
Aebischer, P.4
Deglon, N.5
-
216
-
-
0036615771
-
Corticostriatopallidal neuroprotection by adenovirus-mediated ciliary neurotrophic factor gene transfer in a rat model of progressive striatal degeneration
-
Mittoux V, Ouary S, Monville C et al. : Corticostriatopallidal neuroprotection by adenovirus-mediated ciliary neurotrophic factor gene transfer in a rat model of progressive striatal degeneration. J Neurosci 2002 ; 22 : 4478-86
-
(2002)
J Neurosci
, vol.22
, pp. 4478-4486
-
-
Mittoux, V.1
Ouary, S.2
Monville, C.3
-
217
-
-
0030344755
-
The problems of delivering neuroactive molecules to the CNS
-
Tan SA, Aebischer P : The problems of delivering neuroactive molecules to the CNS. Ciba Found Symp 1996 ; 196 : 211-36
-
Ciba Found Symp 1996
, vol.196
, pp. 211-236
-
-
Tan, S.A.1
Aebischer, P.2
-
218
-
-
0030911441
-
Cellular delivery of human CNTF prevents motor and cognitive dysfunction in a rodent model of Huntington's disease
-
Emerich DF, Cain CK, Greco C et al. : Cellular delivery of human CNTF prevents motor and cognitive dysfunction in a rodent model of Huntington's disease. Cell Transplant 1997 ; 6 : 249-66
-
(1997)
Cell Transplant
, vol.6
, pp. 249-266
-
-
Emerich, D.F.1
Cain, C.K.2
Greco, C.3
-
219
-
-
0030906578
-
Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease
-
Emerich DF, Winn SR, Hantraye PM et al. : Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease. Nature 1997 ; 386 : 395-9
-
(1997)
Nature
, vol.386
, pp. 395-399
-
-
Emerich, D.F.1
Winn, S.R.2
Hantraye, P.M.3
-
220
-
-
15844381714
-
Intrathecal delivery of CNTF using encapsulated genetically modified xenogeneic cells in amyotrophic lateral sclerosis patients
-
Aebischer P, Schluep M, Deglon N et al. : Intrathecal delivery of CNTF using encapsulated genetically modified xenogeneic cells in amyotrophic lateral sclerosis patients. Nat Med 1996 ; 2 : 696-9
-
(1996)
Nat Med
, vol.2
, pp. 696-699
-
-
Aebischer, P.1
Schluep, M.2
Deglon, N.3
-
221
-
-
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-Levi AC, Deglon 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-Levi, A.C.2
Deglon, N.3
-
222
-
-
2342598416
-
Experimental therapeutics in transgenic mouse models of Huntington's disease
-
Beal MF, Ferrante RJ : Experimental therapeutics in transgenic mouse models of Huntington's disease. Nat Rev Neurosci 2004 ; 5 : 373-84
-
(2004)
Nat Rev Neurosci
, vol.5
, pp. 373-384
-
-
Beal, M.F.1
Ferrante, R.J.2
|