-
1
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies, S. W. et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537-548 (1997).
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
-
2
-
-
0031918640
-
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy-correlation between the density of inclusions and IT-15 CAG triplet repeat length
-
Becher, M. W. et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy-correlation between the density of inclusions and IT-15 CAG triplet repeat length. Neurobiol. Dis. 4, 387-397 (1998).
-
(1998)
Neurobiol. Dis.
, vol.4
, pp. 387-397
-
-
Becher, M.W.1
-
3
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia, M. et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993 (1997).
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
-
4
-
-
0031469707
-
Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse
-
Ordway, J. M. et al. Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 91, 753-763 (1997).
-
(1997)
Cell
, vol.91
, pp. 753-763
-
-
Ordway, J.M.1
-
5
-
-
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, M. E. Huntingtin acts in the nucleus to induce apoptosis, but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55-66 (1998).
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
6
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
-
Klement, I. A. et al. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41-53 (1998).
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
-
7
-
-
0033391428
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
-
Cummings, C. J. et al. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron 24, 879-892 (1999).
-
(1999)
Neuron
, vol.24
, pp. 879-892
-
-
Cummings, C.J.1
-
8
-
-
0037388418
-
Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
-
Taylor, J. P. et al. Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein. Hum. Mol. Genet. 12, 749-757 (2003).
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 749-757
-
-
Taylor, J.P.1
-
9
-
-
0037012810
-
Expanded polyglutamine stretches form an 'aggresome'
-
Shimohata, T. et al. Expanded polyglutamine stretches form an 'aggresome'. Neurosci. Lett. 323, 215-218 (2002).
-
(2002)
Neurosci. Lett.
, vol.323
, pp. 215-218
-
-
Shimohata, T.1
-
10
-
-
0032475877
-
Nuclear inclusions in glutamine repeat disorders: Are they pernicious, coincidental or beneficial?
-
Sisodia, S. S. Nuclear inclusions in glutamine repeat disorders: Are they pernicious, coincidental or beneficial? Cell 95, 1-4 (1998).
-
(1998)
Cell
, vol.95
, pp. 1-4
-
-
Sisodia, S.S.1
-
11
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence, N. F., Sampat, R. M. & Kopito, R. R. Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292, 1552-1555 (2001).
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
12
-
-
0031446233
-
Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases
-
Ross, C. A. Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases. Neuron 19, 1147-1150 (1997).
-
(1997)
Neuron
, vol.19
, pp. 1147-1150
-
-
Ross, C.A.1
-
13
-
-
0037174879
-
Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization
-
Poirier, M. A. et al. Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization. J. Biol. Chem. 277, 41032-41037 (2002).
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 41032-41037
-
-
Poirier, M.A.1
-
14
-
-
0035800572
-
Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity
-
Chen, S., Berthelier, V., Yang, W. & Wetzel, R. Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity. J. Mol. Biol. 311, 173-182 (2001).
-
(2001)
J. Mol. Biol.
, vol.311
, pp. 173-182
-
-
Chen, S.1
Berthelier, V.2
Yang, W.3
Wetzel, R.4
-
15
-
-
0034646426
-
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
-
Wyttenbach, A. et al. Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease. Proc. Natl Acad. Sci. USA 97, 2898-2903 (2000).
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 2898-2903
-
-
Wyttenbach, A.1
-
16
-
-
0037154229
-
Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment
-
Muchowski, P. J., Ning, K., D'Souza-Schorey, C. & Fields, S. Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. Proc. Natl Acad. Sci. USA 99, 727-732 (2002).
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 727-732
-
-
Muchowski, P.J.1
Ning, K.2
D'Souza-Schorey, C.3
Fields, S.4
-
17
-
-
0035849879
-
Cause of neural death in neurodegenerative disease attributable to expansion of glutamine repeats
-
Perutz, M. F. & Windle, A. H. Cause of neural death in neurodegenerative disease attributable to expansion of glutamine repeats. Nature 412, 143-144 (2001).
-
(2001)
Nature
, vol.412
, pp. 143-144
-
-
Perutz, M.F.1
Windle, A.H.2
-
18
-
-
7244251306
-
Longitudinal analysis to identify pathogenic factors in a striatal model of Huntington's disease
-
Arrasate, M., Brooks, L., Chang, P., Mitra, S. & Finkbeiner, S. Longitudinal analysis to identify pathogenic factors in a striatal model of Huntington's disease. Soc. Neurosci. Abstr. 29, 209.8 (2003).
-
(2003)
Soc. Neurosci. Abstr.
, vol.29
-
-
Arrasate, M.1
Brooks, L.2
Chang, P.3
Mitra, S.4
Finkbeiner, S.5
-
21
-
-
0033613212
-
Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells
-
Kazantsev, A., Preisinger, E., Dranovsky, A., Goldgaber, D. & Housman, D. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc. Natl Acad. Sci. USA 96, 11404-11409 (1999).
-
(1999)
Proc. Natl. Acad. Sci. USA
, vol.96
, pp. 11404-11409
-
-
Kazantsev, A.1
Preisinger, E.2
Dranovsky, A.3
Goldgaber, D.4
Housman, D.5
-
22
-
-
9344227302
-
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
-
Goldberg, Y. P. et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genet. 13, 442-449 (1996).
-
(1996)
Nature Genet.
, vol.13
, pp. 442-449
-
-
Goldberg, Y.P.1
-
23
-
-
18544400323
-
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
-
Scherzinger, E. et al. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549-558 (1997).
-
(1997)
Cell
, vol.90
, pp. 549-558
-
-
Scherzinger, E.1
-
24
-
-
0032502715
-
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine
-
Wellington, C. L. et al. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine. J. Biol. Chem. 273, 9158-9167 (1998).
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 9158-9167
-
-
Wellington, C.L.1
-
25
-
-
0035940412
-
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis
-
Kim, Y. J. et al. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc. Natl Acad. Sci. USA 98, 12784-12789 (2001).
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 12784-12789
-
-
Kim, Y.J.1
-
26
-
-
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
-
Mende-Mueller, L. M., Toneff, T., Hwang, S. R., Chesselet, M. F. & Hook, V. Y. H. 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. J. Neurosci. 21, 1830-1837 (2001).
-
(2001)
J. Neurosci.
, vol.21
, pp. 1830-1837
-
-
Mende-Mueller, L.M.1
Toneff, T.2
Hwang, S.R.3
Chesselet, M.F.4
Hook, V.Y.H.5
-
27
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini, L. et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87, 493-506 (1996).
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
-
28
-
-
0037062424
-
A monomeric red fluorescent protein
-
Campbell, R. E. et al. A monomeric red fluorescent protein. Proc. Natl Acad. Sci. USA 99, 7877-7882 (2002).
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 7877-7882
-
-
Campbell, R.E.1
-
29
-
-
0035254495
-
Green fluorescent protein as a novel tool to measure apoptosis and necrosis
-
Strebel, A., Harr, T., Bachmann, F., Wernli, M. & Erb, P. Green fluorescent protein as a novel tool to measure apoptosis and necrosis. Cytometry 43, 126-133 (2001).
-
(2001)
Cytometry
, vol.43
, pp. 126-133
-
-
Strebel, A.1
Harr, T.2
Bachmann, F.3
Wernli, M.4
Erb, P.5
-
31
-
-
0034644203
-
A one-hit model of cell death in inherited neuronal degenerations
-
Clarke, G. et al. A one-hit model of cell death in inherited neuronal degenerations. Nature 406, 195-199 (2000).
-
(2000)
Nature
, vol.406
, pp. 195-199
-
-
Clarke, G.1
-
32
-
-
0001589776
-
Differential loss of striatal projection neurons in Huntington disease
-
Reiner, A. et al. Differential loss of striatal projection neurons in Huntington disease. Proc. Natl Acad. Sci. USA 85, 5733-5737 (1988).
-
(1988)
Proc. Natl. Acad. Sci. USA
, vol.85
, pp. 5733-5737
-
-
Reiner, A.1
-
33
-
-
0029586383
-
Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients
-
Richfield, E. K., Maguire-Zeiss, K. A., Vonkeman, H. E. & Voorn, P. Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients. Ann. Neurol. 38, 852-861 (1995).
-
(1995)
Ann. Neurol.
, vol.38
, pp. 852-861
-
-
Richfield, E.K.1
Maguire-Zeiss, K.A.2
Vonkeman, H.E.3
Voorn, P.4
-
34
-
-
0035818579
-
Specificity in intracellular protein aggregation and inclusion body formation
-
Rajan, R. S., Illing, M. E., Bence, N. F. & Kopito, R. R. Specificity in intracellular protein aggregation and inclusion body formation. Proc. Natl Acad. Sci. USA 98, 13060-13065 (2001).
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 13060-13065
-
-
Rajan, R.S.1
Illing, M.E.2
Bence, N.F.3
Kopito, R.R.4
-
35
-
-
0033556160
-
Generation of neuronal intranuclear inclusions by polyglutamine-GFP: Analysis of inclusion clearance and toxicity as a function of polyglutamine length
-
Moulder, K. L., Onodera, O., Burke, J. R., Strittmatter, W. J. & Johnson, E. M. Jr Generation of neuronal intranuclear inclusions by polyglutamine-GFP: Analysis of inclusion clearance and toxicity as a function of polyglutamine length. J. Neurosci. 19, 705-715 (1999).
-
(1999)
J. Neurosci.
, vol.19
, pp. 705-715
-
-
Moulder, K.L.1
Onodera, O.2
Burke, J.R.3
Strittmatter, W.J.4
Johnson Jr., E.M.5
-
36
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Gutekunst, C. A. et al. Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology. J. Neurosci. 19, 2522-2534 (1999).
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
-
37
-
-
0033614768
-
Evidence for a recruitment and sequestration mechanism in Huntington's disease
-
Preisinger, E., Jordan, B. M., Kazantsev, A. & Housman, D. Evidence for a recruitment and sequestration mechanism in Huntington's disease. Phil. Trans. R. Soc. Lond. B 354, 1029-1034 (1999).
-
(1999)
Phil. Trans. R. Soc. Lond. B
, vol.354
, pp. 1029-1034
-
-
Preisinger, E.1
Jordan, B.M.2
Kazantsev, A.3
Housman, D.4
-
38
-
-
0032590053
-
Huntingtin aggregates may not predict neuronal death in Huntington's disease
-
Kuemmerle, S. et al. Huntingtin aggregates may not predict neuronal death in Huntington's disease. Ann. Neurol. 46, 842-849 (1999).
-
(1999)
Ann. Neurol.
, vol.46
, pp. 842-849
-
-
Kuemmerle, S.1
-
39
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
Kim, M. et al. Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition. J. Neurosci. 19, 964-973 (1999).
-
(1999)
J. Neurosci.
, vol.19
, pp. 964-973
-
-
Kim, M.1
-
40
-
-
0033951539
-
Green fluorescent protein as a quantitative tool
-
Hack, N. J. et al. Green fluorescent protein as a quantitative tool. J. Neurosci. Methods 95, 177-184 (2000).
-
(2000)
J. Neurosci. Methods
, vol.95
, pp. 177-184
-
-
Hack, N.J.1
-
41
-
-
0037421691
-
SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity
-
Yoo, S.-Y. et al. SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity. Neuron 37, 383-401 (2003).
-
(2003)
Neuron
, vol.37
, pp. 383-401
-
-
Yoo, S.-Y.1
-
42
-
-
0030175161
-
Differential expression of normal and mutant Huntington's disease gene alleles
-
Persichetti, F. et al. Differential expression of normal and mutant Huntington's disease gene alleles. Neurobiol. Dis. 3, 183-190 (1996).
-
(1996)
Neurobiol. Dis.
, vol.3
, pp. 183-190
-
-
Persichetti, F.1
-
43
-
-
18544368523
-
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
-
Kegel, K. B. et al. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J. Biol. Chem. 277, 7466-7476 (2002).
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 7466-7476
-
-
Kegel, K.B.1
-
44
-
-
0032847708
-
Nuclear targeting of mutant huntingtin increases toxicity
-
Peters, M. F. et al. Nuclear targeting of mutant huntingtin increases toxicity. Mol. Cell. Neurosci. 14, 121-128 (1999).
-
(1999)
Mol. Cell. Neurosci.
, vol.14
, pp. 121-128
-
-
Peters, M.F.1
-
45
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
Ravikumar, B. et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nature Genet. 36, 585-595 (2004).
-
(2004)
Nature Genet.
, vol.36
, pp. 585-595
-
-
Ravikumar, B.1
-
46
-
-
0037461730
-
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders
-
Sánchez, I., Mahlke, C. & Yuan, J. Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373-379 (2003).
-
(2003)
Nature
, vol.421
, pp. 373-379
-
-
Sánchez, I.1
Mahlke, C.2
Yuan, J.3
-
47
-
-
0037947662
-
A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila
-
Apostol, B. L. et al. A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc. Natl Acad. Sci. USA 100, 5950-5955 (2003).
-
(2003)
Proc. Natl. Acad. Sci. USA
, vol.100
, pp. 5950-5955
-
-
Apostol, B.L.1
-
48
-
-
1642633757
-
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
-
Tanaka, M. et al. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nature Med. 10, 148-154 (2004).
-
(2004)
Nature Med.
, vol.10
, pp. 148-154
-
-
Tanaka, M.1
-
49
-
-
0030856172
-
CREB: A major mediator of neuronal neurotrophin responses
-
Finkbeiner, S. et al. CREB: A major mediator of neuronal neurotrophin responses. Neuron 19, 1031-1047 (1997).
-
(1997)
Neuron
, vol.19
, pp. 1031-1047
-
-
Finkbeiner, S.1
-
50
-
-
3242723420
-
A cell-based screen for drugs to treat Huntington's disease
-
Aiken, C. T., Tobin, A. J. & Schweitzer, E. S. A cell-based screen for drugs to treat Huntington's disease. Neurobiol. Dis. 16, 546-555 (2004).
-
(2004)
Neurobiol. Dis.
, vol.16
, pp. 546-555
-
-
Aiken, C.T.1
Tobin, A.J.2
Schweitzer, E.S.3
|