-
1
-
-
34547692622
-
Trinucleotide repeat disorders
-
Orr, H.T. and Zoghbi, H.Y. (2007) Trinucleotide repeat disorders. Annu. Rev. Neurosci., 30, 575-621.
-
(2007)
Annu. Rev. Neurosci.
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
2
-
-
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
-
3
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies, S.W., Turmaine, M., Cozens, B.A., DiFiglia, M., Sharp, A.H., Ross, C.A., Scherzinger, E., Wanker, E.E., Mangiarini, L. and Bates, G.P. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell, 90, 537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
DiFiglia, M.4
Sharp, A.H.5
Ross, C.A.6
Scherzinger, E.7
Wanker, E.E.8
Mangiarini, L.9
Bates, G.P.10
-
4
-
-
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
-
5
-
-
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
-
6
-
-
0033613212
-
Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells
-
Kazantsev, A., Preisinger, E., Dranovsky, A., Goldgaber, D. and Housman, D. (1999) Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc. Natl. Acad. Sci. US A, 96, 11404-11409.
-
(1999)
Proc. Natl. Acad. Sci. US A
, vol.96
, pp. 11404-11409
-
-
Kazantsev, A.1
Preisinger, E.2
Dranovsky, A.3
Goldgaber, D.4
Housman, D.5
-
7
-
-
0033030565
-
Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
-
Chai, Y., Koppenhafer, S.L., Shoesmith, S.J., Perez, M.K. and Paulson, H.L. (1999) Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Hum. Mol. Genet., 8, 673-682.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 673-682
-
-
Chai, Y.1
Koppenhafer, S.L.2
Shoesmith, S.J.3
Perez, M.K.4
Paulson, H.L.5
-
8
-
-
0028143527
-
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32
-
Kawaguchi, Y., Okamoto, T., Taniwaki, M., Aizawa, M., Inoue, M., Katayama, S., Kawakami, H., Nakamura, S., Nishimura, M., Akiguchi, I. et al. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet., 8, 221-228.
-
(1994)
1. Nat. Genet.
, vol.8
, pp. 221-228
-
-
Kawaguchi, Y.1
Okamoto, T.2
Taniwaki, M.3
Aizawa, M.4
Inoue, M.5
Katayama, S.6
Kawakami, H.7
Nakamura, S.8
Nishimura, M.9
Akiguchi, I.10
-
9
-
-
0030936575
-
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
-
Paulson, H.L., Das, S.S., Crino, P.B., Perez, M.K., Patel, S.C., Gotsdiner, D., Fischbeck, K.H. and Pittman, R.N. (1997) Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain. Ann. Neurol., 41, 453-462.
-
(1997)
Ann. Neurol.
, vol.41
, pp. 453-462
-
-
Paulson, H.L.1
Das, S.S.2
Crino, P.B.3
Perez, M.K.4
Patel, S.C.5
Gotsdiner, D.6
Fischbeck, K.H.7
Pittman, R.N.8
-
10
-
-
0031803642
-
Ataxin-3 is transported into the nucleus and associates with the nuclear matrix
-
Tait, D., Riccio, M., Sittler, A., Scherzinger, E., Santi, S., Ognibene, A., Maraldi, N.M., Lehrach, H. and Wanker, E.E. (1998) Ataxin-3 is transported into the nucleus and associates with the nuclear matrix. Hum. Mol. Genet., 7, 991-997.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 991-997
-
-
Tait, D.1
Riccio, M.2
Sittler, A.3
Scherzinger, E.4
Santi, S.5
Ognibene, A.6
Maraldi, N.M.7
Lehrach, H.8
Wanker, E.E.9
-
11
-
-
0032425541
-
Heterogeneous intracellular localization and expression of ataxin-3
-
Trottier, Y., Cancel, G., An-Gourfinkel, I., Lutz, Y., Weber, C., Brice, A., Hirsch, E. and Mandel, J.L. (1998) Heterogeneous intracellular localization and expression of ataxin-3. Neurobiol. Dis., 5, 335-347.
-
(1998)
Neurobiol. Dis.
, vol.5
, pp. 335-347
-
-
Trottier, Y.1
Cancel, G.2
An-Gourfinkel, I.3
Lutz, Y.4
Weber, C.5
Brice, A.6
Hirsch, E.7
Mandel, J.L.8
-
12
-
-
0032735135
-
Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix
-
Perez, M.K., Paulson, H.L. and Pittman, R.N. (1999) Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix. Hum. Mol. Genet., 8, 2377-2385.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 2377-2385
-
-
Perez, M.K.1
Paulson, H.L.2
Pittman, R.N.3
-
13
-
-
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
-
14
-
-
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
-
15
-
-
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., Iwabuchi, K., Yagishita, S., Makifuchi, T., Nakamura, A., Ishida, K., Toru, S., Hirai, S. et al. (2000) Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains. Exp. Neurol., 165, 248-256.
-
(2000)
Exp. Neurol.
, vol.165
, pp. 248-256
-
-
Fujigasaki, H.1
Uchihara, T.2
Koyano, S.3
Iwabuchi, K.4
Yagishita, S.5
Makifuchi, T.6
Nakamura, A.7
Ishida, K.8
Toru, S.9
Hirai, S.10
-
16
-
-
0037631378
-
Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity
-
Nucifora, F.C. Jr, Ellerby, L.M., Wellington, C.L., Wood, J.D., Herring, W.J., Sawa, A., Hayden, M.R., Dawson, V.L., Dawson, T.M. and Ross, C.A. (2003) Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity. J. Biol. Chem., 278, 13047-13055.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 13047-13055
-
-
Nucifora F.C., Jr.1
Ellerby, L.M.2
Wellington, C.L.3
Wood, J.D.4
Herring, W.J.5
Sawa, A.6
Hayden, M.R.7
Dawson, V.L.8
Dawson, T.M.9
Ross, C.A.10
-
17
-
-
11144305102
-
Glial and neuronal expression of polyglutamine proteins induce behavioral changes and aggregate formation in Drosophila
-
Kretzschmar, D., Tschape, J., Bettencourt Da Cruz, A., Asan, E., Poeck, B., Strauss, R. and Pflugfelder, G.O. (2005) Glial and neuronal expression of polyglutamine proteins induce behavioral changes and aggregate formation in Drosophila. Glia, 49, 59-72.
-
(2005)
Glia
, vol.49
, pp. 59-72
-
-
Kretzschmar, D.1
Tschape, J.2
Bettencourt Da Cruz, A.3
Asan, E.4
Poeck, B.5
Strauss, R.6
Pflugfelder, G.O.7
-
18
-
-
0032475941
-
-
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
-
-
18644379256
-
Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy
-
Katsuno, M., Adachi, H., Kume, A., Li, M., Nakagomi, Y., Niwa, H., Sang, C., Kobayashi, Y., Doyu, M. and Sobue, G. (2002) Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron, 35, 843-854.
-
(2002)
Neuron
, vol.35
, pp. 843-854
-
-
Katsuno, M.1
Adachi, H.2
Kume, A.3
Li, M.4
Nakagomi, Y.5
Niwa, H.6
Sang, C.7
Kobayashi, Y.8
Doyu, M.9
Sobue, G.10
-
20
-
-
34447520352
-
Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence
-
Bichelmeier, U., Schmidt, T., Hubener, J., Boy, J., Ruttiger, L., Habig, K., Poths, S., Bonin, M., Knipper, M., Schmidt, W.J. et al. (2007) Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence. J. Neurosci., 27, 7418-7428.
-
(2007)
J. Neurosci.
, vol.27
, pp. 7418-7428
-
-
Bichelmeier, U.1
Schmidt, T.2
Hubener, J.3
Boy, J.4
Ruttiger, L.5
Habig, K.6
Poths, S.7
Bonin, M.8
Knipper, M.9
Schmidt, W.J.10
-
21
-
-
0037160106
-
Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities
-
Li, F., Macfarlan, T., Pittman, R.N. and Chakravarti, D. (2002) Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities. J. Biol. Chem., 277, 45004-45012.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 45004-45012
-
-
Li, F.1
Macfarlan, T.2
Pittman, R.N.3
Chakravarti, D.4
-
22
-
-
0141891166
-
Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3
-
Evert, B.O., Vogt, I.R., Vieira-Saecker, A.M., Ozimek, L., de Vos, R.A., Brunt, E.R., Klockgether, T. and Wullner, U. (2003) Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3. J. Neuropathol. Exp. Neurol., 62, 1006-1018.
-
(2003)
J. Neuropathol. Exp. Neurol.
, vol.62
, pp. 1006-1018
-
-
Evert, B.O.1
Vogt, I.R.2
Vieira-Saecker, A.M.3
Ozimek, L.4
de Vos, R.A.5
Brunt, E.R.6
Klockgether, T.7
Wullner, U.8
-
23
-
-
33750962224
-
Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation
-
Evert, B.O., Araujo, J., Vieira-Saecker, A.M., de Vos, R.A., Harendza, S., Klockgether, T. and Wullner, U. (2006) Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation. J. Neurosci., 26, 11474-11486.
-
(2006)
J. Neurosci.
, vol.26
, pp. 11474-11486
-
-
Evert, B.O.1
Araujo, J.2
Vieira-Saecker, A.M.3
de Vos, R.A.4
Harendza, S.5
Klockgether, T.6
Wullner, U.7
-
24
-
-
45049085458
-
Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation
-
Chou, A.H., Yeh, T.H., Ouyang, P., Chen, Y.L., Chen, S.Y. and Wang, H.L. (2008) Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation. Neurobiol. Dis., 31, 89-101.
-
(2008)
Neurobiol. Dis.
, vol.31
, pp. 89-101
-
-
Chou, A.H.1
Yeh, T.H.2
Ouyang, P.3
Chen, Y.L.4
Chen, S.Y.5
Wang, H.L.6
-
25
-
-
0345099501
-
The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity
-
Burnett, B., Li, F. and Pittman, R.N. (2003) The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum. Mol. Genet., 12, 3195-3205.
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 3195-3205
-
-
Burnett, B.1
Li, F.2
Pittman, R.N.3
-
26
-
-
0242693202
-
Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics
-
Scheel, H., Tomiuk, S. and Hofmann, K. (2003) Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics. Hum. Mol. Genet., 12, 2845-2852.
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 2845-2852
-
-
Scheel, H.1
Tomiuk, S.2
Hofmann, K.3
-
27
-
-
0033867992
-
Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23
-
Wang, G., Sawai, N., Kotliarova, S., Kanazawa, I. and Nukina, N. (2000) Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B. Hum. Mol. Genet., 9, 1795-1803.
-
(2000)
HHR23A and HHR23B. Hum. Mol. Genet.
, vol.9
, pp. 1795-1803
-
-
Wang, G.1
Sawai, N.2
Kotliarova, S.3
Kanazawa, I.4
Nukina, N.5
-
28
-
-
0042691818
-
Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis
-
Doss-Pepe, E.W., Stenroos, E.S., Johnson, W.G. and Madura, K. (2003) Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis. Mol. Cell. Biol., 23, 6469-6483.
-
(2003)
Mol. Cell. Biol.
, vol.23
, pp. 6469-6483
-
-
Doss-Pepe, E.W.1
Stenroos, E.S.2
Johnson, W.G.3
Madura, K.4
-
29
-
-
0942287194
-
Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways
-
Chai, Y., Berke, S.S., Cohen, R.E. and Paulson, H.L. (2004) Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. J. Biol. Chem., 279, 3605-3611.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 3605-3611
-
-
Chai, Y.1
Berke, S.S.2
Cohen, R.E.3
Paulson, H.L.4
-
30
-
-
15444372240
-
The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation
-
Burnett, B.G. and Pittman, R.N. (2005) The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation. Proc. Natl Acad. Sci. U S A, 102, 4330-4335.
-
(2005)
Proc. Natl Acad. Sci. U S A
, vol.102
, pp. 4330-4335
-
-
Burnett, B.G.1
Pittman, R.N.2
-
31
-
-
15944419824
-
Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism
-
Warrick, J.M., Morabito, L.M., Bilen, J., Gordesky-Gold, B., Faust, L.Z., Paulson, H.L. and Bonini, N.M. (2005) Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol. Cell., 18, 37-48.
-
(2005)
Mol. Cell.
, vol.18
, pp. 37-48
-
-
Warrick, J.M.1
Morabito, L.M.2
Bilen, J.3
Gordesky-Gold, B.4
Faust, L.Z.5
Paulson, H.L.6
Bonini, N.M.7
-
32
-
-
23044515498
-
The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition
-
Nicastro, G., Menon, R.P., Masino, L., Knowles, P.P., McDonald, N.Q. and Pastore, A. (2005) The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition. Proc. Natl Acad. Sci. U S A, 102, 10493-10498.
-
(2005)
Proc. Natl Acad. Sci. U S A
, vol.102
, pp. 10493-10498
-
-
Nicastro, G.1
Menon, R.P.2
Masino, L.3
Knowles, P.P.4
McDonald, N.Q.5
Pastore, A.6
-
33
-
-
24644472965
-
Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain
-
Mao, Y., Senic-Matuglia, F., Di Fiore, P.P., Polo, S., Hodsdon, M.E. and De Camilli, P. (2005) Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain. Proc. Natl Acad. Sci. U S A, 102, 12700-12705.
-
(2005)
Proc. Natl Acad. Sci. U S A
, vol.102
, pp. 12700-12705
-
-
Mao, Y.1
Senic-Matuglia, F.2
Di Fiore, P.P.3
Polo, S.4
Hodsdon, M.E.5
De Camilli, P.6
-
34
-
-
33747891213
-
Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates
-
Zhong, X. and Pittman, R.N. (2006) Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates. Hum. Mol. Genet., 15, 2409-2420.
-
(2006)
Hum. Mol. Genet.
, vol.15
, pp. 2409-2420
-
-
Zhong, X.1
Pittman, R.N.2
-
35
-
-
33748988214
-
Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3
-
Wang, Q., Li, L. and Ye, Y. (2006) Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3. J. Cell Biol., 174, 963-971.
-
(2006)
J. Cell Biol.
, vol.174
, pp. 963-971
-
-
Wang, Q.1
Li, L.2
Ye, Y.3
-
36
-
-
33947637692
-
Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3
-
Rodrigues, A.J., Coppola, G., Santos, C., Costa Mdo, C., Ailion, M., Sequeiros, J., Geschwind, D.H. and Maciel, P. (2007) Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3. FASEB J., 21, 1126-1136.
-
(2007)
FASEB J.
, vol.21
, pp. 1126-1136
-
-
Rodrigues, A.J.1
Coppola, G.2
Santos, C.3
Costa Mdo, C.4
Ailion, M.5
Sequeiros, J.6
Geschwind, D.H.7
Maciel, P.8
-
37
-
-
55549086868
-
The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains
-
Winborn, B.J., Travis, S.M., Todi, S.V., Scaglione, K.M., Xu, P., Williams, A.J., Cohen, R.E., Peng, J. and Paulson, H.L. (2008) The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J. Biol. Chem., 283, 26436-26443.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 26436-26443
-
-
Winborn, B.J.1
Travis, S.M.2
Todi, S.V.3
Scaglione, K.M.4
Xu, P.5
Williams, A.J.6
Cohen, R.E.7
Peng, J.8
Paulson, H.L.9
-
38
-
-
60549100850
-
Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3
-
Todi, S.V., Winborn, B.J., Scaglione, K.M., Blount, J.R., Travis, S.M. and Paulson, H.L. (2009) Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J., 28, 372-382.
-
(2009)
EMBO J.
, vol.28
, pp. 372-382
-
-
Todi, S.V.1
Winborn, B.J.2
Scaglione, K.M.3
Blount, J.R.4
Travis, S.M.5
Paulson, H.L.6
-
39
-
-
0042808497
-
Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates
-
Donaldson, K.M., Li, W., Ching, K.A., Batalov, S., Tsai, C.C. and Joazeiro, C.A. (2003) Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates. Proc. Natl Acad. Sci. USA, 100, 8892-8897.
-
(2003)
Proc. Natl Acad. Sci. USA
, vol.100
, pp. 8892-8897
-
-
Donaldson, K.M.1
Li, W.2
Ching, K.A.3
Batalov, S.4
Tsai, C.C.5
Joazeiro, C.A.6
-
40
-
-
0035034417
-
UBA domains of DNA damage-inducible proteins interact with ubiquitin
-
Bertolaet, B.L., Clarke, D.J., Wolff, M., Watson, M.H., Henze, M., Divita, G. and Reed, S.I. (2001) UBA domains of DNA damage-inducible proteins interact with ubiquitin. Nat. Struct. Biol., 8, 417-422.
-
(2001)
Nat. Struct. Biol.
, vol.8
, pp. 417-422
-
-
Bertolaet, B.L.1
Clarke, D.J.2
Wolff, M.3
Watson, M.H.4
Henze, M.5
Divita, G.6
Reed, S.I.7
-
41
-
-
0034798985
-
Proteins containing the UBA domain are able to bind to multi-ubiquitin chains
-
Wilkinson, C.R., Seeger, M., Hartmann-Petersen, R., Stone, M., Wallace, M., Semple, C. and Gordon, C. (2001) Proteins containing the UBA domain are able to bind to multi-ubiquitin chains. Nat. Cell. Biol., 3, 939-943.
-
(2001)
Nat. Cell. Biol.
, vol.3
, pp. 939-943
-
-
Wilkinson, C.R.1
Seeger, M.2
Hartmann-Petersen, R.3
Stone, M.4
Wallace, M.5
Semple, C.6
Gordon, C.7
-
42
-
-
0036277299
-
Rad23 promotes the targeting of proteolytic substrates to the proteasome
-
Chen, L. and Madura, K. (2002) Rad23 promotes the targeting of proteolytic substrates to the proteasome. Mol. Cell. Biol., 22, 4902-4913.
-
(2002)
Mol. Cell. Biol.
, vol.22
, pp. 4902-4913
-
-
Chen, L.1
Madura, K.2
-
43
-
-
0037023716
-
Recognition of specific ubiquitin conjugates is important for the proteolytic functions of the ubiquitin-associated domain proteins Dsk2 and Rad23
-
Rao, H. and Sastry, A. (2002) Recognition of specific ubiquitin conjugates is important for the proteolytic functions of the ubiquitin-associated domain proteins Dsk2 and Rad23. J. Biol. Chem., 277, 11691-11695.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 11691-11695
-
-
Rao, H.1
Sastry, A.2
-
44
-
-
68749103450
-
CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3
-
Mueller, T., Breuer, P., Schmitt, I., Walter, J., Evert, B.O. and Wullner, U. (2009) CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3. Hum. Mol. Genet., 18, 3334-3343.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 3334-3343
-
-
Mueller, T.1
Breuer, P.2
Schmitt, I.3
Walter, J.4
Evert, B.O.5
Wullner, U.6
-
45
-
-
67149129949
-
Nucleocytoplasmic shuttling activity of ataxin-3
-
Macedo-Ribeiro, S., Cortes, L., Maciel, P. and Carvalho, A.L. (2009) Nucleocytoplasmic shuttling activity of ataxin-3. PLoS One., 4, e5834.
-
(2009)
PLoS One.
, vol.4
-
-
Macedo-Ribeiro, S.1
Cortes, L.2
Maciel, P.3
Carvalho, A.L.4
-
46
-
-
70349780383
-
Identification and functional dissection of localization signals within ataxin-3
-
Antony, P.M., Mantele, S., Mollenkopf, P., Boy, J., Kehlenbach, R.H., Riess, O. and Schmidt, T. (2009) Identification and functional dissection of localization signals within ataxin-3. Neurobiol. Dis., 36, 280-292.
-
(2009)
Neurobiol. Dis.
, vol.36
, pp. 280-292
-
-
Antony, P.M.1
Mantele, S.2
Mollenkopf, P.3
Boy, J.4
Kehlenbach, R.H.5
Riess, O.6
Schmidt, T.7
-
47
-
-
0025955517
-
Stress-induced oligomerization and chromosomal relocalization of heat-shock factor
-
Westwood, J.T., Clos, J. and Wu, C. (1991) Stress-induced oligomerization and chromosomal relocalization of heat-shock factor. Nature, 353, 822-827.
-
(1991)
Nature
, vol.353
, pp. 822-827
-
-
Westwood, J.T.1
Clos, J.2
Wu, C.3
-
48
-
-
0027474909
-
Activation of human heat shock genes is accompanied by oligomerization, modification, and rapid translocation of heat shock transcription factor HSF1
-
Baler, R., Dahl, G. and Voellmy, R. (1993) Activation of human heat shock genes is accompanied by oligomerization, modification, and rapid translocation of heat shock transcription factor HSF1. Mol. Cell. Biol., 13, 2486-2496.
-
(1993)
Mol. Cell. Biol.
, vol.13
, pp. 2486-2496
-
-
Baler, R.1
Dahl, G.2
Voellmy, R.3
-
49
-
-
0027461364
-
Activation of heat shock gene transcription by heat shock factor 1 involves oligomerization, acquisition of DNA-binding activity, and nuclear localization and can occur in the absence of stress
-
Sarge, K.D., Murphy, S.P. and Morimoto, R.I. (1993) Activation of heat shock gene transcription by heat shock factor 1 involves oligomerization, acquisition of DNA-binding activity, and nuclear localization and can occur in the absence of stress. Mol. Cell. Biol., 13, 1392-1407.
-
(1993)
Mol. Cell. Biol.
, vol.13
, pp. 1392-1407
-
-
Sarge, K.D.1
Murphy, S.P.2
Morimoto, R.I.3
-
50
-
-
18244384703
-
Analysis of phosphorylation of human heat shock factor 1 in cells experiencing a stress
-
Guettouche, T., Boellmann, F., Lane, W.S. and Voellmy, R. (2005) Analysis of phosphorylation of human heat shock factor 1 in cells experiencing a stress. BMC Biochem., 6,4.
-
(2005)
BMC Biochem.
, vol.6
, pp. 4
-
-
Guettouche, T.1
Boellmann, F.2
Lane, W.S.3
Voellmy, R.4
-
51
-
-
16844366162
-
Polo-like kinase 1 phosphorylates heat shock transcription factor 1 and mediates its nuclear translocation during heat stress
-
Kim, S.A., Yoon, J.H., Lee, S.H. and Ahn, S.G. (2005) Polo-like kinase 1 phosphorylates heat shock transcription factor 1 and mediates its nuclear translocation during heat stress. J. Biol. Chem., 280, 12653-12657.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 12653-12657
-
-
Kim, S.A.1
Yoon, J.H.2
Lee, S.H.3
Ahn, S.G.4
-
52
-
-
0038492408
-
Identification of a consensus motif for Plk (Polo-like kinase) phosphorylation reveals Myt1 as a Plk1 substrate
-
Nakajima, H., Toyoshima-Morimoto, F., Taniguchi, E. and Nishida, E. (2003) Identification of a consensus motif for Plk (Polo-like kinase) phosphorylation reveals Myt1 as a Plk1 substrate. J. Biol. Chem., 278, 25277-25280.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 25277-25280
-
-
Nakajima, H.1
Toyoshima-Morimoto, F.2
Taniguchi, E.3
Nishida, E.4
-
53
-
-
34548058372
-
Pharmacological and functional comparison of the polo-like kinase family: insight into inhibitor and substrate specificity
-
Johnson, E.F., Stewart, K.D., Woods, K.W., Giranda, V.L. and Luo, Y. (2007) Pharmacological and functional comparison of the polo-like kinase family: insight into inhibitor and substrate specificity. Biochemistry, 46, 9551-9563.
-
(2007)
Biochemistry
, vol.46
, pp. 9551-9563
-
-
Johnson, E.F.1
Stewart, K.D.2
Woods, K.W.3
Giranda, V.L.4
Luo, Y.5
-
54
-
-
0342635463
-
Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease
-
Perez-Severiano, F., Rios, C. and Segovia, J. (2000) Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease. Brain Res., 862, 234-237.
-
(2000)
Brain Res.
, vol.862
, pp. 234-237
-
-
Perez-Severiano, F.1
Rios, C.2
Segovia, J.3
-
55
-
-
0035668684
-
Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease
-
Bogdanov, M.B., Andreassen, O.A., Dedeoglu, A., Ferrante, R.J. and Beal, M.F. (2001) Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease. J. Neurochem., 79, 1246-1249.
-
(2001)
J. Neurochem.
, vol.79
, pp. 1246-1249
-
-
Bogdanov, M.B.1
Andreassen, O.A.2
Dedeoglu, A.3
Ferrante, R.J.4
Beal, M.F.5
-
56
-
-
29244479196
-
Proteomic analysis of protein expression and oxidative modification in r6/2 transgenic mice: a model of Huntington disease
-
Perluigi, M., Poon, H.F., Maragos, W., Pierce, W.M., Klein, J.B., Calabrese, V., Cini, C., De Marco, C. and Butterfield, D.A. (2005) Proteomic analysis of protein expression and oxidative modification in r6/2 transgenic mice: a model of Huntington disease. Mol. Cell. Proteomics., 4, 1849-1861.
-
(2005)
Mol. Cell. Proteomics.
, vol.4
, pp. 1849-1861
-
-
Perluigi, M.1
Poon, H.F.2
Maragos, W.3
Pierce, W.M.4
Klein, J.B.5
Calabrese, V.6
Cini, C.7
De Marco, C.8
Butterfield, D.A.9
-
57
-
-
36549083344
-
Oxidative stress in neurodegeneration in dentatorubral-pallidoluysian atrophy
-
Miyata, R., Hayashi, M., Tanuma, N., Shioda, K., Fukatsu, R. and Mizutani, S. (2008) Oxidative stress in neurodegeneration in dentatorubral-pallidoluysian atrophy. J. Neurol. Sci., 264, 133-139.
-
(2008)
J. Neurol. Sci.
, vol.264
, pp. 133-139
-
-
Miyata, R.1
Hayashi, M.2
Tanuma, N.3
Shioda, K.4
Fukatsu, R.5
Mizutani, S.6
-
58
-
-
66049150300
-
Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease
-
Yu, Y.C., Kuo, C.L., Cheng, W.L., Liu, C.S. and Hsieh, M. (2009) Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease. J. Neurosci. Res., 87, 1884-1891.
-
(2009)
J. Neurosci. Res.
, vol.87
, pp. 1884-1891
-
-
Yu, Y.C.1
Kuo, C.L.2
Cheng, W.L.3
Liu, C.S.4
Hsieh, M.5
-
59
-
-
0032504710
-
Mitochondrial dysfunction in neurodegenerative diseases
-
Beal, M.F. (1998) Mitochondrial dysfunction in neurodegenerative diseases. Biochim. Biophys. Acta., 1366, 211-223.
-
(1998)
Biochim. Biophys. Acta.
, vol.1366
, pp. 211-223
-
-
Beal, M.F.1
-
60
-
-
39349083915
-
Adapting proteostasis for disease intervention
-
Balch, W.E., Morimoto, R.I., Dillin, A. and Kelly, J.W. (2008) Adapting proteostasis for disease intervention. Science., 319, 916-919.
-
(2008)
Science
, vol.319
, pp. 916-919
-
-
Balch, W.E.1
Morimoto, R.I.2
Dillin, A.3
Kelly, J.W.4
-
61
-
-
44849094781
-
Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging
-
Morimoto, R.I. (2008) Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging. Genes. Dev., 22, 1427-1438.
-
(2008)
Genes. Dev.
, vol.22
, pp. 1427-1438
-
-
Morimoto, R.I.1
-
62
-
-
69749123786
-
Fine-tuning of the unfolded protein response: assembling the IRE1alpha interactome
-
Hetz, C. and Glimcher, L.H. (2009) Fine-tuning of the unfolded protein response: assembling the IRE1alpha interactome. Mol. Cell., 35, 551-561.
-
(2009)
Mol. Cell.
, vol.35
, pp. 551-561
-
-
Hetz, C.1
Glimcher, L.H.2
-
63
-
-
0033559256
-
A leucine-rich nuclear export signal in the p53 tetramerization domain: regulation of subcellular localization and p53 activity by NES masking
-
Stommel, J.M., Marchenko, N.D., Jimenez, G.S., Moll, U.M., Hope, T.J. and Wahl, G.M. (1999) A leucine-rich nuclear export signal in the p53 tetramerization domain: regulation of subcellular localization and p53 activity by NES masking. EMBO J., 18, 1660-1672.
-
(1999)
EMBO J.
, vol.18
, pp. 1660-1672
-
-
Stommel, J.M.1
Marchenko, N.D.2
Jimenez, G.S.3
Moll, U.M.4
Hope, T.J.5
Wahl, G.M.6
-
64
-
-
13244249573
-
Polo-like kinases in the nervous system
-
Seeburg, D.P., Pak, D. and Sheng, M. (2005) Polo-like kinases in the nervous system. Oncogene, 24, 292-298.
-
(2005)
Oncogene
, vol.24
, pp. 292-298
-
-
Seeburg, D.P.1
Pak, D.2
Sheng, M.3
-
65
-
-
35448994487
-
Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity
-
Atwal, R.S., Xia, J., Pinchev, D., Taylor, J., Epand, R.M. and Truant, R. (2007) Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity. Hum. Mol. Genet., 16, 2600-2615.
-
(2007)
Hum. Mol. Genet.
, vol.16
, pp. 2600-2615
-
-
Atwal, R.S.1
Xia, J.2
Pinchev, D.3
Taylor, J.4
Epand, R.M.5
Truant, R.6
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