-
1
-
-
0029968460
-
Trinucleotide repeats in neurogenetic disorders
-
Paulson, H.L. and Fischbeck, K.H. (1996) Trinucleotide repeats in neurogenetic disorders. Am. Rev. Neurosci., 19, 79-107.
-
(1996)
Am. Rev. Neurosci.
, vol.19
, pp. 79-107
-
-
Paulson, H.L.1
Fischbeck, K.H.2
-
2
-
-
0037194897
-
Polyglutamine pathogenesis: Emergence of unifying mechanisms for Huntington's Disease and related disorders
-
Ross, C.A. (2002) Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's Disease and related disorders. Neuron, 35, 819-822.
-
(2002)
Neuron
, vol.35
, pp. 819-822
-
-
Ross, C.A.1
-
3
-
-
0029991245
-
Neurobiology of Huntington's disease
-
Sharp, A.H. and Ross, C.A. (1996) Neurobiology of Huntington's disease. Neurobiol. Dis., 3, 3-15.
-
(1996)
Neurobiol. Dis.
, vol.3
, pp. 3-15
-
-
Sharp, A.H.1
Ross, C.A.2
-
4
-
-
0032912386
-
Recent advances on the pathogenesis of Huntington's Disease
-
Petersen, A., Mani, K. and Brundin, P. (1999) Recent advances on the pathogenesis of Huntington's Disease. Exp. Neurol., 157, 1-18.
-
(1999)
Exp. Neurol.
, vol.157
, pp. 1-18
-
-
Petersen, A.1
Mani, K.2
Brundin, P.3
-
5
-
-
0031446233
-
Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
-
Ross, C.A. (1997) Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? Neuron, 19, 1147-1150.
-
(1997)
Neuron
, vol.19
, pp. 1147-1150
-
-
Ross, C.A.1
-
6
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuritis in brain
-
DiFiglia, M., Sapp, E., Chase, K.O., Davies, S.W., Bates, G.P., Vonsattel, J.P. and Aronin, N. (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuritis in brain. Science, 277, 1990-1993.
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
Davies, S.W.4
Bates, G.P.5
Vonsattel, J.P.6
Aronin, N.7
-
7
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Gutekunst, C.A., Li, S.H., Yi, H., Mulroy, J.S., Kuemmerle, S., Jones, R., Rye, D., Ferrante, R.J., Hersch, S.M. and Li, X.J. (1999) Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci., 19, 2522-2534.
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
Mulroy, J.S.4
Kuemmerle, S.5
Jones, R.6
Rye, D.7
Ferrante, R.J.8
Hersch, S.M.9
Li, X.J.10
-
8
-
-
0033811788
-
Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human
-
Huynh, D.P., Figueroa, K., Hoang, N. and Pulst, S.M. (2000) Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nat. Genet., 26, 44-50.
-
(2000)
Nat. Genet.
, vol.26
, pp. 44-50
-
-
Huynh, D.P.1
Figueroa, K.2
Hoang, N.3
Pulst, S.M.4
-
9
-
-
0032769095
-
Abundant expression and cytoplasmic aggregations of [alpha] 1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6
-
Ishikawa, K., Fujigasaki, H., Saegusa, H., Ohwada, K., Fujita, T., Iwamoto, H., Komatsuzaki, Y., Toru, S., Toriyama, H., Watanabe, M. et al. (1999) Abundant expression and cytoplasmic aggregations of [alpha] 1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6. Hum. Mol. Genet., 8, 1185-1193.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 1185-1193
-
-
Ishikawa, K.1
Fujigasaki, H.2
Saegusa, H.3
Ohwada, K.4
Fujita, T.5
Iwamoto, H.6
Komatsuzaki, Y.7
Toru, S.8
Toriyama, H.9
Watanabe, M.10
-
10
-
-
7144253143
-
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture
-
Cooper, J.K., Schilling, G., Peters, M.F., Herring, W.J., Sharp, A.H., Kaminsky, Z., Masone, J., Khan, F.A., Delanoy, M., Borchelt, DR. et al. (1998) Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum. Mol. Genet., 7, 783-790.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 783-790
-
-
Cooper, J.K.1
Schilling, G.2
Peters, M.F.3
Herring, W.J.4
Sharp, A.H.5
Kaminsky, Z.6
Masone, J.7
Khan, F.A.8
Delanoy, M.9
Borchelt, D.R.10
-
11
-
-
0033168302
-
Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin
-
Li, S.H., Cheng, A.L., Li, H. and Li, X.J. (1999) Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin. J. Neurosci., 19, 5159-5172.
-
(1999)
J. Neurosci.
, vol.19
, pp. 5159-5172
-
-
Li, S.H.1
Cheng, A.L.2
Li, H.3
Li, X.J.4
-
12
-
-
0030850412
-
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
-
Paulson, H.L., Perez, M.K., Trottier, Y., Trojanowski, J.Q., Subramony, S.H., Das, S.S., Vig, P., Mandel, J.L., Fischbeck, K.H. and Pittman, R.N. (1997) Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron, 19, 333-344.
-
(1997)
Neuron
, vol.19
, pp. 333-344
-
-
Paulson, H.L.1
Perez, M.K.2
Trottier, Y.3
Trojanowski, J.Q.4
Subramony, S.H.5
Das, S.S.6
Vig, P.7
Mandel, J.L.8
Fischbeck, K.H.9
Pittman, R.N.10
-
13
-
-
0032945938
-
Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
-
Stenoien, D.L., Cummings, C.J., Adams, H.P., Mancini, M.G., Patel, K., DeMartino, G.N., Marcelli, M., Weigel, N.L. and Mancini, M.A. (1999) Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet., 8, 731-741.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 731-741
-
-
Stenoien, D.L.1
Cummings, C.J.2
Adams, H.P.3
Mancini, M.G.4
Patel, K.5
DeMartino, G.N.6
Marcelli, M.7
Weigel, N.L.8
Mancini, M.A.9
-
14
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
Cummings, C.J., Mancini, M.A., Antalffy, B., DeFranco, D.B., Orr, H.T. and Zoghbi, H.Y. (1998) Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet., 19, 148-154.
-
(1998)
Nat. Genet.
, vol.19
, pp. 148-154
-
-
Cummings, C.J.1
Mancini, M.A.2
Antalffy, B.3
DeFranco, D.B.4
Orr, H.T.5
Zoghbi, H.Y.6
-
15
-
-
0032101287
-
The influence of huntingtin protein size on nuclear localization and cellular toxicity
-
Hackam, A.S., Singaraja, R., Wellington, C.L., Metzler, M., McCutcheon, K., Zhang, T., Kalchman, M. and Hayden, M.R. (1998) The influence of huntingtin protein size on nuclear localization and cellular toxicity. J. Cell Biol., 141, 1097-1105.
-
(1998)
J. Cell Biol.
, vol.141
, pp. 1097-1105
-
-
Hackam, A.S.1
Singaraja, R.2
Wellington, C.L.3
Metzler, M.4
McCutcheon, K.5
Zhang, T.6
Kalchman, M.7
Hayden, M.R.8
-
16
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale, D., Hackam, A., Wieczorek, A., Ellerby, L., Wellington, C., McCutcheon, K., Singaraja, R., Kazemi-Esfarjani, P., Devon, R., Kim, S.U. et al. (1998) Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat. Genet., 18, 150-154.
-
(1998)
Nat. Genet.
, vol.18
, pp. 150-154
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
Ellerby, L.4
Wellington, C.5
McCutcheon, K.6
Singaraja, R.7
Kazemi-Esfarjani, P.8
Devon, R.9
Kim, S.U.10
-
17
-
-
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. and Greenberg, M.E. (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell, 95, 55-66.
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
18
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
-
Klement, I.A., Skinner, P.J., Kaytor, M.D., Yi, H., Hersch, S.M., Clark, H.B., Zoghbi, H.Y. and Orr, H.T. (1998) Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell, 95, 41-53.
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
Skinner, P.J.2
Kaytor, M.D.3
Yi, H.4
Hersch, S.M.5
Clark, H.B.6
Zoghbi, H.Y.7
Orr, H.T.8
-
19
-
-
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., McIntyre, C., Martin, E.J., Chang, P., Kim, T.W., Williams, M., Reddy, P.H., Tagle, D. et al. (1999) Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J. Neurosci., 19, 964-973.
-
(1999)
J. Neurosci.
, vol.19
, pp. 964-973
-
-
Kim, M.1
Lee, H.S.2
LaForet, G.3
McIntyre, C.4
Martin, E.J.5
Chang, P.6
Kim, T.W.7
Williams, M.8
Reddy, P.H.9
Tagle, D.10
-
20
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
-
Warrick, J.M., Chan, H.Y., Gray-Board, G.L., Chai, Y., Paulson, H.L. and Bonini, N.M. (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet., 23, 425-428.
-
(1999)
Nat. Genet.
, vol.23
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
21
-
-
0033391428
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
-
Cummings, C.J., Reinstein, E., Sun, Y., Antalffy, B., Jiang, Y., Ciechanover, A., Orr, H.T., Beaudet, A.L. and Zoghbi, H.Y. (1999) Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron, 24, 879-892.
-
(1999)
Neuron
, vol.24
, pp. 879-892
-
-
Cummings, C.J.1
Reinstein, E.2
Sun, Y.3
Antalffy, B.4
Jiang, Y.5
Ciechanover, A.6
Orr, H.T.7
Beaudet, A.L.8
Zoghbi, H.Y.9
-
22
-
-
0032475877
-
Nuclear inclusions in glutamine repeat disorders: Are they pernicious, coincidental, or beneficial?
-
Sisodia, S.S. (1998) Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial? Cell, 95, 1-4.
-
(1998)
Cell
, vol.95
, pp. 1-4
-
-
Sisodia, S.S.1
-
23
-
-
0032191848
-
Neuronal intranuclear inclusions in polyglutamine diseases: Nuclear weapons or nuclear fallout?
-
Kim, T.W. and Tanzim, R.E. (1998) Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout? Neuron, 21, 657-659.
-
(1998)
Neuron
, vol.21
, pp. 657-659
-
-
Kim, T.W.1
Tanzim, R.E.2
-
24
-
-
0033396283
-
Intranuclear inclusions and the ubiquitin-proteasome pathway: Digestion of a red herring?
-
Floyd, J.A. and Hamilton, B.A. (1999) Intranuclear inclusions and the ubiquitin-proteasome pathway: digestion of a red herring? Neuron, 24, 765-766.
-
(1999)
Neuron
, vol.24
, pp. 765-766
-
-
Floyd, J.A.1
Hamilton, B.A.2
-
25
-
-
0035871295
-
Beyond the Qs in the polyglutarmine diseases
-
Orr, H.T. (2001) Beyond the Qs in the polyglutarmine diseases. Genes Devl., 15, 925-932.
-
(2001)
Genes Devl.
, vol.15
, pp. 925-932
-
-
Orr, H.T.1
-
26
-
-
23444458415
-
Transcriptional activation modulated by homopolymeric glutamine and proline stretches
-
Gerber, H.P., Seipel, K., Georgiev, O., Hofferer, M., Hug, M., Rusconi, S. and Schaffner, W. (1994) Transcriptional activation modulated by homopolymeric glutamine and proline stretches. Science, 263, 808-811.
-
(1994)
Science
, vol.263
, pp. 808-811
-
-
Gerber, H.P.1
Seipel, K.2
Georgiev, O.3
Hofferer, M.4
Hug, M.5
Rusconi, S.6
Schaffner, W.7
-
27
-
-
12944263711
-
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
-
Steffan, J.S., Kazantsev, A., Spasic-Boskovic, O., Greenwald, M., Zhu, Y.Z., Gohler, H., Wanker, E.E., Bates, G.P., Housman, D.E. and Thompson, L.M. (2000) The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA, 97, 6763-6768.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 6763-6768
-
-
Steffan, J.S.1
Kazantsev, A.2
Spasic-Boskovic, O.3
Greenwald, M.4
Zhu, Y.Z.5
Gohler, H.6
Wanker, E.E.7
Bates, G.P.8
Housman, D.E.9
Thompson, L.M.10
-
28
-
-
0033818112
-
Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription
-
Shimohata, T., Nakajima, T., Yamada, M., Uchida, C., Onodera, O., Naruse, S., Kimura, T., Koide, R., Nozaki, K., Sano, Y. et al. (2000) Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet., 26, 29-36.
-
(2000)
Nat. Genet.
, vol.26
, pp. 29-36
-
-
Shimohata, T.1
Nakajima, T.2
Yamada, M.3
Uchida, C.4
Onodera, O.5
Naruse, S.6
Kimura, T.7
Koide, R.8
Nozaki, K.9
Sano, Y.10
-
29
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity
-
Nucifora, F.C. Jr, Sasaki, M., Peters, M.F., Huang, H., Cooper, J.K., Yamada, M., Takahashi, H., Tsuji, S., Troncoso, J., Dawson, V.L., Dawson, T.M. and Ross, C.A. (2001) Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity. Science, 291, 2423-2428.
-
(2001)
Science
, vol.291
, pp. 2423-2428
-
-
Nucifora F.C., Jr.1
Sasaki, M.2
Peters, M.F.3
Huang, H.4
Cooper, J.K.5
Yamada, M.6
Takahashi, H.7
Tsuji, S.8
Troncoso, J.9
Dawson, V.L.10
Dawson, T.M.11
Ross, C.A.12
-
30
-
-
0035031779
-
Polyglutamine and CBP: Fatal attraction?
-
McCampbell, A. and Fischbeck, K.H. (2001) Polyglutamine and CBP: fatal attraction? Nat. Med., 7, 528-530.
-
(2001)
Nat. Med.
, vol.7
, pp. 528-530
-
-
McCampbell, A.1
Fischbeck, K.H.2
-
31
-
-
0034234237
-
CBP/p300 in cell growth, transformation, and development
-
Goodman, R.H. and Smolik, S. (2000) CBP/p300 in cell growth, transformation, and development. Genes Devl., 14, 1553-1577.
-
(2000)
Genes Devl.
, vol.14
, pp. 1553-1577
-
-
Goodman, R.H.1
Smolik, S.2
-
32
-
-
0036578784
-
Disruption of CREB function in brain leads to neurodegeneration
-
Mantamadiotis, T., Lemberger, T., Bleckmann, S.C., Kern, H., Kretz, O., Martin Villalba, A., Tronche, F., Kellendonk, C., Gau, D., Kapfhammer, J. et al. (2002) Disruption of CREB function in brain leads to neurodegeneration. Nat. Genet., 31, 47-54.
-
(2002)
Nat. Genet.
, vol.31
, pp. 47-54
-
-
Mantamadiotis, T.1
Lemberger, T.2
Bleckmann, S.C.3
Kern, H.4
Kretz, O.5
Martin Villalba, A.6
Tronche, F.7
Kellendonk, C.8
Gau, D.9
Kapfhammer, J.10
-
33
-
-
0034285017
-
CREB-binding protein sequestration by expanded polyglutamine
-
McCampbell, A., Taylor, J.P, Taye, A.A., Robitschek, J., Li, M., Walcott, J., Merry, D., Chai, Y., Paulson, H., Sobue, G. and Fischbeck, K.H. (2000) CREB-binding protein sequestration by expanded polyglutamine. Hum. Mol. Genet., 9, 2197-2202.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2197-2202
-
-
McCampbell, A.1
Taylor, J.P.2
Taye, A.A.3
Robitschek, J.4
Li, M.5
Walcott, J.6
Merry, D.7
Chai, Y.8
Paulson, H.9
Sobue, G.10
Fischbeck, K.H.11
-
34
-
-
0030867994
-
A role for 12-lipoxygenase in nerve cell death caused by glutathione depletion
-
Li, Y., Maher, P. and Schubert, D. (1997) A role for 12-lipoxygenase in nerve cell death caused by glutathione depletion. Neuron, 19, 453-463.
-
(1997)
Neuron
, vol.19
, pp. 453-463
-
-
Li, Y.1
Maher, P.2
Schubert, D.3
-
35
-
-
0032526940
-
The regulation of reactive oxygen species production during programmed cell death
-
Tan, S., Sagara, Y., Liu, Y., Maher P. and Schubert, D. (1998) The regulation of reactive oxygen species production during programmed cell death. J. Cell. Biol., 141, 1423-1432.
-
(1998)
J. Cell Biol.
, vol.141
, pp. 1423-1432
-
-
Tan, S.1
Sagara, Y.2
Liu, Y.3
Maher, P.4
Schubert, D.5
-
36
-
-
0034697364
-
Persistent activation of ERK contributes to glutamate-induced oxidative toxicity in a neuronal cell line and primary cortical neuron cultures
-
Stanciu, M., Wang, Y., Kentor, R., Burke, N., Watkins, S., Kress, G., Reynolds, I., Klann, E., Angiolieri, M.R., Johnson, J.W. and DeFranco, D.B. (2000) Persistent activation of ERK contributes to glutamate-induced oxidative toxicity in a neuronal cell line and primary cortical neuron cultures. J. Biol. Chem., 275, 12200-12206.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 12200-12206
-
-
Stanciu, M.1
Wang, Y.2
Kentor, R.3
Burke, N.4
Watkins, S.5
Kress, G.6
Reynolds, I.7
Klann, E.8
Angiolieri, M.R.9
Johnson, J.W.10
DeFranco, D.B.11
-
37
-
-
0032903802
-
Oxidative stress in Huntington's Disease
-
Browne, S.E., Ferrante, R.J. and Beal, M.F. (1999) Oxidative stress in Huntington's Disease. Brain Pathol., 9, 147-163.
-
(1999)
Brain Pathol.
, vol.9
, pp. 147-163
-
-
Browne, S.E.1
Ferrante, R.J.2
Beal, M.F.3
-
38
-
-
0034754875
-
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
-
Waelter, S., Boeddrich, A., Lurz, R., Scherzinger, E., Lueder, G., Lehrach, H. and Wanker, E.E. (2001) Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol. Biol. Cell., 12, 1393-1407.
-
(2001)
Mol. Biol. Cell
, vol.12
, pp. 1393-1407
-
-
Waelter, S.1
Boeddrich, A.2
Lurz, R.3
Scherzinger, E.4
Lueder, G.5
Lehrach, H.6
Wanker, E.E.7
-
39
-
-
0035880474
-
Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease
-
Wyttenbach, A., Swartz, J., Kita, H., Thykjaer, T., Carmichael, J., Bradley, J., Brown, R., Maxwell, M., Schapira, A., Orntoft, T.F., Kato, K. and Rubinsztein, D.C. (2001) Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease. Hum. Mol. Genet., 10, 1829-1845.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1829-1845
-
-
Wyttenbach, A.1
Swartz, J.2
Kita, H.3
Thykjaer, T.4
Carmichael, J.5
Bradley, J.6
Brown, R.7
Maxwell, M.8
Schapira, A.9
Orntoft, T.F.10
Kato, K.11
Rubinsztein, D.C.12
-
40
-
-
0033499931
-
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
-
Chai, Y., Koppenhafer, S.L., Bonini, N.M. and Paulson, H.L. (1999) Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J. Neurosci., 19, 10338-10347.
-
(1999)
J. Neurosci.
, vol.19
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
41
-
-
0034708793
-
Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract
-
Kobayashi, Y., Kume, A., Li, M., Doyu, M., Hata, M., Ohtsuka, K. and Sobue, G. (2000) Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract. J. Biol. Chem., 275, 8772-8778.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 8772-8778
-
-
Kobayashi, Y.1
Kume, A.2
Li, M.3
Doyu, M.4
Hata, M.5
Ohtsuka, K.6
Sobue, G.7
-
42
-
-
0036173896
-
Interaction of Huntington disease protein with transcriptional activator Sp1
-
Li, S.H., Cheng, A.L., Zhou, H., Lam, S., Rao, M., Li, H. and Li, X.J. (2002) Interaction of Huntington disease protein with transcriptional activator Sp1. Mol. Cell. Biol., 22, 1277-1287.
-
(2002)
Mol. Cell Biol.
, vol.22
, pp. 1277-1287
-
-
Li, S.H.1
Cheng, A.L.2
Zhou, H.3
Lam, S.4
Rao, M.5
Li, H.6
Li, X.J.7
-
43
-
-
0033638393
-
The 26S proteasome is required for estrogen receptor-alpha and coactivator turnover and for efficient estrogen receptor-alpha transactivation
-
Lonard, D.M., Nawaz, Z., Smith, C.L. and O'Malley, B.W. (2000) The 26S proteasome is required for estrogen receptor-alpha and coactivator turnover and for efficient estrogen receptor-alpha transactivation. Mol. Cell, 5, 939-948.
-
(2000)
Mol. Cell
, vol.5
, pp. 939-948
-
-
Lonard, D.M.1
Nawaz, Z.2
Smith, C.L.3
O'Malley, B.W.4
-
44
-
-
0030898710
-
Proteasome inhibition leads to a heat-shock response, induction of endoplasmic reticulum chaperones, and thermotolerance
-
Bush, K.T., Goldberg, A.L. and Nigam, S.K. (1997) Proteasome inhibition leads to a heat-shock response, induction of endoplasmic reticulum chaperones, and thermotolerance. J. Biol. Chem., 272, 9086-9092.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 9086-9092
-
-
Bush, K.T.1
Goldberg, A.L.2
Nigam, S.K.3
-
45
-
-
0036718782
-
Glucocorticoid receptors in hippocampal neurons that do not engage proteasomes escape from hormone-dependent down-regulation but maintain transactivation activity
-
Wang, X., Pongrac, J.L. and DeFranco, D.B. (2002) Glucocorticoid receptors in hippocampal neurons that do not engage proteasomes escape from hormone-dependent down-regulation but maintain transactivation activity. Mol. Endocrinol., 16, 1987-1998.
-
(2002)
Mol. Endocrinol.
, vol.16
, pp. 1987-1998
-
-
Wang, X.1
Pongrac, J.L.2
DeFranco, D.B.3
-
46
-
-
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., Carmichael, J., Swartz, J., Furlong, R.A., Narain, Y., Rankin, J. and Rubinsztein, D.C. (2000) 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)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 2898-2903
-
-
Wyttenbach, A.1
Carmichael, J.2
Swartz, J.3
Furlong, R.A.4
Narain, Y.5
Rankin, J.6
Rubinsztein, D.C.7
-
47
-
-
0032847708
-
Nuclear targeting of mutant Huntingtin increases toxicity
-
Peters, M.F., Nucifora, F.C. Jr, Kushi, J., Seaman, H.C., Cooper, J.K., Herring, W.J., Dawson, V.L., Dawson, T.M. and Ross, C.A. (1999) Nuclear targeting of mutant Huntingtin increases toxicity. Mol. Cell Neurosci., 14, 121-128.
-
(1999)
Mol. Cell Neurosci.
, vol.14
, pp. 121-128
-
-
Peters, M.F.1
Nucifora F.C., Jr.2
Kushi, J.3
Seaman, H.C.4
Cooper, J.K.5
Herring, W.J.6
Dawson, V.L.7
Dawson, T.M.8
Ross, C.A.9
-
48
-
-
0031610371
-
Measurement of cell death
-
Loo, D.T. and Rillema, J.R. (1998) Measurement of cell death. Meth. Cell Biol., 57, 251-264.
-
(1998)
Meth. Cell Biol.
, vol.57
, pp. 251-264
-
-
Loo, D.T.1
Rillema, J.R.2
-
49
-
-
0031671566
-
Expressed luciferase viability assay (ELVA) for the measurement of cell growth and viability
-
Coombe, D.R., Nakhoul, A.M., Stevenson, S.M., Peroni, S.E. and Sanderson, C.J. (1998) Expressed luciferase viability assay (ELVA) for the measurement of cell growth and viability. J. Immunol. Meth., 215, 145-150.
-
(1998)
J. Immunol. Meth.
, vol.215
, pp. 145-150
-
-
Coombe, D.R.1
Nakhoul, A.M.2
Stevenson, S.M.3
Peroni, S.E.4
Sanderson, C.J.5
-
50
-
-
0034916613
-
p300/CBP proteins: HATs for transcriptional bridges and scaffolds
-
Chan, H.M. and La Thangue, N.B. (2001) p300/CBP proteins: HATs for transcriptional bridges and scaffolds. J. Cell Sci., 114, 2363-2373.
-
(2001)
J. Cell Sci.
, vol.114
, pp. 2363-2373
-
-
Chan, H.M.1
La Thangue, N.B.2
-
51
-
-
0035909330
-
Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
-
Steffan, J.S., Bodai, L., Pallos, J., Poelman, M., McCampbell, A., Apostol, B.L., Kazantsev, A, Schmidt, E., Zhu, Y.Z., Greenwald, M. et al. (2001) Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature, 413, 739-743.
-
(2001)
Nature
, vol.413
, pp. 739-743
-
-
Steffan, J.S.1
Bodai, L.2
Pallos, J.3
Poelman, M.4
McCampbell, A.5
Apostol, B.L.6
Kazantsev, A.7
Schmidt, E.8
Zhu, Y.Z.9
Greenwald, M.10
-
52
-
-
0032517816
-
Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation
-
Perez, M.K., Paulson, H.L., Pendse, S.J., Saionz, S.J., Bonini, N.M. and Pittman, R.N. (1998) Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J. Cell Biol., 143, 1457-1470.
-
(1998)
J. Cell Biol.
, vol.143
, pp. 1457-1470
-
-
Perez, M.K.1
Paulson, H.L.2
Pendse, S.J.3
Saionz, S.J.4
Bonini, N.M.5
Pittman, R.N.6
-
53
-
-
18544368523
-
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
-
Kegel, K.B., Meloni, A.R., Yi, Y., Kim, Y.J., Doyle, E., Cuiffo, B.G., Sapp, E., Wang, Y., Qin, Z.H., Chen, J.D. et al. (2002) 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)
J. Biol. Chem.
, vol.277
, pp. 7466-7476
-
-
Kegel, K.B.1
Meloni, A.R.2
Yi, Y.3
Kim, Y.J.4
Doyle, E.5
Cuiffo, B.G.6
Sapp, E.7
Wang, Y.8
Qin, Z.H.9
Chen, J.D.10
-
54
-
-
0032946228
-
In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease
-
Hackam, A.S., Singaraja, R., Zhang, T., Gan, L. and Hayden, M.R. (1999) In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease. Hum. Mol. Genet., 8, 25-33.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 25-33
-
-
Hackam, A.S.1
Singaraja, R.2
Zhang, T.3
Gan, L.4
Hayden, M.R.5
-
55
-
-
0034678342
-
Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch
-
Becker, M., Martin, E., Schneikert, J., Krug, H.F. and Cato, A.C. (2000) Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch. J. Cell Biol., 149, 255-262.
-
(2000)
J. Cell Biol.
, vol.149
, pp. 255-262
-
-
Becker, M.1
Martin, E.2
Schneikert, J.3
Krug, H.F.4
Cato, A.C.5
-
56
-
-
0035336658
-
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release
-
Jana, N.R., Zemskov, E.A., Wang, G. and Nukina, N. (2001) Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Hum. Mol. Genet., 10, 1049-1059.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1049-1059
-
-
Jana, N.R.1
Zemskov, E.A.2
Wang, G.3
Nukina, N.4
-
57
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence, N.F., Sampat, R.M. and Kopito, R.R. (2001) Impairment of the ubiquitin-proteasome system by protein aggregation. Science, 292, 1552-1555.
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
58
-
-
0037041420
-
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
-
Bucciantini, M., Giannoni, E., Chiti, F., Baroni, E, Formigli, L., Zurdo, J., Taddei, N., Ramponi, G., Dobson, C.M. and Stefani, M. (2002) Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature, 416, 507-511.
-
(2002)
Nature
, vol.416
, pp. 507-511
-
-
Bucciantini, M.1
Giannoni, E.2
Chiti, F.3
Baroni, E.4
Formigli, L.5
Zurdo, J.6
Taddei, N.7
Ramponi, G.8
Dobson, C.M.9
Stefani, M.10
-
59
-
-
0037134460
-
Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival
-
Ding, Q., Lewis, J.J., Strum, K.M., Dimayuga, E., Bruce-Keller, A.J., Dunn, J.C. and Keller, J.N. (2002) Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival. J. Biol. Chem., 277, 13935-13942.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 13935-13942
-
-
Ding, Q.1
Lewis, J.J.2
Strum, K.M.3
Dimayuga, E.4
Bruce-Keller, A.J.5
Dunn, J.C.6
Keller, J.N.7
-
60
-
-
0034307476
-
Huntingtin expression stimulates endosomallysosomal activity, endosome tubulation, and autophagy
-
Kegel, K.B., Kim, M., Sapp, E., McIntyre, C., Castano, J.G., Aronin, N. and DiFiglia, M. (2000) Huntingtin expression stimulates endosomallysosomal activity, endosome tubulation, and autophagy. J. Neurosci., 20, 7268-7278.
-
(2000)
J. Neurosci.
, vol.20
, pp. 7268-7278
-
-
Kegel, K.B.1
Kim, M.2
Sapp, E.3
McIntyre, C.4
Castano, J.G.5
Aronin, N.6
DiFiglia, M.7
-
61
-
-
0032006110
-
Intracellular distribution of proteasomes
-
Rivett, A.J. (1998) Intracellular distribution of proteasomes. Curr. Opin. Immunol., 10, 110-114.
-
(1998)
Curr. Opin. Immunol.
, vol.10
, pp. 110-114
-
-
Rivett, A.J.1
-
62
-
-
0037150687
-
Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease
-
Dunah, A.W., Jeong, H., Griffin, A., Kim, Y.M., Standaert, D.G., Hersch, S.M., Mouradian, M.M., Young, A.B., Tanese, N. and Krainc, D. (2002) Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science, 296, 2238-2243.
-
(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
Mouradian, M.M.7
Young, A.B.8
Tanese, N.9
Krainc, D.10
-
63
-
-
0034858068
-
Altered trafficking of membrane proteins in Purkinje cells of SCA1 transgenic mice
-
Skinner, P.J., Vierra-Green, C.A., Clark, H.B., Zoghbi, H.Y. and Orr, H.T. (2001) Altered trafficking of membrane proteins in Purkinje cells of SCA1 transgenic mice. Am. J. Pathol., 159, 905-913.
-
(2001)
Am. J. Pathol.
, vol.159
, pp. 905-913
-
-
Skinner, P.J.1
Vierra-Green, C.A.2
Clark, H.B.3
Zoghbi, H.Y.4
Orr, H.T.5
-
64
-
-
0029858155
-
Differential regulation of c-Jun and JunD by ubiquitin-dependent protein degradation
-
Musti, A.M., Treier, M., Peverali, F.A. and Bohmann, D. (1996) Differential regulation of c-Jun and JunD by ubiquitin-dependent protein degradation. Biol. Chem., 377, 619-624.
-
(1996)
Biol. Chem.
, vol.377
, pp. 619-624
-
-
Musti, A.M.1
Treier, M.2
Peverali, F.A.3
Bohmann, D.4
-
65
-
-
0028136156
-
Differential roles of heat shock protein 70 in the in vitro nuclear import of glucocorticoid receptor and simian virus 40 large tumor antigen
-
Yang, J. and DeFranco, D.B. (1994) Differential roles of heat shock protein 70 in the in vitro nuclear import of glucocorticoid receptor and simian virus 40 large tumor antigen. Mol. Cell. Biol., 14, 5088-5098.
-
(1994)
Mol. Cell Biol.
, vol.14
, pp. 5088-5098
-
-
Yang, J.1
DeFranco, D.B.2
-
66
-
-
0033625751
-
Interaction of the tau2 transcriptional activation domain of glucocorticoid receptor with a novel steroid receptor coactivator, Hic-5, which localizes to both focal adhesions and the nuclear matrix
-
Yang, L., Guerrero, J., Hong, H., DeFranco, D.B. and Stallcup, M.R. (2000) Interaction of the tau2 transcriptional activation domain of glucocorticoid receptor with a novel steroid receptor coactivator, Hic-5, which localizes to both focal adhesions and the nuclear matrix. Mol. Biol. Cell., 11, 2007-2018.
-
(2000)
Mol. Biol. Cell
, vol.11
, pp. 2007-2018
-
-
Yang, L.1
Guerrero, J.2
Hong, H.3
DeFranco, D.B.4
Stallcup, M.R.5
|