-
1
-
-
0027164698
-
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
-
Orr HT, Chung MY, Banfi S, et al. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat Genet 1993;4:221-6.
-
(1993)
Nat Genet
, vol.4
, pp. 221-226
-
-
Orr, H.T.1
Chung, M.Y.2
Banfi, S.3
-
2
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi HY, Orr HT. Glutamine repeats and neurodegeneration. Annu Rev Neurosci 2000;23:217-47.
-
(2000)
Annu Rev Neurosci
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
-
3
-
-
0033621398
-
Aberrant protein deposition and neurological disease
-
Kaytor M, Warren S. Aberrant protein deposition and neurological disease. J Biol Chem 1999;274:37507-10.
-
(1999)
J Biol Chem
, vol.274
, pp. 37507-37510
-
-
Kaytor, M.1
Warren, S.2
-
4
-
-
0032475877
-
Nuclear inclusions in glutamine repeat disorders: Are they pernicious, coincidental, or beneficial?
-
Sisodia SS. Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial? Cell 1998;95:1-4.
-
(1998)
Cell
, vol.95
, pp. 1-4
-
-
Sisodia, S.S.1
-
5
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
Yamamoto A, Lucas JJ, Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000;101:57-66.
-
(2000)
Cell
, vol.101
, pp. 57-66
-
-
Yamamoto, A.1
Lucas, J.J.2
Hen, R.3
-
6
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
Ona VO, Li M, Vonsattel JP, et al. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 1999;399:263-7.
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.3
-
7
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
Kim M, Lee H-S, LaForet G, et al. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J Neurosci 1999;19:964-73.
-
(1999)
J Neurosci
, vol.19
, pp. 964-973
-
-
Kim, M.1
Lee, H.-S.2
LaForet, G.3
-
8
-
-
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, et al. 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
-
9
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
-
Klement IA, Skinner PJ, Kaytor MD, et al. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 1998;95:41-53.
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
Skinner, P.J.2
Kaytor, M.D.3
-
10
-
-
0032590053
-
Huntingtin aggregates may not predict neuronal death in Huntington's disease
-
Kuemmerle S, Gutekunst C-A, Klein AM, et al. Huntingtin 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
-
11
-
-
0035060505
-
Recruitment of non-expanded polyglutamine proteins in intranuclear aggregates of neuronal intranuclear hyaline inclusion disease
-
Takahashi J, Tanaka J, Arai K, et al. Recruitment of non-expanded polyglutamine proteins in intranuclear aggregates of neuronal intranuclear hyaline inclusion disease. J Neuropathol Exp Neurol 2001;60:369-76.
-
(2001)
J Neuropathol Exp Neurol
, vol.60
, pp. 369-376
-
-
Takahashi, J.1
Tanaka, J.2
Arai, K.3
-
12
-
-
0035900182
-
Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: Ultrastructural and morphometric studies of the cerebellar granule cells
-
Takahashi H, Egawa S, Piao Y-S, et al. Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: ultrastructural and morphometric studies of the cerebellar granule cells. Brain Res 2001;919:12-19.
-
(2001)
Brain Res
, vol.919
, pp. 12-19
-
-
Takahashi, H.1
Egawa, S.2
Piao, Y.-S.3
-
13
-
-
0036445864
-
Attenuated nuclear shrinkage in neurons with nuclear aggregates - A morphometric study on pontine neurons of Machado-Joseph disease brains
-
Uchihara T, Iwabuchi K, Funata N, et al. Attenuated nuclear shrinkage in neurons with nuclear aggregates - a morphometric study on pontine neurons of Machado-Joseph disease brains. Exp Neurol 2002;178:124-8.
-
(2002)
Exp Neurol
, vol.178
, pp. 124-128
-
-
Uchihara, T.1
Iwabuchi, K.2
Funata, N.3
-
14
-
-
0037316179
-
Influence of intranuclear inclusion on nuclear size - Morphometric study on pontine neurons of neuronal intranuclear inclusion disease cases
-
Uchihara T, Tanaka J, Funata N, et al. Influence of intranuclear inclusion on nuclear size - morphometric study on pontine neurons of neuronal intranuclear inclusion disease cases. Acta Neuropathol 2003;105:103-8.
-
(2003)
Acta Neuropathol
, vol.105
, pp. 103-108
-
-
Uchihara, T.1
Tanaka, J.2
Funata, N.3
-
15
-
-
0020566596
-
Nuclear membrane indentations in Huntington's chorea
-
Roos RAC, Bots GTAM. Nuclear membrane indentations in Huntington's chorea. J Neurol Sci 1983;61:37-47.
-
(1983)
J Neurol Sci
, vol.61
, pp. 37-47
-
-
Roos, R.A.C.1
Bots, G.T.A.M.2
-
16
-
-
0019425263
-
Neuropathological changes of the nucleus accumbens in Huntington's chorea
-
Bots GTAM, Bruyn GW. Neuropathological changes of the nucleus accumbens in Huntington's chorea. Acta Neuropathol 1981;55:21-2.
-
(1981)
Acta Neuropathol
, vol.55
, pp. 21-22
-
-
Bots, G.T.A.M.1
Bruyn, G.W.2
-
17
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies SW, Turmaine M, Cozens BA, et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997;90:537-48.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
-
18
-
-
0033391428
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
-
Cummings CJ, Reinstein E, Sun Y, et al. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron 1999;24:879-92.
-
(1999)
Neuron
, vol.24
, pp. 879-892
-
-
Cummings, C.J.1
Reinstein, E.2
Sun, Y.3
-
19
-
-
0033802841
-
Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains
-
Fujigasaki H, Uchihara T, Koyano S, et al. Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains. Exp Neurol 2000;165:248-56.
-
(2000)
Exp Neurol
, vol.165
, pp. 248-256
-
-
Fujigasaki, H.1
Uchihara, T.2
Koyano, S.3
-
20
-
-
0034088724
-
Neuronal intranuclear hyaline inclusion disease with polyglutamine immunoreactive inclusions
-
Takahashi J, Fukuda T, Tanaka J, et al. Neuronal intranuclear hyaline inclusion disease with polyglutamine immunoreactive inclusions. Acta Neuropathol 2000;99:589-94.
-
(2000)
Acta Neuropathol
, vol.99
, pp. 589-594
-
-
Takahashi, J.1
Fukuda, T.2
Tanaka, J.3
-
21
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
Cummings CJ, Mancini MA, Antalffy B, et al. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat Genet 1998;19:148-54.
-
(1998)
Nat Genet
, vol.19
, pp. 148-154
-
-
Cummings, C.J.1
Mancini, M.A.2
Antalffy, B.3
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