-
3
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is unstable on Huntington's Disease chromosomes
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
5
-
-
0033556344
-
Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease
-
(1999)
Neurology
, vol.52
, pp. 392-394
-
-
Nance, M.A.1
-
6
-
-
0030935035
-
The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size
-
(1997)
Am. J. Hum. Genet.
, vol.60
, pp. 1202-1210
-
-
Brinkman, R.R.1
-
8
-
-
0029082383
-
Inactivation of the mouse Huntington's disease gene homolog Hdh
-
(1995)
Science
, vol.269
, pp. 407-410
-
-
Duyao, M.P.1
-
9
-
-
0029055717
-
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
-
(1995)
Cell
, vol.81
, pp. 811-823
-
-
Nasir, J.1
-
10
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
-
(1995)
Nat. Genet.
, vol.11
, pp. 155-163
-
-
Zeitlin, S.1
-
11
-
-
0030613177
-
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
-
(1997)
Nat. Genet.
, vol.17
, pp. 404-410
-
-
White, J.K.1
-
12
-
-
0033757718
-
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
-
(2000)
Nat. Genet.
, vol.26
, pp. 300-306
-
-
Dragatsis, I.1
-
13
-
-
0035919701
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
-
(2001)
Science
, vol.293
, pp. 493-498
-
-
Zuccato, C.1
-
15
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
-
16
-
-
0033560924
-
Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
-
(1999)
J. Neurosci.
, vol.19
, pp. 3248-3257
-
-
Carter, R.J.1
-
17
-
-
0033009587
-
Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes
-
(1999)
Diabetes
, vol.48
, pp. 649-651
-
-
Hurlbert, M.S.1
-
18
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
-
20
-
-
0031446233
-
Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
-
(1997)
Neuron
, vol.19
, pp. 1147-1150
-
-
Ross, C.A.1
-
21
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
-
22
-
-
0031918640
-
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length
-
(1998)
Neurobiol. Dis.
, vol.4
, pp. 387-397
-
-
Becher, M.W.1
-
23
-
-
0032102186
-
Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset
-
(1998)
NeuroReport
, vol.9
, pp. 1823-1826
-
-
Gourfinkel-An, I.1
-
24
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
(1998)
Cell
, vol.95
, pp. 55-56
-
-
Saudou, F.1
-
25
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
(1999)
J. Neurosci.
, vol.19
, pp. 964-973
-
-
Kim, M.1
-
26
-
-
0034702030
-
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 1259-1271
-
-
Luthi-Carter, R.1
-
28
-
-
0034048845
-
Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease
-
(2000)
Exp. Neurol.
, vol.162
, pp. 328-342
-
-
Menalled, L.1
-
30
-
-
0028873186
-
Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis
-
(1995)
Neuroscience
, vol.64
, pp. 397-404
-
-
Sapp, E.1
-
32
-
-
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
-
(2000)
Neuroscience
, vol.98
, pp. 705-713
-
-
Denovan-Wright, E.M.1
Robertson, H.A.2
-
33
-
-
0033571743
-
Enhanced sensitivity to N-methyl-d-aspartate receptor activation in transgenic and knockin mouse models of Huntington's Disease
-
(1999)
J. Neurosci. Res.
, vol.58
, pp. 515-532
-
-
Levine, M.S.1
-
37
-
-
0033054555
-
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 397-407
-
-
Schilling, G.1
-
38
-
-
17344367977
-
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
-
(1998)
Nat. Genet.
, vol.20
, pp. 198-202
-
-
Reddy, P.H.1
-
39
-
-
0033136692
-
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
-
(1999)
Neuron
, vol.23
, pp. 181-192
-
-
Hodgson, J.G.1
-
41
-
-
0034163497
-
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 503-513
-
-
Wheeler, V.C.1
-
42
-
-
0032949459
-
Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 763-774
-
-
Shelbourne, P.F.1
-
43
-
-
0035862896
-
Neurological abnormalities in a knock-in mouse model of Huntington's disease
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 137-144
-
-
Lin, C.H.1
-
44
-
-
2042547083
-
Early motor deficits in CAG repeat knock-in mouse model of Huntington's disease
-
(2000)
Soc. Neurosci.
, pp. 1295
-
-
Menalled, L.1
-
45
-
-
0034426013
-
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
-
(2000)
Nat. Genet.
, vol.25
, pp. 385-389
-
-
Li, H.1
-
46
-
-
0034598808
-
Motor disorder in Huntington's disease begins as a dysfunction in error feedback control
-
(2000)
Nature
, vol.403
, pp. 544-549
-
-
Smith, M.A.1
-
47
-
-
0034641887
-
Dramatic mutation instability in HD mouse striatum: Does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2539-2544
-
-
Kennedy, L.1
Shelbourne, P.F.2
-
48
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
(1998)
Nat. Genet.
, vol.18
, pp. 150-154
-
-
Martindale, D.1
-
49
-
-
0008187510
-
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
-
(1966)
Nat. Genet.
, vol.13
, pp. 380-382
-
-
Goldberg, Y.P.1
-
50
-
-
0032502715
-
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 9158-9167
-
-
Wellington, C.L.1
-
51
-
-
0035869544
-
Tissue-specific proteolysis of Huntingtin (htt) in human brain: Evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum
-
(2001)
J. Neurosci.
, vol.21
, pp. 1830-1837
-
-
Mende-Mueller, L.M.1
-
52
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
-
53
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
-
54
-
-
0032811511
-
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 1227-1236
-
-
Li, H.1
-
55
-
-
0032568517
-
Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene
-
(1998)
Proc. Natl. Acad. Sci. U. S. A.
, vol.95
, pp. 6480-6485
-
-
Cha, J.H.1
-
57
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity
-
(2001)
Science
, vol.291
, pp. 2423-2428
-
-
Nucifora Jr., F.C.1
-
59
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
(2000)
Cell
, vol.101
, pp. 57-66
-
-
Yamamoto, A.1
-
60
-
-
0033912716
-
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
-
(2000)
Nat. Med.
, vol.6
, pp. 797-801
-
-
Chen, M.1
-
61
-
-
0034955984
-
Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease
-
(2001)
Ann. Neurol.
, vol.50
, pp. 112-117
-
-
Anderassen, O.A.1
-
62
-
-
0034660457
-
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
-
(2000)
J. Neurosci.
, vol.20
, pp. 4389-4397
-
-
Ferrante, R.J.1
-
63
-
-
0034743672
-
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease
-
(2001)
Neurobiol. Dis.
, vol.8
, pp. 479-491
-
-
Anderassen, O.A.1
-
64
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
-
65
-
-
0020685951
-
Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain
-
(1983)
Ann. Neurol.
, vol.13
, pp. 72-78
-
-
Sorbi, S.1
-
67
-
-
0342635463
-
Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease
-
(2000)
Brain Res.
, vol.862
, pp. 234-237
-
-
Perez-Severiano, F.1
-
68
-
-
0033982887
-
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
-
(2000)
Ann. Neurol.
, vol.47
, pp. 80-86
-
-
Tabrizi, S.J.1
-
69
-
-
0032897760
-
Impaired synaptic plasticity in mice carrying the Huntington's disease mutation
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 839-846
-
-
Usdin, M.T.1
|