-
1
-
-
34547692622
-
Trinucleotide repeat disorders
-
2-s2.0-34547692622
-
Orr H. T., Zoghbi H. Y., Trinucleotide repeat disorders. Annual Review of Neuroscience 2007 30 575 621 10.1146/annurev.neuro.29.051605.113042 2-s2.0-34547692622
-
(2007)
Annual Review of Neuroscience
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
2
-
-
68149134683
-
Nuclear accumulation of polyglutamine disease proteins and neuropathology
-
2-s2.0-68149134683
-
Havel L. S., Li S., Li X.-J., Nuclear accumulation of polyglutamine disease proteins and neuropathology. Molecular Brain 2009 2 1, article 21 10.1186/1756-6606-2-21 2-s2.0-68149134683
-
(2009)
Molecular Brain
, vol.2
, Issue.1 ARTICLE 21
-
-
Havel, L.S.1
Li, S.2
Li, X.-J.3
-
3
-
-
0033499931
-
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
-
2-s2.0-0033499931
-
Chai Y., Koppenhafer S. L., Bonini N. M., Paulson H. L., Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. Journal of Neuroscience 1999 19 23 10338 10347 2-s2.0-0033499931
-
(1999)
Journal of Neuroscience
, vol.19
, Issue.23
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
4
-
-
0034110465
-
Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila
-
2-s2.0-0034110465
-
Marsh J. L., Walker H., Theisen H., Zhu Y.-Z., Fielder T., Purcell J., Thompson L. M., Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila. Human Molecular Genetics 2000 9 1 13 25 10.1093/hmg/9.1.13 2-s2.0-0034110465
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.1
, pp. 13-25
-
-
Marsh, J.L.1
Walker, H.2
Theisen, H.3
Zhu, Y.-Z.4
Fielder, T.5
Purcell, J.6
Thompson, L.M.7
-
5
-
-
33746635502
-
Flanking sequences profoundly after polyglutamine toxicity in yeast
-
2-s2.0-33746635502
-
Duennwald M. L., Jagadish S., Muchowski P. J., Lindquist S., Flanking sequences profoundly after polyglutamine toxicity in yeast. Proceedings of the National Academy of Sciences of the United States of America 2006 103 29 11045 11050 10.1073/pnas.0604547103 2-s2.0-33746635502
-
(2006)
Proceedings of the National Academy of Sciences of the United States of America
, vol.103
, Issue.29
, pp. 11045-11050
-
-
Duennwald, M.L.1
Jagadish, S.2
Muchowski, P.J.3
Lindquist, S.4
-
6
-
-
84866139184
-
Polyglutamine (polyQ) disorders: The chromatin connection
-
2-s2.0-84874153820
-
Cohen-Carmon D., Meshorer E., Polyglutamine (polyQ) disorders: the chromatin connection. Nucleus 2012 3 5 433 441 2-s2.0-84874153820
-
(2012)
Nucleus
, vol.3
, Issue.5
, pp. 433-441
-
-
Cohen-Carmon, D.1
Meshorer, E.2
-
7
-
-
0037314215
-
Neuronal degeneration and mitochondrial dysfunction
-
2-s2.0-0037314215
-
Schon E. A., Manfredi G., Neuronal degeneration and mitochondrial dysfunction. Journal of Clinical Investigation 2003 111 3 303 312 10.1172/JCI200317741 2-s2.0-0037314215
-
(2003)
Journal of Clinical Investigation
, vol.111
, Issue.3
, pp. 303-312
-
-
Schon, E.A.1
Manfredi, G.2
-
8
-
-
0030058208
-
Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo
-
2-s2.0-0030058208
-
Ikeda H., Yamaguchi M., Sugai S., Aze Y., Narumiya S., Kakizuka A., Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nature Genetics 1996 13 2 198 202 2-s2.0-0030058208
-
(1996)
Nature Genetics
, vol.13
, Issue.2
, pp. 198-202
-
-
Ikeda, H.1
Yamaguchi, M.2
Sugai, S.3
Aze, Y.4
Narumiya, S.5
Kakizuka, A.6
-
9
-
-
16044373842
-
Exon i of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
2-s2.0-16044373842
-
Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S. W., Bates G. P., Exon I of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996 87 3 493 506 10.1016/S0092-8674(00)81369-0 2-s2.0-16044373842
-
(1996)
Cell
, vol.87
, Issue.3
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
Cozens, B.4
Harper, A.5
Hetherington, C.6
Lawton, M.7
Trottier, Y.8
Lehrach, H.9
Davies, S.W.10
Bates, G.P.11
-
10
-
-
0030791305
-
Of molecular interactions, mice and mechanisms: New insights into Huntington's disease
-
2-s2.0-0030791305
-
Wellington C. L., Hayden M. R., Of molecular interactions, mice and mechanisms: new insights into Huntington's disease. Current Opinion in Neurology 1997 10 4 291 298 10.1097/00019052-199708000-00003 2-s2.0-0030791305
-
(1997)
Current Opinion in Neurology
, vol.10
, Issue.4
, pp. 291-298
-
-
Wellington, C.L.1
Hayden, M.R.2
-
11
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutarnine- induced disease in SCA1 transgenic mice
-
2-s2.0-0032475941
-
Klement I. A., Skinner P. J., Kaytor M. D., Yi H., Hersch S. M., Clark H. B., Zoghbi H. Y., Orr H. T., Ataxin-1 nuclear localization and aggregation: role in polyglutarnine- induced disease in SCA1 transgenic mice. Cell 1998 95 1 41 53 10.1016/S0092-8674(00)81781-X 2-s2.0-0032475941
-
(1998)
Cell
, vol.95
, Issue.1
, 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
-
12
-
-
0033811788
-
Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human
-
2-s2.0-0033811788
-
Huynh D. P., Figueroa K., Hoang N., Pulst S.-M., Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nature Genetics 2000 26 1 44 50 10.1038/79162 2-s2.0-0033811788
-
(2000)
Nature Genetics
, vol.26
, Issue.1
, pp. 44-50
-
-
Huynh, D.P.1
Figueroa, K.2
Hoang, N.3
Pulst, S.-M.4
-
13
-
-
20844462057
-
A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration
-
2-s2.0-20844462057
-
Goti D., Katzen S. M., Mez J., Kurtis N., Kiluk J., Ben-Haïem L., Jenkins N. A., Copeland N. G., Kakizuka A., Sharp A. H., Ross C. A., Mouton P. R., Colomer V., A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration. Journal of Neuroscience 2004 24 45 10266 10279 10.1523/JNEUROSCI.2734-04.2004 2-s2.0-20844462057
-
(2004)
Journal of Neuroscience
, vol.24
, Issue.45
, pp. 10266-10279
-
-
Goti, D.1
Katzen, S.M.2
Mez, J.3
Kurtis, N.4
Kiluk, J.5
Ben-Haïem, L.6
Jenkins, N.A.7
Copeland, N.G.8
Kakizuka, A.9
Sharp, A.H.10
Ross, C.A.11
Mouton, P.R.12
Colomer, V.13
-
14
-
-
34548329581
-
A Mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice
-
2-s2.0-34548329581
-
Colomer Gould V. F., Goti D., Pearce D., Gonzalez G. A., Gao H., Bermudez de Leon M., Jenkins N. A., Copeland N. G., Ross C. A., Brown D. R., A Mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice. Neurobiology of Disease 2007 27 3 362 369 10.1016/j.nbd.2007.06.005 2-s2.0-34548329581
-
(2007)
Neurobiology of Disease
, vol.27
, Issue.3
, pp. 362-369
-
-
Colomer Gould, V.F.1
Goti, D.2
Pearce, D.3
Gonzalez, G.A.4
Gao, H.5
Bermudez De Leon, M.6
Jenkins, N.A.7
Copeland, N.G.8
Ross, C.A.9
Brown, D.R.10
-
15
-
-
0037464386
-
Proteolytic cleavage and cellular toxicity of the human &;1A calcium channel in spinocerebellar ataxia type 6
-
2-s2.0-0037464386
-
Kubodera T., Yokota T., Ohwada K., Ishikawa K., Miura H., Matsuoka T., Mizusawa H., Proteolytic cleavage and cellular toxicity of the human &1A calcium channel in spinocerebellar ataxia type 6. Neuroscience Letters 2003 341 1 74 78 10.1016/S0304-3940(03)00156-3 2-s2.0-0037464386
-
(2003)
Neuroscience Letters
, vol.341
, Issue.1
, pp. 74-78
-
-
Kubodera, T.1
Yokota, T.2
Ohwada, K.3
Ishikawa, K.4
Miura, H.5
Matsuoka, T.6
Mizusawa, H.7
-
16
-
-
0034641891
-
Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice
-
2-s2.0-0034641891
-
Yvert G., Lindenberg K. S., Picaud S., Landwehrmeyer G. B., Sahel J.-A., Mandel J.-L., Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice. Human Molecular Genetics 2000 9 17 2491 2506 10.1093/hmg/9.17.2491 2-s2.0-0034641891
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.17
, pp. 2491-2506
-
-
Yvert, G.1
Lindenberg, K.S.2
Picaud, S.3
Landwehrmeyer, G.B.4
Sahel, J.-A.5
Mandel, J.-L.6
-
17
-
-
0037096365
-
Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice
-
2-s2.0-0037096365
-
Garden G. A., Libby R. T., Fu Y.-H., Kinoshita Y., Huang J., Possin D. E., Smith A. C., Martinez R. A., Fine G. C., Grote S. K., Ware C. B., Einum D. D., Morrison R. S., Ptacek L. J., Sopher B. L., La Spada A. R., Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice. Journal of Neuroscience 2002 22 12 4897 4905 2-s2.0-0037096365
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.12
, pp. 4897-4905
-
-
Garden, G.A.1
Libby, R.T.2
Fu, Y.-H.3
Kinoshita, Y.4
Huang, J.5
Possin, D.E.6
Smith, A.C.7
Martinez, R.A.8
Fine, G.C.9
Grote, S.K.10
Ware, C.B.11
Einum, D.D.12
Morrison, R.S.13
Ptacek, L.J.14
Sopher, B.L.15
La Spada, A.R.16
-
18
-
-
43749091298
-
Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity
-
2-s2.0-43749091298
-
Friedman M. J., Wang C.-E., Li X.-J., Li S., Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity. Journal of Biological Chemistry 2008 283 13 8283 8290 10.1074/jbc.M709674200 2-s2.0-43749091298
-
(2008)
Journal of Biological Chemistry
, vol.283
, Issue.13
, pp. 8283-8290
-
-
Friedman, M.J.1
Wang, C.-E.2
Li, X.-J.3
Li, S.4
-
19
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
2-s2.0-0033119123
-
Gutekunst C.-A., Li S.-H., Yi H., Mulroy J. S., Kuemmerle S., Jones R., Rye D., Ferrante R. J., Hersch S. M., Li X.-J., Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. Journal of Neuroscience 1999 19 7 2522 2534 2-s2.0-0033119123
-
(1999)
Journal of Neuroscience
, vol.19
, Issue.7
, 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
-
20
-
-
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 calpaindependent proteolysis
-
2-s2.0-0035940412
-
Kim Y. J., Yi Y., Sapp E., Wang Y., Cuiffo B., Kegel K. B., Qin Z.-H., Aronin N., DiFiglia M., 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 calpaindependent proteolysis. Proceedings of the National Academy of Sciences of the United States of America 2001 98 22 12784 12789 10.1073/pnas.221451398 2-s2.0-0035940412
-
(2001)
Proceedings of the National Academy of Sciences of the United States of America
, vol.98
, Issue.22
, pp. 12784-12789
-
-
Kim, Y.J.1
Yi, Y.2
Sapp, E.3
Wang, Y.4
Cuiffo, B.5
Kegel, K.B.6
Qin, Z.-H.7
Aronin, N.8
Difiglia, M.9
-
21
-
-
9344227302
-
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
-
2-s2.0-9344227302
-
Goldberg Y. P., Nicholson D. W., Rasper D. M., Kalchman M. A., Koide H. B., Graham R. K., Bromm M., Kazemi-Esfarjani P., Thornberry N. A., Vaillancourt J. P., Hayden M. R., Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genetics 1996 13 4 442 449 10.1038/ng0896-442 2-s2.0-9344227302
-
(1996)
Nature Genetics
, vol.13
, Issue.4
, pp. 442-449
-
-
Goldberg, Y.P.1
Nicholson, D.W.2
Rasper, D.M.3
Kalchman, M.A.4
Koide, H.B.5
Graham, R.K.6
Bromm, M.7
Kazemi-Esfarjani, P.8
Thornberry, N.A.9
Vaillancourt, J.P.10
Hayden, M.R.11
-
22
-
-
0032502715
-
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
-
2-s2.0-0032502715
-
Wellington C. L., Ellerby L. M., Hackam A. S., Margolis R. L., Trifiro M. A., Singaraja R., McCutcheon K., Salvesen G. S., Propp S. S., Bromm M., Rowland K. J., Zhang T., Rasper D., Roy S., Thornberry N., Pinsky L., Kakizuka A., Ross C. A., Nicholson D. W., Bredesen D. E., Hayden M. R., Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. Journal of Biological Chemistry 1998 273 15 9158 9167 10.1074/jbc.273.15.9158 2-s2.0-0032502715
-
(1998)
Journal of Biological Chemistry
, vol.273
, Issue.15
, pp. 9158-9167
-
-
Wellington, C.L.1
Ellerby, L.M.2
Hackam, A.S.3
Margolis, R.L.4
Trifiro, M.A.5
Singaraja, R.6
McCutcheon, K.7
Salvesen, G.S.8
Propp, S.S.9
Bromm, M.10
Rowland, K.J.11
Zhang, T.12
Rasper, D.13
Roy, S.14
Thornberry, N.15
Pinsky, L.16
Kakizuka, A.17
Ross, C.A.18
Nicholson, D.W.19
Bredesen, D.E.20
Hayden, M.R.21
more..
-
23
-
-
0037107151
-
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease
-
2-s2.0-0037107151
-
Wellington C. L., Ellerby L. M., Gutekunst C.-A., Rogers D., Warby S., Graham R. K., Loubser O., Van Raamsdonk J., Singaraja R., Yang Y.-Z., Gafni J., Bredesen D., Hersch S. M., Leavitt B. R., Roy S., Nicholson D. W., Hayden M. R., Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. Journal of Neuroscience 2002 22 18 7862 7872 2-s2.0-0037107151
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.18
, pp. 7862-7872
-
-
Wellington, C.L.1
Ellerby, L.M.2
Gutekunst, C.-A.3
Rogers, D.4
Warby, S.5
Graham, R.K.6
Loubser, O.7
Van Raamsdonk, J.8
Singaraja, R.9
Yang, Y.-Z.10
Gafni, J.11
Bredesen, D.12
Hersch, S.M.13
Leavitt, B.R.14
Roy, S.15
Nicholson, D.W.16
Hayden, M.R.17
-
24
-
-
35648964788
-
Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation
-
2-s2.0-35648964788
-
Young J. E., Gouw L., Propp S., Sopher B. L., Taylor J., Lin A., Hermel E., Logvinova A., Chen S. F., Chen S., Bredesen D. E., Truant R., Ptacek L. J., La Spada A. R., Ellerby L. M., Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation. Journal of Biological Chemistry 2007 282 41 30150 30160 10.1074/jbc.M705265200 2-s2.0-35648964788
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.41
, pp. 30150-30160
-
-
Young, J.E.1
Gouw, L.2
Propp, S.3
Sopher, B.L.4
Taylor, J.5
Lin, A.6
Hermel, E.7
Logvinova, A.8
Chen, S.F.9
Chen, S.10
Bredesen, D.E.11
Truant, R.12
Ptacek, L.J.13
La Spada, A.R.14
Ellerby, L.M.15
-
25
-
-
0037096376
-
Calpain Activation in Huntington's Disease
-
2-s2.0-0037096376
-
Gafni J., Ellerby L. M., Calpain Activation in Huntington's Disease. Journal of Neuroscience 2002 22 12 4842 4849 2-s2.0-0037096376
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.12
, pp. 4842-4849
-
-
Gafni, J.1
Ellerby, L.M.2
-
26
-
-
2442631459
-
Inhibition of calpain cleavage of Huntingtin reduces toxicity: Accumulation of calpain/caspase fragments in the nucleus
-
2-s2.0-2442631459
-
Gafni J., Hermel E., Young J. E., Wellington C. L., Hayden M. R., Ellerby L. M., Inhibition of calpain cleavage of Huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. Journal of Biological Chemistry 2004 279 19 20211 20220 10.1074/jbc.M401267200 2-s2.0-2442631459
-
(2004)
Journal of Biological Chemistry
, vol.279
, Issue.19
, pp. 20211-20220
-
-
Gafni, J.1
Hermel, E.2
Young, J.E.3
Wellington, C.L.4
Hayden, M.R.5
Ellerby, L.M.6
-
27
-
-
0037131263
-
Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons
-
2-s2.0-0037131263
-
Goffredo D., Rigamonti D., Tartari M., De Micheli A., Verderio C., Matteoli M., Zuccato C., Cattaneo E., Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons. Journal of Biological Chemistry 2002 277 42 39594 39598 10.1074/jbc.C200353200 2-s2.0-0037131263
-
(2002)
Journal of Biological Chemistry
, vol.277
, Issue.42
, pp. 39594-39598
-
-
Goffredo, D.1
Rigamonti, D.2
Tartari, M.3
De Micheli, A.4
Verderio, C.5
Matteoli, M.6
Zuccato, C.7
Cattaneo, E.8
-
28
-
-
34547129609
-
Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3
-
2-s2.0-34547129609
-
Haacke A., Hartl F. U., Breuer P., Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3. Journal of Biological Chemistry 2007 282 26 18851 18856 10.1074/jbc.M611914200 2-s2.0-34547129609
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.26
, pp. 18851-18856
-
-
Haacke, A.1
Hartl, F.U.2
Breuer, P.3
-
29
-
-
84872416147
-
Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3)
-
2-s2.0-84872416147
-
Hübener J., Weber J. J., Richter C., Honold L., Weiss A., Murad F., Breuer P., Wüllner U., Bellstedt P., Paquet-Durand F., Takano J., Saido T. C., Riess O., Nguyen H. P., Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). Human Molecular Genetics 2013 22 3 508 518 10.1093/hmg/dds449 2-s2.0-84872416147
-
(2013)
Human Molecular Genetics
, vol.22
, Issue.3
, pp. 508-518
-
-
Hübener, J.1
Weber, J.J.2
Richter, C.3
Honold, L.4
Weiss, A.5
Murad, F.6
Breuer, P.7
Wüllner, U.8
Bellstedt, P.9
Paquet-Durand, F.10
Takano, J.11
Saido, T.C.12
Riess, O.13
Nguyen, H.P.14
-
30
-
-
0035968933
-
Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation
-
2-s2.0-0035968933
-
Nozaki K., Onodera O., Takano H., Tsuji S., Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation. NeuroReport 2001 12 15 3357 3364 10.1097/00001756-200110290-00042 2-s2.0-0035968933
-
(2001)
NeuroReport
, vol.12
, Issue.15
, pp. 3357-3364
-
-
Nozaki, K.1
Onodera, O.2
Takano, H.3
Tsuji, S.4
-
31
-
-
36049003085
-
Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat
-
2-s2.0-36049003085
-
Ng H., Pulst S.-M., Huynh D. P., Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat. Experimental Neurology 2007 208 2 207 215 10.1016/j.expneurol.2007.07.023 2-s2.0-36049003085
-
(2007)
Experimental Neurology
, vol.208
, Issue.2
, pp. 207-215
-
-
Ng, H.1
Pulst, S.-M.2
Huynh, D.P.3
-
32
-
-
0030850412
-
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
-
2-s2.0-0030850412
-
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., Pittman R. N., Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 1997 19 2 333 344 10.1016/S0896-6273(00)80943-5 2-s2.0-0030850412
-
(1997)
Neuron
, vol.19
, Issue.2
, 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
-
33
-
-
32144436256
-
Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3
-
2-s2.0-32144436256
-
Haacke A., Broadley S. A., Boteva R., Tzvetkov N., Hartl F. U., Breuer P., Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3. Human Molecular Genetics 2006 15 4 555 568 10.1093/hmg/ddi472 2-s2.0-32144436256
-
(2006)
Human Molecular Genetics
, vol.15
, Issue.4
, pp. 555-568
-
-
Haacke, A.1
Broadley, S.A.2
Boteva, R.3
Tzvetkov, N.4
Hartl, F.U.5
Breuer, P.6
-
34
-
-
0032101287
-
The influence of huntingtin protein size on nuclear localization and cellular toxicity
-
2-s2.0-0032101287
-
Hackam A. S., Singaraja R., Wellington C. L., Metzler M., McCutcheon K., Zhang T., Kalchman M., Hayden M. R., The influence of huntingtin protein size on nuclear localization and cellular toxicity. Journal of Cell Biology 1998 141 5 1097 1105 10.1083/jcb.141.5.1097 2-s2.0-0032101287
-
(1998)
Journal of Cell Biology
, vol.141
, Issue.5
, 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
-
35
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
2-s2.0-17344363559
-
Martindale D., Hackam A., Wieczorek A., Ellerby L., Wellington C., McCutcheon K., Singaraja R., Kazemi-Esfarjani P., Devon R., Kim S. U., Bredesen D. E., Tufaro F., Hayden M. R., Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nature Genetics 1998 18 2 150 154 10.1038/ng0298-150 2-s2.0-17344363559
-
(1998)
Nature Genetics
, vol.18
, Issue.2
, 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
Bredesen, D.E.11
Tufaro, F.12
Hayden, M.R.13
-
36
-
-
0036314167
-
Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain
-
2-s2.0-0036314167
-
Toneff T., Mende-Mueller L., Wu Y., Hwang S.-R., Bundey R., Thompson L. M., Chesselet M.-F., Hook V., Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain. Journal of Neurochemistry 2002 82 1 84 92 10.1046/j.1471-4159.2002.00940.x 2-s2.0-0036314167
-
(2002)
Journal of Neurochemistry
, vol.82
, Issue.1
, pp. 84-92
-
-
Toneff, T.1
Mende-Mueller, L.2
Wu, Y.3
Hwang, S.-R.4
Bundey, R.5
Thompson, L.M.6
Chesselet, M.-F.7
Hook, V.8
-
37
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
2-s2.0-0030752709
-
DiFiglia M., Sapp E., Chase K. O., Davies S. W., Bates G. P., Vonsattel J. P., Aronin N., Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997 277 5334 1990 1993 10.1126/science.277.5334.1990 2-s2.0-0030752709
-
(1997)
Science
, vol.277
, Issue.5334
, 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
-
38
-
-
7344234800
-
An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients
-
2-s2.0-7344234800
-
Schmidt T., Bernhard Landwehrmeyer G., Schmitt I., Trottier Y., Auburger G., Laccone F., Klockgether T., Völpel M., Epplen J. T., Schöls L., Riess O., An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients. Brain Pathology 1998 8 4 669 679 10.1111/j.1750-3639.1998.tb00193.x 2-s2.0-7344234800
-
(1998)
Brain Pathology
, vol.8
, Issue.4
, pp. 669-679
-
-
Schmidt, T.1
Bernhard Landwehrmeyer, G.2
Schmitt, I.3
Trottier, Y.4
Auburger, G.5
Laccone, F.6
Klockgether, T.7
Völpel, M.8
Epplen, J.T.9
Schöls, L.10
Riess, O.11
-
39
-
-
0033037919
-
Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein
-
2-s2.0-0033037919
-
Sieradzan K. A., Mechan A. O., Jones L., Wanker E. E., Nukina N., Mann D. M. A., Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein. Experimental Neurology 1999 156 1 92 99 10.1006/exnr.1998.7005 2-s2.0-0033037919
-
(1999)
Experimental Neurology
, vol.156
, Issue.1
, pp. 92-99
-
-
Sieradzan, K.A.1
Mechan, A.O.2
Jones, L.3
Wanker, E.E.4
Nukina, N.5
Mann, D.M.A.6
-
40
-
-
23844472610
-
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions
-
2-s2.0-23844472610
-
Slow E. J., Graham R. K., Osmand A. P., Devon R. S., Lu G., Deng Y., Pearson J., Vaid K., Bissada N., Wetzel R., Leavitt B. R., Hayden M. R., Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proceedings of the National Academy of Sciences of the United States of America 2005 102 32 11402 11407 10.1073/pnas.0503634102 2-s2.0-23844472610
-
(2005)
Proceedings of the National Academy of Sciences of the United States of America
, vol.102
, Issue.32
, pp. 11402-11407
-
-
Slow, E.J.1
Graham, R.K.2
Osmand, A.P.3
Devon, R.S.4
Lu, G.5
Deng, Y.6
Pearson, J.7
Vaid, K.8
Bissada, N.9
Wetzel, R.10
Leavitt, B.R.11
Hayden, M.R.12
-
41
-
-
79959694745
-
N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation
-
2-s2.0-79959694745
-
Hübener J., Vauti F., Funke C., Wolburg H., Ye Y., Schmidt T., Wolburg-Buchholz K., Schmitt I., Gardyan A., Drießen S., Arnold H.-H., Nguyen H. P., Riess O., N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation. Brain 2011 134 7 1925 1942 10.1093/brain/awr118 2-s2.0-79959694745
-
(2011)
Brain
, vol.134
, Issue.7
, pp. 1925-1942
-
-
Hübener, J.1
Vauti, F.2
Funke, C.3
Wolburg, H.4
Ye, Y.5
Schmidt, T.6
Wolburg-Buchholz, K.7
Schmitt, I.8
Gardyan, A.9
Drießen, S.10
Arnold, H.-H.11
Nguyen, H.P.12
Riess, O.13
-
42
-
-
54249096028
-
Caspases in apoptosis and beyond
-
2-s2.0-54249096028
-
Li J., Yuan J., Caspases in apoptosis and beyond. Oncogene 2008 27 48 6194 6206 10.1038/onc.2008.297 2-s2.0-54249096028
-
(2008)
Oncogene
, vol.27
, Issue.48
, pp. 6194-6206
-
-
Li, J.1
Yuan, J.2
-
44
-
-
0033912716
-
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
-
2-s2.0-0033912716
-
Chen M., Ona V. O., Li M., Ferrante R. J., Fink K. B., Zhu S., Bian J., Guo L., Farrell L. A., Hersch S. M., Hobbs W., Vonsattei J.-P., Cha J.-H. J., Friedlander R. M., Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nature Medicine 2000 6 7 797 801 10.1038/77528 2-s2.0-0033912716
-
(2000)
Nature Medicine
, vol.6
, Issue.7
, pp. 797-801
-
-
Chen, M.1
Ona, V.O.2
Li, M.3
Ferrante, R.J.4
Fink, K.B.5
Zhu, S.6
Bian, J.7
Guo, L.8
Farrell, L.A.9
Hersch, S.M.10
Hobbs, W.11
Vonsattei, J.-P.12
Cha, J.-H.J.13
Friedlander, R.M.14
-
45
-
-
11144357398
-
Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease
-
2-s2.0-11144357398
-
Hermel E., Gafni J., Propp S. S., Leavitt B. R., Wellington C. L., Young J. E., Hackam A. S., Logvinova A. V., Peel A. L., Chen S. F., Hook V., Singaraja R., Krajewski S., Goldsmith P. C., Ellerby H. M., Hayden M. R., Bredesen D. E., Ellerby L. M., Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death and Differentiation 2004 11 4 424 438 10.1038/sj.cdd.4401358 2-s2.0-11144357398
-
(2004)
Cell Death and Differentiation
, vol.11
, Issue.4
, pp. 424-438
-
-
Hermel, E.1
Gafni, J.2
Propp, S.S.3
Leavitt, B.R.4
Wellington, C.L.5
Young, J.E.6
Hackam, A.S.7
Logvinova, A.V.8
Peel, A.L.9
Chen, S.F.10
Hook, V.11
Singaraja, R.12
Krajewski, S.13
Goldsmith, P.C.14
Ellerby, H.M.15
Hayden, M.R.16
Bredesen, D.E.17
Ellerby, L.M.18
-
46
-
-
0033081766
-
Mutant Huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
2-s2.0-0033081766
-
Kim M., Lee H.-S., LaForet G., McIntyre C., Martin E. J., Chang P., Kim T. W., Williams M., Reddy P. H., Tagle D., Boyce F. M., Won L., Heller A., Aronin N., DiFiglia M., Mutant Huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. Journal of Neuroscience 1999 19 3 964 973 2-s2.0-0033081766
-
(1999)
Journal of Neuroscience
, vol.19
, Issue.3
, 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
Boyce, F.M.11
Won, L.12
Heller, A.13
Aronin, N.14
Difiglia, M.15
-
47
-
-
0029072690
-
Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
-
2-s2.0-0029072690
-
Portera-Cailliau C., Hedreen J. C., Price D. L., Koliatsos V. E., Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models. Journal of Neuroscience 1995 15 5 3775 3787 2-s2.0-0029072690
-
(1995)
Journal of Neuroscience
, vol.15
, Issue.5
, pp. 3775-3787
-
-
Portera-Cailliau, C.1
Hedreen, J.C.2
Price, D.L.3
Koliatsos, V.E.4
-
48
-
-
0031680014
-
A cellular model that recapitulates major pathogenic steps of Huntington's disease
-
2-s2.0-0031680014
-
Lunkes A., Mandel J.-L., A cellular model that recapitulates major pathogenic steps of Huntington's disease. Human Molecular Genetics 1998 7 9 1355 1361 10.1093/hmg/7.9.1355 2-s2.0-0031680014
-
(1998)
Human Molecular Genetics
, vol.7
, Issue.9
, pp. 1355-1361
-
-
Lunkes, A.1
Mandel, J.-L.2
-
49
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
2-s2.0-0033587128
-
Ona V. O., Li M., Vonsattel J. P. G., John Andrews L., Khan S. Q., Chung W. M., Frey A. S., Menon A. S., Li X.-J., Stieg P. E., Yuan J., Penney J. B., Young A. B., Cha J.-H. J., Friedlander R. M., Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 1999 399 6733 263 267 10.1038/20446 2-s2.0-0033587128
-
(1999)
Nature
, vol.399
, Issue.6733
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.G.3
John Andrews, L.4
Khan, S.Q.5
Chung, W.M.6
Frey, A.S.7
Menon, A.S.8
Li, X.-J.9
Stieg, P.E.10
Yuan, J.11
Penney, J.B.12
Young, A.B.13
Cha, J.-H.J.14
Friedlander, R.M.15
-
50
-
-
0034703874
-
Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis
-
2-s2.0-0034703874
-
Li S.-H., Lam S., Cheng A. L., Li X.-J., Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis. Human Molecular Genetics 2000 9 19 2859 2867 10.1093/hmg/9.19.2859 2-s2.0-0034703874
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.19
, pp. 2859-2867
-
-
Li, S.-H.1
Lam, S.2
Cheng, A.L.3
Li, X.-J.4
-
51
-
-
0344010993
-
Sequential activation of individual caspases, and of alterations in Bcl-2 proapoptotic signals in a mouse model of Huntington's disease
-
2-s2.0-0344010993
-
Zhang Y., Ona V. O., Li M., Drozda M., Dubois-Dauphin M., Przedborski S., Ferrante R. J., Friedlander R. M., Sequential activation of individual caspases, and of alterations in Bcl-2 proapoptotic signals in a mouse model of Huntington's disease. Journal of Neurochemistry 2003 87 5 1184 1192 10.1046/j.1471-4159.2003.02105.x 2-s2.0-0344010993
-
(2003)
Journal of Neurochemistry
, vol.87
, Issue.5
, pp. 1184-1192
-
-
Zhang, Y.1
Ona, V.O.2
Li, M.3
Drozda, M.4
Dubois-Dauphin, M.5
Przedborski, S.6
Ferrante, R.J.7
Friedlander, R.M.8
-
52
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
2-s2.0-18544410106
-
Davies S. W., Turmaine M., Cozens B. A., DiFiglia M., Sharp A. H., Ross C. A., Scherzinger E., Wanker E. E., Mangiarini L., Bates G. P., Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997 90 3 537 548 10.1016/S0092-8674(00)80513-9 2-s2.0-18544410106
-
(1997)
Cell
, vol.90
, Issue.3
, 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
-
53
-
-
30744474942
-
Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL
-
2-s2.0-30744474942
-
Chou A.-H., Yeh T.-H., Kuo Y.-L., Kao Y.-C., Jou M.-J., Hsu C.-Y., Tsai S.-R., Kakizuka A., Wang H.-L., Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL. Neurobiology of Disease 2006 21 2 333 345 10.1016/j.nbd.2005.07.011 2-s2.0-30744474942
-
(2006)
Neurobiology of Disease
, vol.21
, Issue.2
, pp. 333-345
-
-
Chou, A.-H.1
Yeh, T.-H.2
Kuo, Y.-L.3
Kao, Y.-C.4
Jou, M.-J.5
Hsu, C.-Y.6
Tsai, S.-R.7
Kakizuka, A.8
Wang, H.-L.9
-
54
-
-
0035888620
-
Similarities between spinocerebellar ataxia type 7 (SCA7) cell models and human brain: Proteins recruited in inclusions and activation of caspase-3
-
2-s2.0-0035888620
-
Zander C., Takahashi J., El Hachimi K. H., Fujigasaki H., Albanese V., Lebre A. S., Stevanin G., Duyckaerts C., Brice A., Similarities between spinocerebellar ataxia type 7 (SCA7) cell models and human brain: Proteins recruited in inclusions and activation of caspase-3. Human Molecular Genetics 2001 10 22 2569 2579 10.1093/hmg/10.22.2569 2-s2.0-0035888620
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.22
, pp. 2569-2579
-
-
Zander, C.1
Takahashi, J.2
El Hachimi, K.H.3
Fujigasaki, H.4
Albanese, V.5
Lebre, A.S.6
Stevanin, G.7
Duyckaerts, C.8
Brice, A.9
-
55
-
-
33847769390
-
A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes
-
2-s2.0-33847769390
-
Latouche M., Lasbleiz C., Martin E., Monnier V., Debeir T., Mouatt-Prigent A., Muriel M.-P., Morel L., Ruberg M., Brice A., Stevanin G., Tricoire H., A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes. Journal of Neuroscience 2007 27 10 2483 2492 10.1523/JNEUROSCI.5453-06.2007 2-s2.0-33847769390
-
(2007)
Journal of Neuroscience
, vol.27
, Issue.10
, pp. 2483-2492
-
-
Latouche, M.1
Lasbleiz, C.2
Martin, E.3
Monnier, V.4
Debeir, T.5
Mouatt-Prigent, A.6
Muriel, M.-P.7
Morel, L.8
Ruberg, M.9
Brice, A.10
Stevanin, G.11
Tricoire, H.12
-
56
-
-
78149487690
-
Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo
-
2-s2.0-78149487690
-
Graham R. K., Deng Y., Carroll J., Vaid K., Cowan C., Pouladi M. A., Metzler M., Bissada N., Wang L., Faull R. L. M., Gray M., Yang X. W., Raymond L. A., Hayden M. R., Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. Journal of Neuroscience 2010 30 45 15019 15029 10.1523/JNEUROSCI.2071-10.2010 2-s2.0-78149487690
-
(2010)
Journal of Neuroscience
, vol.30
, Issue.45
, pp. 15019-15029
-
-
Graham, R.K.1
Deng, Y.2
Carroll, J.3
Vaid, K.4
Cowan, C.5
Pouladi, M.A.6
Metzler, M.7
Bissada, N.8
Wang, L.9
Faull, R.L.M.10
Gray, M.11
Yang, X.W.12
Raymond, L.A.13
Hayden, M.R.14
-
57
-
-
33745003424
-
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin
-
2-s2.0-33745003424
-
Graham R. K., Deng Y., Slow E. J., Haigh B., Bissada N., Lu G., Pearson J., Shehadeh J., Bertram L., Murphy Z., Warby S. C., Doty C. N., Roy S., Wellington C. L., Leavitt B. R., Raymond L. A., Nicholson D. W., Hayden M. R., Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 2006 125 6 1179 1191 10.1016/j.cell.2006.04.026 2-s2.0-33745003424
-
(2006)
Cell
, vol.125
, Issue.6
, pp. 1179-1191
-
-
Graham, R.K.1
Deng, Y.2
Slow, E.J.3
Haigh, B.4
Bissada, N.5
Lu, G.6
Pearson, J.7
Shehadeh, J.8
Bertram, L.9
Murphy, Z.10
Warby, S.C.11
Doty, C.N.12
Roy, S.13
Wellington, C.L.14
Leavitt, B.R.15
Raymond, L.A.16
Nicholson, D.W.17
Hayden, M.R.18
-
58
-
-
74549181538
-
Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice
-
2-s2.0-74549181538
-
Milnerwood A. J., Gladding C. M., Pouladi M. A., Kaufman A. M., Hines R. M., Boyd J. D., Ko R. W. Y., Vasuta O. C., Graham R. K., Hayden M. R., Murphy T. H., Raymond L. A., Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron 2010 65 2 178 190 10.1016/j.neuron.2010.01.008 2-s2.0-74549181538
-
(2010)
Neuron
, vol.65
, Issue.2
, pp. 178-190
-
-
Milnerwood, A.J.1
Gladding, C.M.2
Pouladi, M.A.3
Kaufman, A.M.4
Hines, R.M.5
Boyd, J.D.6
Ko, R.W.Y.7
Vasuta, O.C.8
Graham, R.K.9
Hayden, M.R.10
Murphy, T.H.11
Raymond, L.A.12
-
59
-
-
84861630493
-
Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment
-
2-s2.0-84861630493
-
Gafni J., Papanikolaou T., Degiacomo F., Holcomb J., Chen S., Menalled L., Kudwa A., Fitzpatrick J., Miller S., Ramboz S., Tuunanen P. I., Lehtimäki K. K., Yang X. W., Park L., Kwak S., Howland D., Park H., Ellerby L. M., Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment. Journal of Neuroscience 2012 32 22 7454 7465 10.1523/JNEUROSCI.6379-11.2012 2-s2.0-84861630493
-
(2012)
Journal of Neuroscience
, vol.32
, Issue.22
, pp. 7454-7465
-
-
Gafni, J.1
Papanikolaou, T.2
Degiacomo, F.3
Holcomb, J.4
Chen, S.5
Menalled, L.6
Kudwa, A.7
Fitzpatrick, J.8
Miller, S.9
Ramboz, S.10
Tuunanen, P.I.11
Lehtimäki, K.K.12
Yang, X.W.13
Park, L.14
Kwak, S.15
Howland, D.16
Park, H.17
Ellerby, L.M.18
-
60
-
-
84867459264
-
Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 Knock-in mouse model of Huntington's disease
-
2-s2.0-84867459264, e4fd085bfc9973
-
Landles C., Weiss A., Franklin S., Howland D., Bates G. P., Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 Knock-in mouse model of Huntington's disease. PLoS Currents 2012 e4fd085bfc9973 10.1371/4fd085bfc9973 2-s2.0-84867459264
-
(2012)
PLoS Currents
-
-
Landles, C.1
Weiss, A.2
Franklin, S.3
Howland, D.4
Bates, G.P.5
-
61
-
-
2542445545
-
Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3
-
2-s2.0-2542445545
-
Berke S. J., Schmied F. A., Brunt E. R., Ellerby L. M., Paulson H. L., Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. Journal of Neurochemistry 2004 89 4 908 918 10.1111/j.1471-4159.2004.02369.x 2-s2.0-2542445545
-
(2004)
Journal of Neurochemistry
, vol.89
, Issue.4
, pp. 908-918
-
-
Berke, S.J.1
Schmied, F.A.2
Brunt, E.R.3
Ellerby, L.M.4
Paulson, H.L.5
-
62
-
-
42649132458
-
Study of subcellular localization and proteolysis of ataxin-3
-
2-s2.0-42649132458
-
Pozzi C., Valtorta M., Tedeschi G., Galbusera E., Pastori V., Bigi A., Nonnis S., Grassi E., Fusi P., Study of subcellular localization and proteolysis of ataxin-3. Neurobiology of Disease 2008 30 2 190 200 10.1016/j.nbd.2008.01.011 2-s2.0-42649132458
-
(2008)
Neurobiology of Disease
, vol.30
, Issue.2
, pp. 190-200
-
-
Pozzi, C.1
Valtorta, M.2
Tedeschi, G.3
Galbusera, E.4
Pastori, V.5
Bigi, A.6
Nonnis, S.7
Grassi, E.8
Fusi, P.9
-
63
-
-
70450191208
-
Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3
-
2-s2.0-70450191208
-
Jung J., Xu K., Lessing D., Bonini N. M., Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3. Human Molecular Genetics 2009 18 24 4843 4852 10.1093/hmg/ddp456 2-s2.0-70450191208
-
(2009)
Human Molecular Genetics
, vol.18
, Issue.24
, pp. 4843-4852
-
-
Jung, J.1
Xu, K.2
Lessing, D.3
Bonini, N.M.4
-
64
-
-
84902381345
-
CDK5 protects from caspase-induced ataxin-3 cleavage and neurodegeneration
-
2-s2.0-84896583584
-
Liman J., Deeg S., Voigt A., Voßfeldt H., Dohm C. P., Karch A., Weishaupt J., Schulz J. B., Bähr M., Kermer P., CDK5 protects from caspase-induced ataxin-3 cleavage and neurodegeneration. Journal of Neurochemistry 2014 129 6 1013 1023 10.1111/jnc.12684 2-s2.0-84896583584
-
(2014)
Journal of Neurochemistry
, vol.129
, Issue.6
, pp. 1013-1023
-
-
Liman, J.1
Deeg, S.2
Voigt, A.3
Voßfeldt, H.4
Dohm, C.P.5
Karch, A.6
Weishaupt, J.7
Schulz, J.B.8
Bähr, M.9
Kermer, P.10
-
65
-
-
84355161952
-
Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease
-
2-s2.0-84355161952
-
Koch P., Breuer P., Peitz M., Jungverdorben J., Kesavan J., Poppe D., Doerr J., Ladewig J., Mertens J., Tüting T., Hoffmann P., Klockgether T., Evert B. O., Wüllner U., Brüstle O., Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease. Nature 2011 480 7378 543 546 10.1038/nature10671 2-s2.0-84355161952
-
(2011)
Nature
, vol.480
, Issue.7378
, pp. 543-546
-
-
Koch, P.1
Breuer, P.2
Peitz, M.3
Jungverdorben, J.4
Kesavan, J.5
Poppe, D.6
Doerr, J.7
Ladewig, J.8
Mertens, J.9
Tüting, T.10
Hoffmann, P.11
Klockgether, T.12
Evert, B.O.13
Wüllner, U.14
Brüstle, O.15
-
67
-
-
84866685475
-
Calpains, mitochondria, and apoptosis
-
2-s2.0-84866685475
-
Smith M. A., Schnellmann R. G., Calpains, mitochondria, and apoptosis. Cardiovascular Research 2012 96 1 32 37 10.1093/cvr/cvs163 2-s2.0-84866685475
-
(2012)
Cardiovascular Research
, vol.96
, Issue.1
, pp. 32-37
-
-
Smith, M.A.1
Schnellmann, R.G.2
-
68
-
-
58149373434
-
Polyglutamine-modulated striatal calpain activity in YAC transgenic huntington disease mouse model: Impact on NMDA receptor function and toxicity
-
2-s2.0-58149373434
-
Cowan C. M., Fan M. M. Y., Fan J., Shehadeh J., Zhang L. Y. J., Graham R. K., Hayden M. R., Raymond L. A., Polyglutamine-modulated striatal calpain activity in YAC transgenic huntington disease mouse model: impact on NMDA receptor function and toxicity. Journal of Neuroscience 2008 28 48 12725 12735 2-s2.0-58149373434 10.1523/JNEUROSCI.4619-08.2008
-
(2008)
Journal of Neuroscience
, vol.28
, Issue.48
, pp. 12725-12735
-
-
Cowan, C.M.1
Fan, M.M.Y.2
Fan, J.3
Shehadeh, J.4
Zhang, L.Y.J.5
Graham, R.K.6
Hayden, M.R.7
Raymond, L.A.8
-
69
-
-
84865078592
-
Calpain and STriatal-Enriched protein tyrosine Phosphatase (STEP) activation contribute to extrasynaptic NMDA receptor localization in a huntington's disease mouse model
-
2-s2.0-84865078592
-
Gladding C. M., Sepers M. D., Xu J., Zhang L. Y. J., Milnerwood A. J., Lombroso P. J., Raymond L. A., Calpain and STriatal-Enriched protein tyrosine Phosphatase (STEP) activation contribute to extrasynaptic NMDA receptor localization in a huntington's disease mouse model. Human Molecular Genetics 2012 21 17 3739 3752 10.1093/hmg/dds154 2-s2.0-84865078592
-
(2012)
Human Molecular Genetics
, vol.21
, Issue.17
, pp. 3739-3752
-
-
Gladding, C.M.1
Sepers, M.D.2
Xu, J.3
Zhang, L.Y.J.4
Milnerwood, A.J.5
Lombroso, P.J.6
Raymond, L.A.7
-
70
-
-
84890027845
-
Chronic blockade of extrasynaptic NMDA receptors ameliorates synaptic dysfunction and pro-death signaling in Huntington disease transgenic mice
-
2-s2.0-84890027845
-
Dau A., Gladding C. M., Sepers M. D., Raymond L. A., Chronic blockade of extrasynaptic NMDA receptors ameliorates synaptic dysfunction and pro-death signaling in Huntington disease transgenic mice. Neurobiology of Disease 2014 62 533 542 10.1016/j.nbd.2013.11.013 2-s2.0-84890027845
-
(2014)
Neurobiology of Disease
, vol.62
, pp. 533-542
-
-
Dau, A.1
Gladding, C.M.2
Sepers, M.D.3
Raymond, L.A.4
-
71
-
-
77950584656
-
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in huntington disease
-
2-s2.0-77950584656
-
Landles C., Sathasivam K., Weiss A., Woodman B., Moffitt H., Finkbeiner S., Sun B., Gafni J., Ellerby L. M., Trottier Y., Richards W. G., Osmand A., Paganetti P., Bates G. P., Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in huntington disease. Journal of Biological Chemistry 2010 285 12 8808 8823 10.1074/jbc.M109.075028 2-s2.0-77950584656
-
(2010)
Journal of Biological Chemistry
, vol.285
, Issue.12
, pp. 8808-8823
-
-
Landles, C.1
Sathasivam, K.2
Weiss, A.3
Woodman, B.4
Moffitt, H.5
Finkbeiner, S.6
Sun, B.7
Gafni, J.8
Ellerby, L.M.9
Trottier, Y.10
Richards, W.G.11
Osmand, A.12
Paganetti, P.13
Bates, G.P.14
-
72
-
-
33747633422
-
Huntingtin phosphorylation sites mapped by mass spectrometry: Modulation of cleavage and toxicity
-
2-s2.0-33747633422
-
Schilling B., Gafni J., Torcassi C., Cong X., Row R. H., LaFevre-Bernt M. A., Cusack M. P., Ratovitski T., Hirschhorn R., Ross C. A., Gibson B. W., Ellerby L. M., Huntingtin phosphorylation sites mapped by mass spectrometry: modulation of cleavage and toxicity. Journal of Biological Chemistry 2006 281 33 23686 23697 10.1074/jbc.M513507200 2-s2.0-33747633422
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.33
, pp. 23686-23697
-
-
Schilling, B.1
Gafni, J.2
Torcassi, C.3
Cong, X.4
Row, R.H.5
Lafevre-Bernt, M.A.6
Cusack, M.P.7
Ratovitski, T.8
Hirschhorn, R.9
Ross, C.A.10
Gibson, B.W.11
Ellerby, L.M.12
-
73
-
-
0042190049
-
Huntingtin is degraded to small fragments by calpain after ischemic injury
-
2-s2.0-0042190049
-
Kim M., Roh J.-K., Yoon B. W., Kang L., Kim Y. J., Aronin N., DiFiglia M., Huntingtin is degraded to small fragments by calpain after ischemic injury. Experimental Neurology 2003 183 1 109 115 10.1016/S0014-4886(03)00132-8 2-s2.0-0042190049
-
(2003)
Experimental Neurology
, vol.183
, Issue.1
, pp. 109-115
-
-
Kim, M.1
Roh, J.-K.2
Yoon, B.W.3
Kang, L.4
Kim, Y.J.5
Aronin, N.6
Difiglia, M.7
-
74
-
-
84864658791
-
Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease
-
2-s2.0-84864658791
-
Simões A. T., Gonçalves N., Koeppen A., Déglon N., Kügler S., Duarte C. B., Pereira De Almeida L., Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease. Brain 2012 135 8 2428 2439 10.1093/brain/aws177 2-s2.0-84864658791
-
(2012)
Brain
, vol.135
, Issue.8
, pp. 2428-2439
-
-
Simões, A.T.1
Gonçalves, N.2
Koeppen, A.3
Déglon, N.4
Kügler, S.5
Duarte, C.B.6
Pereira De Almeida, L.7
-
75
-
-
33646121970
-
Lysosomal proteases are involved in generation of N-terminal huntingtin fragments
-
2-s2.0-33646121970
-
Kim Y. J., Sapp E., Cuiffo B. G., Sobin L., Yoder J., Kegel K. B., Qin Z.-H., Detloff P., Aronin N., DiFiglia M., Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiology of Disease 2006 22 2 346 356 10.1016/j.nbd.2005.11.012 2-s2.0-33646121970
-
(2006)
Neurobiology of Disease
, vol.22
, Issue.2
, pp. 346-356
-
-
Kim, Y.J.1
Sapp, E.2
Cuiffo, B.G.3
Sobin, L.4
Yoder, J.5
Kegel, K.B.6
Qin, Z.-H.7
Detloff, P.8
Aronin, N.9
Difiglia, M.10
-
76
-
-
0348013126
-
Autophagy regulates the processing of amino terminal huntingtin fragments
-
2-s2.0-0348013126
-
Qin Z.-H., Wang Y., Kegel K. B., Kazantsev A., Apostol B. L., Thompson L. M., Yoder J., Aronin N., DiFiglia M., Autophagy regulates the processing of amino terminal huntingtin fragments. Human Molecular Genetics 2003 12 24 3231 3244 10.1093/hmg/ddg346 2-s2.0-0348013126
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.24
, pp. 3231-3244
-
-
Qin, Z.-H.1
Wang, Y.2
Kegel, K.B.3
Kazantsev, A.4
Apostol, B.L.5
Thompson, L.M.6
Yoder, J.7
Aronin, N.8
Difiglia, M.9
-
77
-
-
79953325705
-
Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin
-
2-s2.0-79953325705
-
Ratovitski T., Chighladze E., Waldron E., Hirschhorn R. R., Ross C. A., Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin. Journal of Biological Chemistry 2011 286 14 12578 12589 10.1074/jbc.M110.185348 2-s2.0-79953325705
-
(2011)
Journal of Biological Chemistry
, vol.286
, Issue.14
, pp. 12578-12589
-
-
Ratovitski, T.1
Chighladze, E.2
Waldron, E.3
Hirschhorn, R.R.4
Ross, C.A.5
-
78
-
-
0036671821
-
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions
-
2-s2.0-0036671821
-
Lunkes A., Lindenberg K. S., Ben-Haem L., Weber C., Devys D., Landwehrmeyer G. B., Mandel J.-L., Trottier Y., Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions. Molecular Cell 2002 10 2 259 269 10.1016/S1097-2765(02)00602-0 2-s2.0-0036671821
-
(2002)
Molecular Cell
, vol.10
, Issue.2
, pp. 259-269
-
-
Lunkes, A.1
Lindenberg, K.S.2
Ben-Haem, L.3
Weber, C.4
Devys, D.5
Landwehrmeyer, G.B.6
Mandel, J.-L.7
Trottier, Y.8
-
79
-
-
78650035000
-
Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors
-
2-s2.0-78650035000
-
Kegel K. B., Sapp E., Alexander J., Reeves P., Bleckmann D., Sobin L., Masso N., Valencia A., Jeong H., Krainc D., Palacino J., Curtis D., Kuhn R., Betschart C., Sena-Esteves M., Aronin N., Paganetti P., Difiglia M., Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors. Molecular Neurodegeneration 2010 5 1, article 58 10.1186/1750-1326-5-58 2-s2.0-78650035000
-
(2010)
Molecular Neurodegeneration
, vol.5
, Issue.1 ARTICLE 58
-
-
Kegel, K.B.1
Sapp, E.2
Alexander, J.3
Reeves, P.4
Bleckmann, D.5
Sobin, L.6
Masso, N.7
Valencia, A.8
Jeong, H.9
Krainc, D.10
Palacino, J.11
Curtis, D.12
Kuhn, R.13
Betschart, C.14
Sena-Esteves, M.15
Aronin, N.16
Paganetti, P.17
Difiglia, M.18
-
80
-
-
77955500335
-
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease
-
2-s2.0-77955500335
-
Miller J. P., Holcomb J., Al-Ramahi I., de Haro M., Gafni J., Zhang N., Kim E., Sanhueza M., Torcassi C., Kwak S., Botas J., Hughes R. E., Ellerby L. M., Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease. Neuron 2010 67 2 199 212 10.1016/j.neuron.2010.06.021 2-s2.0-77955500335
-
(2010)
Neuron
, vol.67
, Issue.2
, pp. 199-212
-
-
Miller, J.P.1
Holcomb, J.2
Al-Ramahi, I.3
De Haro, M.4
Gafni, J.5
Zhang, N.6
Kim, E.7
Sanhueza, M.8
Torcassi, C.9
Kwak, S.10
Botas, J.11
Hughes, R.E.12
Ellerby, L.M.13
-
81
-
-
84873463075
-
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease
-
2-s2.0-84873463075
-
Sathasivam K., Neueder A., Gipson T. A., Landles C., Benjamin A. C., Bondulich M. K., Smith D. L., Faull R. L. M., Roos R. A. C., Howland D., Detloff P. J., Housman D. E., Bates G. P., Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proceedings of the National Academy of Sciences of the United States of America 2013 110 6 2366 2370 10.1073/pnas.1221891110 2-s2.0-84873463075
-
(2013)
Proceedings of the National Academy of Sciences of the United States of America
, vol.110
, Issue.6
, pp. 2366-2370
-
-
Sathasivam, K.1
Neueder, A.2
Gipson, T.A.3
Landles, C.4
Benjamin, A.C.5
Bondulich, M.K.6
Smith, D.L.7
Faull, R.L.M.8
Roos, R.A.C.9
Howland, D.10
Detloff, P.J.11
Housman, D.E.12
Bates, G.P.13
-
82
-
-
33748319345
-
Ataxin-3 is subject to autolytic cleavage
-
2-s2.0-33748319345
-
Mauri P. L., Riva M., Ambu D., De Palma A., Secundo F., Benazzi L., Valtorta M., Tortora P., Fusi P., Ataxin-3 is subject to autolytic cleavage. The FEBS Journal 2006 273 18 4277 4286 10.1111/j.1742-4658.2006.05419.x 2-s2.0-33748319345
-
(2006)
The FEBS Journal
, vol.273
, Issue.18
, pp. 4277-4286
-
-
Mauri, P.L.1
Riva, M.2
Ambu, D.3
De Palma, A.4
Secundo, F.5
Benazzi, L.6
Valtorta, M.7
Tortora, P.8
Fusi, P.9
-
83
-
-
0034733607
-
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
-
2-s2.0-0034733607
-
Wellington C. L., Singaraja R., Ellerby L., Savill J., Roy S., Leavitt B., Cattaneo E., Hackam A., Sharp A., Thornberry N., Nicholson D. W., Bredesen D. E., Hayden M. R., Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. Journal of Biological Chemistry 2000 275 26 19831 19838 10.1074/jbc.M001475200 2-s2.0-0034733607
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.26
, pp. 19831-19838
-
-
Wellington, C.L.1
Singaraja, R.2
Ellerby, L.3
Savill, J.4
Roy, S.5
Leavitt, B.6
Cattaneo, E.7
Hackam, A.8
Sharp, A.9
Thornberry, N.10
Nicholson, D.W.11
Bredesen, D.E.12
Hayden, M.R.13
-
84
-
-
37549065909
-
N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease
-
2-s2.0-37549065909
-
Ratovitski T., Nakamura M., D'Ambola J., Chighladze E., Liang Y., Wang W., Graham R., Hayden M. R., Borchelt D. R., Hirschhorn R. R., Ross C. A., N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease. Cell Cycle 2007 6 23 2970 2981 10.4161/cc.6.23.4992 2-s2.0-37549065909
-
(2007)
Cell Cycle
, vol.6
, Issue.23
, pp. 2970-2981
-
-
Ratovitski, T.1
Nakamura, M.2
D'Ambola, J.3
Chighladze, E.4
Liang, Y.5
Wang, W.6
Graham, R.7
Hayden, M.R.8
Borchelt, D.R.9
Hirschhorn, R.R.10
Ross, C.A.11
-
85
-
-
80052243629
-
Minocycline inhibits cell death and decreases mutant Huntingtin aggregation by targeting Apaf-1
-
2-s2.0-80052243629
-
Sancho M., Herrera A. E., Gortat A., Carbajo R. J., Pineda-Lucena A., Orzáez M., Pérez-Payá E., Minocycline inhibits cell death and decreases mutant Huntingtin aggregation by targeting Apaf-1. Human Molecular Genetics 2011 20 18 3545 3553 10.1093/hmg/ddr271 2-s2.0-80052243629
-
(2011)
Human Molecular Genetics
, vol.20
, Issue.18
, pp. 3545-3553
-
-
Sancho, M.1
Herrera, A.E.2
Gortat, A.3
Carbajo, R.J.4
Pineda-Lucena, A.5
Orzáez, M.6
Pérez-Payá, E.7
-
86
-
-
22344439156
-
Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: Implications for mutant huntingtin toxicity
-
2-s2.0-22344439156
-
Luo S., Vacher C., Davies J. E., Rubinsztein D. C., Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity. Journal of Cell Biology 2005 169 4 647 656 10.1083/jcb.200412071 2-s2.0-22344439156
-
(2005)
Journal of Cell Biology
, vol.169
, Issue.4
, pp. 647-656
-
-
Luo, S.1
Vacher, C.2
Davies, J.E.3
Rubinsztein, D.C.4
-
87
-
-
84893555011
-
Preventing formation of toxic N-terminal huntingtin fragments through antisense oligonucleotide-mediated protein modification
-
2-s2.0-84893555011
-
Evers M. M., Tran H.-D., Zalachoras I., Meijer O. C., Den Dunnen J. T., Van Ommen G.-J. B., Aartsma-Rus A., Van Roon-Mom W. M. C., Preventing formation of toxic N-terminal huntingtin fragments through antisense oligonucleotide-mediated protein modification. Nucleic Acid Therapeutics 2014 24 1 4 12 10.1089/nat.2013.0452 2-s2.0-84893555011
-
(2014)
Nucleic Acid Therapeutics
, vol.24
, Issue.1
, pp. 4-12
-
-
Evers, M.M.1
Tran, H.-D.2
Zalachoras, I.3
Meijer, O.C.4
Den Dunnen, J.T.5
Van Ommen, G.-J.B.6
Aartsma-Rus, A.7
Van Roon-Mom, W.M.C.8
-
88
-
-
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
-
2-s2.0-0031918640
-
Becher M. W., Kotzuk J. A., Sharp A. H., Davies S. W., Bates G. P., Price D. L., Ross C. A., Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiology of Disease 1998 4 6 387 397 10.1006/nbdi.1998.0168 2-s2.0-0031918640
-
(1998)
Neurobiology of Disease
, vol.4
, Issue.6
, pp. 387-397
-
-
Becher, M.W.1
Kotzuk, J.A.2
Sharp, A.H.3
Davies, S.W.4
Bates, G.P.5
Price, D.L.6
Ross, C.A.7
-
89
-
-
0030666001
-
Ataxin-1 with an expanded glutamine tract alters nuclear matrix- associated structures
-
2-s2.0-0030666001
-
Skinner P. J., Koshy B. T., Cummings C. J., Klement I. A., Helin K., Servadio A., Zoghbi H. Y., Orr H. T., Ataxin-1 with an expanded glutamine tract alters nuclear matrix- associated structures. Nature 1997 389 6654 971 974 10.1038/40153 2-s2.0-0030666001
-
(1997)
Nature
, vol.389
, Issue.6654
, pp. 971-974
-
-
Skinner, P.J.1
Koshy, B.T.2
Cummings, C.J.3
Klement, I.A.4
Helin, K.5
Servadio, A.6
Zoghbi, H.Y.7
Orr, H.T.8
-
90
-
-
0032769095
-
Abundant expression and cytoplasmic aggregations of &1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6
-
2-s2.0-0032769095
-
Ishikawa K., Fujigasaki H., Saegusa H., Ohwada K., Fujita T., Iwamoto H., Komatsuzaki Y., Toru S., Toriyama H., Watanabe M., Ohkoshi N., Shoji S., Kanazawa I., Tanabe T., Mizusawa H., Abundant expression and cytoplasmic aggregations of &1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6. Human Molecular Genetics 1999 8 7 1185 1193 10.1093/hmg/8.7.1185 2-s2.0-0032769095
-
(1999)
Human Molecular Genetics
, vol.8
, Issue.7
, 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
Ohkoshi, N.11
Shoji, S.12
Kanazawa, I.13
Tanabe, T.14
Mizusawa, H.15
-
91
-
-
0036317942
-
Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content
-
2-s2.0-0036317942
-
Takahashi J., Fujigasaki H., Zander C., El Hachimi K. H., Stevanin G., Dürr A., Lebre A.-S., Yvert G., Trottier Y., De Thé H., Hauw J.-J., Duyckaerts C., Brice A., Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content. Brain 2002 125 7 1534 1543 10.1093/brain/awf154 2-s2.0-0036317942
-
(2002)
Brain
, vol.125
, Issue.7
, pp. 1534-1543
-
-
Takahashi, J.1
Fujigasaki, H.2
Zander, C.3
El Hachimi, K.H.4
Stevanin, G.5
Dürr, A.6
Lebre, A.-S.7
Yvert, G.8
Trottier, Y.9
De Thé, H.10
Hauw, J.-J.11
Duyckaerts, C.12
Brice, A.13
-
92
-
-
0035393427
-
SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein
-
2-s2.0-0035393427
-
Nakamura K., Jeong S.-Y., Uchihara T., Anno M., Nagashima K., Nagashima T., Ikeda S.-I., Tsuji S., Kanazawa I., SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Human Molecular Genetics 2001 10 14 1441 1448 10.1093/hmg/10.14.1441 2-s2.0-0035393427
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.14
, pp. 1441-1448
-
-
Nakamura, K.1
Jeong, S.-Y.2
Uchihara, T.3
Anno, M.4
Nagashima, K.5
Nagashima, T.6
Ikeda, S.-I.7
Tsuji, S.8
Kanazawa, I.9
-
93
-
-
0029611008
-
Autosomal dominant cerebellar ataxia type i in Martinique (French West Indies). Clinical and neuropathological analysis of 53 patients from three unrelated SCA2 families
-
2-s2.0-0029611008
-
Durr A., Smadja D., Cancel G., Lezin A., Stevanin G., Mikol J., Bellance R., Buisson G.-G., Chneiweiss H., Dellanave J., Agid Y., Brice A., Vernant J.-C., Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies). Clinical and neuropathological analysis of 53 patients from three unrelated SCA2 families. Brain 1995 118 6 1573 1581 10.1093/brain/118.6.1573 2-s2.0-0029611008
-
(1995)
Brain
, vol.118
, Issue.6
, pp. 1573-1581
-
-
Durr, A.1
Smadja, D.2
Cancel, G.3
Lezin, A.4
Stevanin, G.5
Mikol, J.6
Bellance, R.7
Buisson, G.-G.8
Chneiweiss, H.9
Dellanave, J.10
Agid, Y.11
Brice, A.12
Vernant, J.-C.13
-
94
-
-
0033044001
-
Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2
-
AID-ANA14>3.0.CO;2-7
-
Huynh D. P., Del Bigio M. R., Ho D. H., Pulst S. M., Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2. Annals of Neurology 1999 45 2 232 241 10.1002/1531-8249(199902)45:2<232::AID-ANA14>3.0.CO;2-7
-
(1999)
Annals of Neurology
, vol.45
, Issue.2
, pp. 232-241
-
-
Huynh, D.P.1
Del Bigio, M.R.2
Ho, D.H.3
Pulst, S.M.4
-
95
-
-
0032840052
-
Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: Triple-labeling immunofluorescent study
-
2-s2.0-0032840052
-
Koyano S., Uchihara T., Fujigasaki H., Nakamura A., Yagishita S., Iwabuchi K., Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study. Neuroscience Letters 1999 273 2 117 120 10.1016/S0304-3940(99)00656-4 2-s2.0-0032840052
-
(1999)
Neuroscience Letters
, vol.273
, Issue.2
, pp. 117-120
-
-
Koyano, S.1
Uchihara, T.2
Fujigasaki, H.3
Nakamura, A.4
Yagishita, S.5
Iwabuchi, K.6
-
96
-
-
0036185711
-
Neuronal intranuclear inclusions in SCA2: A genetic, morphological and immunohistochemical study of two cases
-
2-s2.0-0036185711
-
Pang J. T., Giunti P., Chamberlain S., An S. F., Vitaliani R., Scaravilli T., Martinian L., Wood N. W., Scaravilli F., Ansorge O., Neuronal intranuclear inclusions in SCA2: a genetic, morphological and immunohistochemical study of two cases. Brain 2002 125 3 656 663 10.1093/brain/awf060 2-s2.0-0036185711
-
(2002)
Brain
, vol.125
, Issue.3
, pp. 656-663
-
-
Pang, J.T.1
Giunti, P.2
Chamberlain, S.3
An, S.F.4
Vitaliani, R.5
Scaravilli, T.6
Martinian, L.7
Wood, N.W.8
Scaravilli, F.9
Ansorge, O.10
-
97
-
-
33846160381
-
Polymorphism in the intermediates and products of amyloid assembly
-
2-s2.0-33846160381
-
Kodali R., Wetzel R., Polymorphism in the intermediates and products of amyloid assembly. Current Opinion in Structural Biology 2007 17 1 48 57 10.1016/j.sbi.2007.01.007 2-s2.0-33846160381
-
(2007)
Current Opinion in Structural Biology
, vol.17
, Issue.1
, pp. 48-57
-
-
Kodali, R.1
Wetzel, R.2
-
98
-
-
79952189175
-
Assays for studying nucleated aggregation of polyglutamine proteins
-
2-s2.0-79952189175
-
Jayaraman M., Thakur A. K., Kar K., Kodali R., Wetzel R., Assays for studying nucleated aggregation of polyglutamine proteins. Methods 2011 53 3 246 254 10.1016/j.ymeth.2011.01.001 2-s2.0-79952189175
-
(2011)
Methods
, vol.53
, Issue.3
, pp. 246-254
-
-
Jayaraman, M.1
Thakur, A.K.2
Kar, K.3
Kodali, R.4
Wetzel, R.5
-
99
-
-
33745195252
-
The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step
-
2-s2.0-33745195252
-
Ellisdon A. M., Thomas B., Bottomley S. P., The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step. Journal of Biological Chemistry 2006 281 25 16888 16896 10.1074/jbc.M601470200 2-s2.0-33745195252
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.25
, pp. 16888-16896
-
-
Ellisdon, A.M.1
Thomas, B.2
Bottomley, S.P.3
-
100
-
-
84894469743
-
Prion protein aggregation and fibrillogenesis in vitro
-
2-s2.0-84894469743
-
Stöhr J., Prion protein aggregation and fibrillogenesis in vitro. Sub-cellular biochemistry 2012 65 91 108 10.1007/978-94-007-5416-4-5 2-s2.0-84894469743
-
(2012)
Sub-cellular Biochemistry
, vol.65
, pp. 91-108
-
-
Stöhr, J.1
-
101
-
-
84859400325
-
Kinetically competing huntingtin aggregation pathways control amyloid polymorphism and properties
-
2-s2.0-84859400325
-
Jayaraman M., Mishra R., Kodali R., Thakur A. K., Koharudin L. M. I., Gronenborn A. M., Wetzel R., Kinetically competing huntingtin aggregation pathways control amyloid polymorphism and properties. Biochemistry 2012 51 13 2706 2716 10.1021/bi3000929 2-s2.0-84859400325
-
(2012)
Biochemistry
, vol.51
, Issue.13
, pp. 2706-2716
-
-
Jayaraman, M.1
Mishra, R.2
Kodali, R.3
Thakur, A.K.4
Koharudin, L.M.I.5
Gronenborn, A.M.6
Wetzel, R.7
-
102
-
-
84884920984
-
Different ataxin-3 amyloid aggregates induce intracellular Ca2+ deregulation by different mechanisms in cerebellar granule cells
-
2-s2.0-84884920984
-
Pellistri F., Bucciantini M., Invernizzi G., Gatta E., Penco A., Frana A. M., Nosi D., Relini A., Regonesi M. E., Gliozzi A., Tortora P., Robello M., Stefani M., Different ataxin-3 amyloid aggregates induce intracellular Ca2+ deregulation by different mechanisms in cerebellar granule cells. Biochimica et Biophysica Acta-Molecular Cell Research 2013 1833 12 3155 3165 10.1016/j.bbamcr.2013.08.019 2-s2.0-84884920984
-
(2013)
Biochimica et Biophysica Acta - Molecular Cell Research
, vol.1833
, Issue.12
, pp. 3155-3165
-
-
Pellistri, F.1
Bucciantini, M.2
Invernizzi, G.3
Gatta, E.4
Penco, A.5
Frana, A.M.6
Nosi, D.7
Relini, A.8
Regonesi, M.E.9
Gliozzi, A.10
Tortora, P.11
Robello, M.12
Stefani, M.13
-
103
-
-
0032590053
-
Huntington aggregates may not predict neuronal death in Huntingtons disease
-
Kuemmerle S., Gutekunst C. A., Klein A. M., Li X. J., Li S. H., Beal M. F., Hersch S. M., Ferrante R. J., Huntington aggregates may not predict neuronal death in Huntingtons disease. Annals of Neurology 1999 46 6 842 849
-
(1999)
Annals of Neurology
, vol.46
, Issue.6
, pp. 842-849
-
-
Kuemmerle, S.1
Gutekunst, C.A.2
Klein, A.M.3
Li, X.J.4
Li, S.H.5
Beal, M.F.6
Hersch, S.M.7
Ferrante, R.J.8
-
104
-
-
0037406093
-
Role of histidine interruption in mitigating the pathological effects of long polyglutamine stretches in SCA1: A molecular approach
-
2-s2.0-0037406093
-
Sen S., Dash D., Pasha S., Brahmachari S. K., Role of histidine interruption in mitigating the pathological effects of long polyglutamine stretches in SCA1: a molecular approach. Protein Science 2003 12 5 953 962 10.1110/ps.0224403 2-s2.0-0037406093
-
(2003)
Protein Science
, vol.12
, Issue.5
, pp. 953-962
-
-
Sen, S.1
Dash, D.2
Pasha, S.3
Brahmachari, S.K.4
-
105
-
-
0037041420
-
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
-
2-s2.0-0037041420
-
Bucciantini M., Giannoni E., Chiti F., Baroni F., Formigli L., Zurdo J., Taddei N., Ramponi G., Dobson C. M., Stefani M., Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature 2002 416 6880 507 511 10.1038/416507a 2-s2.0-0037041420
-
(2002)
Nature
, vol.416
, Issue.6880
, pp. 507-511
-
-
Bucciantini, M.1
Giannoni, E.2
Chiti, F.3
Baroni, F.4
Formigli, L.5
Zurdo, J.6
Taddei, N.7
Ramponi, G.8
Dobson, C.M.9
Stefani, M.10
-
106
-
-
38049139425
-
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease
-
2-s2.0-38049139425
-
Weiss A., Klein C., Woodman B., Sathasivam K., Bibel M., Régulier E., Bates G. P., Paganetti P., Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. Journal of Neurochemistry 2008 104 3 846 858 10.1111/j.1471-4159.2007.05032.x 2-s2.0-38049139425
-
(2008)
Journal of Neurochemistry
, vol.104
, Issue.3
, pp. 846-858
-
-
Weiss, A.1
Klein, C.2
Woodman, B.3
Sathasivam, K.4
Bibel, M.5
Régulier, E.6
Bates, G.P.7
Paganetti, P.8
-
107
-
-
70350041634
-
The p62 antibody reveals various cytoplasmic protein aggregates in spinocerebellar ataxia Type 6
-
2-s2.0-70350041634
-
Seidel K., Brunt E. R. P., de Vos R. A. I., Dijk F., van der Want H. J. L., Kampinga H. H., Rüb U., den Dunnen W. F. A., The p62 antibody reveals various cytoplasmic protein aggregates in spinocerebellar ataxia Type 6. Clinical Neuropathology 2009 28 5 344 349 10.5414/NPP28344 2-s2.0-70350041634
-
(2009)
Clinical Neuropathology
, vol.28
, Issue.5
, pp. 344-349
-
-
Seidel, K.1
Brunt, E.R.P.2
De Vos, R.A.I.3
Dijk, F.4
Van Der Want, H.J.L.5
Kampinga, H.H.6
Rüb, U.7
Den Dunnen, W.F.A.8
-
108
-
-
67649856863
-
Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity
-
2-s2.0-67649856863
-
Nekooki-Machida Y., Kurosawa M., Nukina N., Ito K., Oda T., Tanaka M., Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity. Proceedings of the National Academy of Sciences of the United States of America 2009 106 24 9679 9684 10.1073/pnas.0812083106 2-s2.0-67649856863
-
(2009)
Proceedings of the National Academy of Sciences of the United States of America
, vol.106
, Issue.24
, pp. 9679-9684
-
-
Nekooki-Machida, Y.1
Kurosawa, M.2
Nukina, N.3
Ito, K.4
Oda, T.5
Tanaka, M.6
-
109
-
-
0036850529
-
Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells
-
2-s2.0-0036850529
-
Yang W., Dunlap J. R., Andrews R. B., Wetzel R., Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells. Human Molecular Genetics 2002 11 23 2905 2917 2-s2.0-0036850529
-
(2002)
Human Molecular Genetics
, vol.11
, Issue.23
, pp. 2905-2917
-
-
Yang, W.1
Dunlap, J.R.2
Andrews, R.B.3
Wetzel, R.4
-
110
-
-
59649095699
-
Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates
-
2-s2.0-59649095699
-
Ren P.-H., Lauckner J. E., Kachirskaia I., Heuser J. E., Melki R., Kopito R. R., Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates. Nature Cell Biology 2009 11 2 219 225 10.1038/ncb1830 2-s2.0-59649095699
-
(2009)
Nature Cell Biology
, vol.11
, Issue.2
, pp. 219-225
-
-
Ren, P.-H.1
Lauckner, J.E.2
Kachirskaia, I.3
Heuser, J.E.4
Melki, R.5
Kopito, R.R.6
-
111
-
-
0345701297
-
Oligomeric and polymeric aggregates formed by proteins containing expanded polyglutamine
-
2-s2.0-0345701297
-
Iuchi S., Hoffner G., Verbeke P., Djian P., Green H., Oligomeric and polymeric aggregates formed by proteins containing expanded polyglutamine. Proceedings of the National Academy of Sciences of the United States of America 2003 100 5 2409 2414 10.1073/pnas.0437660100 2-s2.0-0345701297
-
(2003)
Proceedings of the National Academy of Sciences of the United States of America
, vol.100
, Issue.5
, pp. 2409-2414
-
-
Iuchi, S.1
Hoffner, G.2
Verbeke, P.3
Djian, P.4
Green, H.5
-
112
-
-
38349158062
-
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
-
2-s2.0-38349158062
-
Takahashi T., Kikuchi S., Katada S., Nagai Y., Nishizawa M., Onodera O., Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Human Molecular Genetics 2008 17 3 345 356 10.1093/hmg/ddm311 2-s2.0-38349158062
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.3
, pp. 345-356
-
-
Takahashi, T.1
Kikuchi, S.2
Katada, S.3
Nagai, Y.4
Nishizawa, M.5
Onodera, O.6
-
113
-
-
77951988103
-
Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo
-
2-s2.0-77951988103
-
Legleiter J., Mitchell E., Lotz G. P., Sapp E., Ng C., DiFiglia M., Thompson L. M., Muchowski P. J., Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. Journal of Biological Chemistry 2010 285 19 14777 14790 10.1074/jbc.M109.093708 2-s2.0-77951988103
-
(2010)
Journal of Biological Chemistry
, vol.285
, Issue.19
, pp. 14777-14790
-
-
Legleiter, J.1
Mitchell, E.2
Lotz, G.P.3
Sapp, E.4
Ng, C.5
Difiglia, M.6
Thompson, L.M.7
Muchowski, P.J.8
-
114
-
-
77649297870
-
Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease
-
2-s2.0-77649297870
-
Sathasivam K., Lane A., Legleiter J., Warley A., Woodman B., Finkbeiner S., Paganetti P., Muchowski P. J., Wilson S., Bates G. P., Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease. Human Molecular Genetics 2009 19 1 65 78 10.1093/hmg/ddp467 2-s2.0-77649297870
-
(2009)
Human Molecular Genetics
, vol.19
, Issue.1
, pp. 65-78
-
-
Sathasivam, K.1
Lane, A.2
Legleiter, J.3
Warley, A.4
Woodman, B.5
Finkbeiner, S.6
Paganetti, P.7
Muchowski, P.J.8
Wilson, S.9
Bates, G.P.10
-
115
-
-
0038104366
-
Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context
-
2-s2.0-0038104366
-
Jackson W. S., Tallaksen-Greene S. J., Albin R. L., Detloff P. J., Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context. Human Molecular Genetics 2003 12 13 1621 1629 10.1093/hmg/ddg163 2-s2.0-0038104366
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.13
, pp. 1621-1629
-
-
Jackson, W.S.1
Tallaksen-Greene, S.J.2
Albin, R.L.3
Detloff, P.J.4
-
116
-
-
34447520352
-
Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3:in vivo evidence
-
2-s2.0-34447520352
-
Bichelmeier U., Schmidt T., Hübener J., Boy J., Rüttiger L., Häbig K., Poths S., Bonin M., Knipper M., Schmidt W. J., Wilbertz J., Wolburg H., Laccone F., Riess O., Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3:in vivo evidence. Journal of Neuroscience 2007 27 28 7418 7428 10.1523/JNEUROSCI.4540-06.2007 2-s2.0-34447520352
-
(2007)
Journal of Neuroscience
, vol.27
, Issue.28
, pp. 7418-7428
-
-
Bichelmeier, U.1
Schmidt, T.2
Hübener, J.3
Boy, J.4
Rüttiger, L.5
Häbig, K.6
Poths, S.7
Bonin, M.8
Knipper, M.9
Schmidt, W.J.10
Wilbertz, J.11
Wolburg, H.12
Laccone, F.13
Riess, O.14
-
117
-
-
0037013954
-
The permeability barrier of nuclear pore complexes appears to operate via hydrophobic exclusion
-
2-s2.0-0037013954
-
Ribbeck K., Görlich D., The permeability barrier of nuclear pore complexes appears to operate via hydrophobic exclusion. The EMBO Journal 2002 21 11 2664 2671 10.1093/emboj/21.11.2664 2-s2.0-0037013954
-
(2002)
The EMBO Journal
, vol.21
, Issue.11
, pp. 2664-2671
-
-
Ribbeck, K.1
Görlich, D.2
-
118
-
-
14544299215
-
Mechanisms of receptor-mediated nuclear import and nuclear export
-
2-s2.0-14544299215
-
Pemberton L. F., Paschal B. M., Mechanisms of receptor-mediated nuclear import and nuclear export. Traffic 2005 6 3 187 198 10.1111/j.1600-0854.2005.00270.x 2-s2.0-14544299215
-
(2005)
Traffic
, vol.6
, Issue.3
, pp. 187-198
-
-
Pemberton, L.F.1
Paschal, B.M.2
-
119
-
-
0032858212
-
Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7
-
2-s2.0-0032858212
-
Kaytor M. D., Duvick L. A., Skinner P. J., Koob M. D., Ranum L. P. W., Orr H. T., Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7. Human Molecular Genetics 1999 8 9 1657 1664 10.1093/hmg/8.9.1657 2-s2.0-0032858212
-
(1999)
Human Molecular Genetics
, vol.8
, Issue.9
, pp. 1657-1664
-
-
Kaytor, M.D.1
Duvick, L.A.2
Skinner, P.J.3
Koob, M.D.4
Ranum, L.P.W.5
Orr, H.T.6
-
120
-
-
0037701612
-
Huntingtin contains a highly conserved nuclear export signal
-
2-s2.0-0037701612
-
Xia J., Lee D. H., Taylor J., Vandelft M., Truant R., Huntingtin contains a highly conserved nuclear export signal. Human Molecular Genetics 2003 12 12 1393 1403 10.1093/hmg/ddg156 2-s2.0-0037701612
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.12
, pp. 1393-1403
-
-
Xia, J.1
Lee, D.H.2
Taylor, J.3
Vandelft, M.4
Truant, R.5
-
121
-
-
14044266141
-
RNA association and nucleocytoplasmic shuttling by ataxin-1
-
2-s2.0-14044266141
-
Irwin S., Vandelft M., Pinchev D., Howell J. L., Graczyk J., Orr H. T., Truant R., RNA association and nucleocytoplasmic shuttling by ataxin-1. Journal of Cell Science 2005 118 1 233 242 10.1242/jcs.01611 2-s2.0-14044266141
-
(2005)
Journal of Cell Science
, vol.118
, Issue.1
, pp. 233-242
-
-
Irwin, S.1
Vandelft, M.2
Pinchev, D.3
Howell, J.L.4
Graczyk, J.5
Orr, H.T.6
Truant, R.7
-
122
-
-
33646378692
-
Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal
-
2-s2.0-33646378692
-
Taylor J., Grote S. K., Xia J., Vandelft M., Graczyk J., Ellerby L. M., La Spada A. R., Truant R., Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal. Journal of Biological Chemistry 2006 281 5 2730 2739 10.1074/jbc.M506751200 2-s2.0-33646378692
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.5
, pp. 2730-2739
-
-
Taylor, J.1
Grote, S.K.2
Xia, J.3
Vandelft, M.4
Graczyk, J.5
Ellerby, L.M.6
La Spada, A.R.7
Truant, R.8
-
123
-
-
70349780383
-
Identification and functional dissection of localization signals within ataxin-3
-
2-s2.0-70349780383
-
Antony P. M. A., Mäntele S., Mollenkopf P., Boy J., Kehlenbach R. H., Riess O., Schmidt T., Identification and functional dissection of localization signals within ataxin-3. Neurobiology of Disease 2009 36 2 280 292 10.1016/j.nbd.2009.07.020 2-s2.0-70349780383
-
(2009)
Neurobiology of Disease
, vol.36
, Issue.2
, pp. 280-292
-
-
Antony, P.M.A.1
Mäntele, S.2
Mollenkopf, P.3
Boy, J.4
Kehlenbach, R.H.5
Riess, O.6
Schmidt, T.7
-
124
-
-
80053405457
-
14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus
-
2-s2.0-80053405457
-
Lai S., O'Callaghan B., Zoghbi H. Y., Orr H. T., 14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus. Journal of Biological Chemistry 2011 286 40 34606 34616 10.1074/jbc.M111.238527 2-s2.0-80053405457
-
(2011)
Journal of Biological Chemistry
, vol.286
, Issue.40
, pp. 34606-34616
-
-
Lai, S.1
O'Callaghan, B.2
Zoghbi, H.Y.3
Orr, H.T.4
-
125
-
-
42049086100
-
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1
-
2-s2.0-42049086100
-
Lim J., Crespo-Barreto J., Jafar-Nejad P., Bowman A. B., Richman R., Hill D. E., Orr H. T., Zoghbi H. Y., Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature 2008 452 7188 713 718 10.1038/nature06731 2-s2.0-42049086100
-
(2008)
Nature
, vol.452
, Issue.7188
, pp. 713-718
-
-
Lim, J.1
Crespo-Barreto, J.2
Jafar-Nejad, P.3
Bowman, A.B.4
Richman, R.5
Hill, D.E.6
Orr, H.T.7
Zoghbi, H.Y.8
-
126
-
-
0032847708
-
Nuclear targeting of mutant huntingtin increases toxicity
-
2-s2.0-0032847708
-
Peters M. F., Nucifora F. C. Jr., Kushi J., Seaman H. C., Cooper J. K., Herring W. J., Dawson V. L., Dawson T. M., Ross C. A., Nuclear targeting of mutant huntingtin increases toxicity. Molecular and Cellular Neurosciences 1999 14 2 121 128 10.1006/mcne.1999.0773 2-s2.0-0032847708
-
(1999)
Molecular and Cellular Neurosciences
, vol.14
, Issue.2
, pp. 121-128
-
-
Peters, M.F.1
Nucifora, F.C.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
-
127
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
2-s2.0-0032475931
-
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 1998 95 1 55 56 10.1016/S0092-8674(00)81782-1 2-s2.0-0032475931
-
(1998)
Cell
, vol.95
, Issue.1
, pp. 55-56
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
128
-
-
4344636957
-
Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice
-
2-s2.0-4344636957
-
Schilling G., Savonenko A. V., Klevytska A., Morton J. L., Tucker S. M., Poirier M., Gale A., Chan N., Gonzales V., Slunt H. H., Coonfield M. L., Jenkins N. A., Copeland N. G., Ross C. A., Borchelt D. R., Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice. Human Molecular Genetics 2004 13 15 1599 1610 10.1093/hmg/ddh175 2-s2.0-4344636957
-
(2004)
Human Molecular Genetics
, vol.13
, Issue.15
, pp. 1599-1610
-
-
Schilling, G.1
Savonenko, A.V.2
Klevytska, A.3
Morton, J.L.4
Tucker, S.M.5
Poirier, M.6
Gale, A.7
Chan, N.8
Gonzales, V.9
Slunt, H.H.10
Coonfield, M.L.11
Jenkins, N.A.12
Copeland, N.G.13
Ross, C.A.14
Borchelt, D.R.15
-
129
-
-
35448994487
-
Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity
-
2-s2.0-35448994487
-
Atwal R. S., Xia J., Pinchev D., Taylor J., Epand R. M., Truant R., Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity. Human Molecular Genetics 2007 16 21 2600 2615 10.1093/hmg/ddm217 2-s2.0-35448994487
-
(2007)
Human Molecular Genetics
, vol.16
, Issue.21
, pp. 2600-2615
-
-
Atwal, R.S.1
Xia, J.2
Pinchev, D.3
Taylor, J.4
Epand, R.M.5
Truant, R.6
-
130
-
-
77949450752
-
Proteotoxic stress increases nuclear localization of ataxin-3
-
2-s2.0-77949450752
-
Reina C. P., Zhong X., Pittman R. N., Proteotoxic stress increases nuclear localization of ataxin-3. Human Molecular Genetics 2009 19 2 235 249 10.1093/hmg/ddp482 2-s2.0-77949450752
-
(2009)
Human Molecular Genetics
, vol.19
, Issue.2
, pp. 235-249
-
-
Reina, C.P.1
Zhong, X.2
Pittman, R.N.3
-
131
-
-
79955456732
-
Mutant huntingtin causes defective actin remodeling during stress: Defining a new role for transglutaminase 2 in neurodegenerative disease
-
2-s2.0-79955456732
-
Munsie L., Caron N., Atwal R. S., Marsden I., Wild E. J., Bamburg J. R., Tabrizi S. J., Truant R., Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease. Human Molecular Genetics 2011 20 10 1937 1951 10.1093/hmg/ddr075 2-s2.0-79955456732
-
(2011)
Human Molecular Genetics
, vol.20
, Issue.10
, pp. 1937-1951
-
-
Munsie, L.1
Caron, N.2
Atwal, R.S.3
Marsden, I.4
Wild, E.J.5
Bamburg, J.R.6
Tabrizi, S.J.7
Truant, R.8
-
132
-
-
84873340106
-
The huntingtin N17 domain is a multifunctional CRM1 and ran-dependent nuclear and cilial export signal
-
2-s2.0-84873340106
-
Maiuri T., Woloshansky T., Xia J., Truant R., The huntingtin N17 domain is a multifunctional CRM1 and ran-dependent nuclear and cilial export signal. Human Molecular Genetics 2013 22 7 1383 1394 10.1093/hmg/dds554 2-s2.0-84873340106
-
(2013)
Human Molecular Genetics
, vol.22
, Issue.7
, pp. 1383-1394
-
-
Maiuri, T.1
Woloshansky, T.2
Xia, J.3
Truant, R.4
-
133
-
-
84895546954
-
Phosphorylation of mutant huntingtin at serine 116 modulates neuronal toxicity
-
e88284
-
Watkin E. E., Arbez N., Waldron-Roby E., O'Meally R., Ratovitski T T., Cole R. N., Ross C. A., Phosphorylation of mutant huntingtin at serine 116 modulates neuronal toxicity. PLoS ONE 2014 9 2 e88284
-
(2014)
PLoS ONE
, vol.9
, Issue.2
-
-
Watkin, E.E.1
Arbez, N.2
Waldron-Roby, E.3
O'Meally, R.4
Ratovitski, T.T.5
Cole, R.N.6
Ross, C.A.7
-
134
-
-
68749103450
-
CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3
-
2-s2.0-68749103450
-
Mueller T., Breuer P., Schmitt I., Walter J., Evert B. O., Wüllner U., CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3. Human Molecular Genetics 2009 18 17 3334 3343 10.1093/hmg/ddp274 2-s2.0-68749103450
-
(2009)
Human Molecular Genetics
, vol.18
, Issue.17
, pp. 3334-3343
-
-
Mueller, T.1
Breuer, P.2
Schmitt, I.3
Walter, J.4
Evert, B.O.5
Wüllner, U.6
-
135
-
-
79954464577
-
Expanded polyglutamine domain possesses nuclear export activity which modulates subcellular localization and toxicity of polyQ disease protein via exportin-1
-
2-s2.0-79954464577
-
Chan W. M., Tsoi H., Wu C. C., Wong C. H., Cheng T. C., Li H. Y., Lau K. F., Shaw P. C., Perrimon N., Chan H. Y. E., Expanded polyglutamine domain possesses nuclear export activity which modulates subcellular localization and toxicity of polyQ disease protein via exportin-1. Human Molecular Genetics 2011 20 9 1738 1750 10.1093/hmg/ddr049 2-s2.0-79954464577
-
(2011)
Human Molecular Genetics
, vol.20
, Issue.9
, pp. 1738-1750
-
-
Chan, W.M.1
Tsoi, H.2
Wu, C.C.3
Wong, C.H.4
Cheng, T.C.5
Li, H.Y.6
Lau, K.F.7
Shaw, P.C.8
Perrimon, N.9
Chan, H.Y.E.10
-
136
-
-
84873947122
-
Identification of a karyopherin β 1/ β 2 proline-tyrosine nuclear localization signal in huntingtin protein
-
2-s2.0-84873947122
-
Desmond C. R., Atwal R. S., Xia J., Truant R., Identification of a karyopherin β 1/ β 2 proline-tyrosine nuclear localization signal in huntingtin protein. The Journal of Biological Chemistry 2012 287 47 39626 39633 10.1074/jbc.M112.412379 2-s2.0-84873947122
-
(2012)
The Journal of Biological Chemistry
, vol.287
, Issue.47
, pp. 39626-39633
-
-
Desmond, C.R.1
Atwal, R.S.2
Xia, J.3
Truant, R.4
-
137
-
-
80051632945
-
Nuclear transport, oxidative stress, and neurodegeneration
-
2-s2.0-80051632945
-
Patel V. P., Chu C. T., Nuclear transport, oxidative stress, and neurodegeneration. International Journal of Clinical and Experimental Pathology 2011 4 3 215 229 2-s2.0-80051632945
-
(2011)
International Journal of Clinical and Experimental Pathology
, vol.4
, Issue.3
, pp. 215-229
-
-
Patel, V.P.1
Chu, C.T.2
-
138
-
-
84869870525
-
Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)
-
2-s2.0-84869870525
-
Katsuno M., Tanaka F., Adachi H., Banno H., Suzuki K., Watanabe H., Sobue G., Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA). Progress in Neurobiology 2012 99 3 246 256 10.1016/j.pneurobio.2012.05.007 2-s2.0-84869870525
-
(2012)
Progress in Neurobiology
, vol.99
, Issue.3
, pp. 246-256
-
-
Katsuno, M.1
Tanaka, F.2
Adachi, H.3
Banno, H.4
Suzuki, K.5
Watanabe, H.6
Sobue, G.7
-
139
-
-
65549163327
-
Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy
-
2-s2.0-65549163327
-
Montie H. I., Cho M. S., Holder L., Liu Y., Tsvetkov A. S., Finkbeiner S., Merry D. E., Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy. Human Molecular Genetics 2009 18 11 1937 1950 10.1093/hmg/ddp115 2-s2.0-65549163327
-
(2009)
Human Molecular Genetics
, vol.18
, Issue.11
, pp. 1937-1950
-
-
Montie, H.I.1
Cho, M.S.2
Holder, L.3
Liu, Y.4
Tsvetkov, A.S.5
Finkbeiner, S.6
Merry, D.E.7
-
140
-
-
84912118712
-
Neuropathological staging of spinocerebellar ataxia type 2 by semiquantitative 1C2-positive neuron typing. Nuclear translocation of cytoplasmic 1C2 underlies disease progression of spinocerebellar ataxia type 2
-
2-s2.0-84901101574
-
Koyano S., Yagishita S., Kuroiwa Y., Tanaka F., Uchihara T., Neuropathological staging of spinocerebellar ataxia type 2 by semiquantitative 1C2-positive neuron typing. Nuclear translocation of cytoplasmic 1C2 underlies disease progression of spinocerebellar ataxia type 2. Brain Pathology 2014 10.1111/bpa.12146 2-s2.0-84901101574
-
(2014)
Brain Pathology
-
-
Koyano, S.1
Yagishita, S.2
Kuroiwa, Y.3
Tanaka, F.4
Uchihara, T.5
-
141
-
-
77956155218
-
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
-
2-s2.0-77956155218
-
Elden A. C., Kim H.-J., Hart M. P., Chen-Plotkin A. S., Johnson B. S., Fang X., Armakola M., Geser F., Greene R., Lu M. M., Padmanabhan A., Clay-Falcone D., McCluskey L., Elman L., Juhr D., Gruber P. J., Rüb U., Auburger G., Trojanowski J. Q., Lee V. M.-Y., Van Deerlin V. M., Bonini N. M., Gitler A. D., Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature 2010 466 7310 1069 1075 10.1038/nature09320 2-s2.0-77956155218
-
(2010)
Nature
, vol.466
, Issue.7310
, pp. 1069-1075
-
-
Elden, A.C.1
Kim, H.-J.2
Hart, M.P.3
Chen-Plotkin, A.S.4
Johnson, B.S.5
Fang, X.6
Armakola, M.7
Geser, F.8
Greene, R.9
Lu, M.M.10
Padmanabhan, A.11
Clay-Falcone, D.12
McCluskey, L.13
Elman, L.14
Juhr, D.15
Gruber, P.J.16
Rüb, U.17
Auburger, G.18
Trojanowski, J.Q.19
Lee, V.M.-Y.20
Van Deerlin, V.M.21
Bonini, N.M.22
Gitler, A.D.23
more..
-
142
-
-
40149101562
-
Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila
-
2-s2.0-40149101562
-
Lessing D., Bonini N. M., Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila. PLoS Biology 2008 6 2, article e29 0266 0274 10.1371/journal.pbio.0060029 2-s2.0-40149101562
-
(2008)
PLoS Biology
, vol.6
, Issue.2 ARTICLE E29
, pp. 0266-0274
-
-
Lessing, D.1
Bonini, N.M.2
-
143
-
-
33745207300
-
2+ channel &1A subunits translocate to nuclei and promote polyglutamine-mediated toxicity
-
2-s2.0-33745207300
-
2+ channel &1A subunits translocate to nuclei and promote polyglutamine-mediated toxicity. Human Molecular Genetics 2006 15 10 1587 1599 10.1093/hmg/ddl080 2-s2.0-33745207300
-
(2006)
Human Molecular Genetics
, vol.15
, Issue.10
, pp. 1587-1599
-
-
Kordasiewicz, H.B.1
Thompson, R.M.2
Clark, H.B.3
Gomez, C.M.4
-
144
-
-
13944275615
-
Polyglutamine expansion of huntingtin impairs its nuclear export
-
2-s2.0-13944275615
-
Cornett J., Cao F., Wang C.-E., Ross C. A., Bates G. P., Li S.-H., Li X.-J., Polyglutamine expansion of huntingtin impairs its nuclear export. Nature Genetics 2005 37 2 198 204 10.1038/ng1503 2-s2.0-13944275615
-
(2005)
Nature Genetics
, vol.37
, Issue.2
, pp. 198-204
-
-
Cornett, J.1
Cao, F.2
Wang, C.-E.3
Ross, C.A.4
Bates, G.P.5
Li, S.-H.6
Li, X.-J.7
-
145
-
-
0037047123
-
Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis
-
2-s2.0-0037047123
-
Chai Y., Shao J., Miller V. M., Williams A., Paulson H. L., Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis. Proceedings of the National Academy of Sciences of the United States of America 2002 99 14 9310 9315 10.1073/pnas.152101299 2-s2.0-0037047123
-
(2002)
Proceedings of the National Academy of Sciences of the United States of America
, vol.99
, Issue.14
, pp. 9310-9315
-
-
Chai, Y.1
Shao, J.2
Miller, V.M.3
Williams, A.4
Paulson, H.L.5
-
146
-
-
34948841925
-
Location, location, location: Altered transcription factor trafficking in neurodegeneration
-
2-s2.0-34948841925
-
Chu C. T., Plowey E. D., Wang Y., Patel V., Jordan-Sciutto K. L., Location, location, location: altered transcription factor trafficking in neurodegeneration. Journal of Neuropathology and Experimental Neurology 2007 66 10 873 883 10.1097/nen.0b013e318156a3d7 2-s2.0-34948841925
-
(2007)
Journal of Neuropathology and Experimental Neurology
, vol.66
, Issue.10
, pp. 873-883
-
-
Chu, C.T.1
Plowey, E.D.2
Wang, Y.3
Patel, V.4
Jordan-Sciutto, K.L.5
-
147
-
-
27644484061
-
Autophagy: Molecular machinery for self-eating
-
2-s2.0-27644484061
-
Yorimitsu T., Klionsky D. J., Autophagy: molecular machinery for self-eating. Cell Death and Differentiation 2005 12 supplement 2 1542 1552 10.1038/sj.cdd.4401765 2-s2.0-27644484061
-
(2005)
Cell Death and Differentiation
, vol.12
, pp. 1542-1552
-
-
Yorimitsu, T.1
Klionsky, D.J.2
-
148
-
-
33644859083
-
The ubiquitin proteolytic system: From a vague idea, through basic mechanisms, and onto human diseases and drug targeting
-
2-s2.0-33644859083
-
Ciechanover A., The ubiquitin proteolytic system: From a vague idea, through basic mechanisms, and onto human diseases and drug targeting. Neurology 2006 66 2 S7 S19 10.1212/01.wnl.0000192261.02023.b8 2-s2.0-33644859083
-
(2006)
Neurology
, vol.66
, Issue.2
, pp. S7-S19
-
-
Ciechanover, A.1
-
149
-
-
0033520987
-
Posttranslational quality control: Folding, refolding, and degrading proteins
-
2-s2.0-0033520987
-
Wickner S., Maurizi M. R., Gottesman S., Posttranslational quality control: folding, refolding, and degrading proteins. Science 1999 286 5446 1888 1893 10.1126/science.286.5446.1888 2-s2.0-0033520987
-
(1999)
Science
, vol.286
, Issue.5446
, pp. 1888-1893
-
-
Wickner, S.1
Maurizi, M.R.2
Gottesman, S.3
-
150
-
-
84862742197
-
Interactions of the proteasomal system with chaperones: Protein triage and protein quality control
-
2-s2.0-84862742197
-
Kästle M., Grune T., Interactions of the proteasomal system with chaperones: protein triage and protein quality control. Progress in Molecular Biology and Translational Science 2012 109 113 160 10.1016/B978-0-12-397863-9.00004-3 2-s2.0-84862742197
-
(2012)
Progress in Molecular Biology and Translational Science
, vol.109
, pp. 113-160
-
-
Kästle, M.1
Grune, T.2
-
151
-
-
0033867992
-
Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B
-
2-s2.0-0033867992
-
Wang G.-H., Sawai N., Kotliarova S., Kanazawa I., Nukina N., Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B. Human Molecular Genetics 2000 9 12 1795 1803 10.1093/hmg/9.12.1795 2-s2.0-0033867992
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.12
, pp. 1795-1803
-
-
Wang, G.-H.1
Sawai, N.2
Kotliarova, S.3
Kanazawa, I.4
Nukina, N.5
-
152
-
-
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
-
2-s2.0-0042691818
-
Doss-Pepe E. W., Stenroos E. S., Johnson W. G., Madura K., 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. Molecular and Cellular Biology 2003 23 18 6469 6483 10.1128/MCB.23.18.6469-6483.2003 2-s2.0-0042691818
-
(2003)
Molecular and Cellular Biology
, vol.23
, Issue.18
, pp. 6469-6483
-
-
Doss-Pepe, E.W.1
Stenroos, E.S.2
Johnson, W.G.3
Madura, K.4
-
153
-
-
0034703397
-
Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein
-
2-s2.0-0034703397
-
Davidson J. D., Riley B., Burright E. N., Duvick L. A., Zoghbi H. Y., Orr H. T., Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. Human Molecular Genetics 2000 9 15 2305 2312 10.1093/oxfordjournals.hmg.a018922 2-s2.0-0034703397
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.15
, pp. 2305-2312
-
-
Davidson, J.D.1
Riley, B.2
Burright, E.N.3
Duvick, L.A.4
Zoghbi, H.Y.5
Orr, H.T.6
-
154
-
-
0036311587
-
USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product
-
2-s2.0-0036311587
-
Hong S., Kim S.-J., Ka S., Choi I., Kang S., USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product. Molecular and Cellular Neuroscience 2002 20 2 298 306 10.1006/mcne.2002.1103 2-s2.0-0036311587
-
(2002)
Molecular and Cellular Neuroscience
, vol.20
, Issue.2
, pp. 298-306
-
-
Hong, S.1
Kim, S.-J.2
Ka, S.3
Choi, I.4
Kang, S.5
-
155
-
-
43149123766
-
UbcH6 interacts with and ubiquitinates the SCA1 gene product ataxin-1
-
2-s2.0-43149123766
-
Hong S., Lee S., Cho S.-G., Kang S., UbcH6 interacts with and ubiquitinates the SCA1 gene product ataxin-1. Biochemical and Biophysical Research Communications 2008 371 2 256 260 10.1016/j.bbrc.2008.04.066 2-s2.0-43149123766
-
(2008)
Biochemical and Biophysical Research Communications
, vol.371
, Issue.2
, pp. 256-260
-
-
Hong, S.1
Lee, S.2
Cho, S.-G.3
Kang, S.4
-
156
-
-
46149116525
-
The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1
-
2-s2.0-46149116525
-
Lee S., Hong S., Kang S., The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1. Biochemical and Biophysical Research Communications 2008 372 4 735 740 10.1016/j.bbrc.2008.05.125 2-s2.0-46149116525
-
(2008)
Biochemical and Biophysical Research Communications
, vol.372
, Issue.4
, pp. 735-740
-
-
Lee, S.1
Hong, S.2
Kang, S.3
-
157
-
-
0035891863
-
Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex
-
2-s2.0-0035891863
-
Matilla A., Gorbea C., Einum D. D., Townsend J., Michalik A., Van Broeckhoven C., Jensen C. C., Murphy K. J., Ptácek L. J., Fu Y.-H., Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex. Human Molecular Genetics 2001 10 24 2821 2831 10.1093/hmg/10.24.2821 2-s2.0-0035891863
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.24
, pp. 2821-2831
-
-
Matilla, A.1
Gorbea, C.2
Einum, D.D.3
Townsend, J.4
Michalik, A.5
Van Broeckhoven, C.6
Jensen, C.C.7
Murphy, K.J.8
Ptácek, L.J.9
Fu, Y.-H.10
-
158
-
-
0034754875
-
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
-
2-s2.0-0034754875
-
Waelter S., Boeddrich A., Lurz R., Scherzinger E., Lueder G., Lehrach H., Wanker E. E., Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Molecular Biology of the Cell 2001 12 5 1393 1407 10.1091/mbc.12.5.1393 2-s2.0-0034754875
-
(2001)
Molecular Biology of the Cell
, vol.12
, Issue.5
, 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
-
159
-
-
84866556522
-
Protein aggregates in Huntington's disease
-
2-s2.0-84866556522
-
Arrasate M., Finkbeiner S., Protein aggregates in Huntington's disease. Experimental Neurology 2012 238 1 1 11 10.1016/j.expneurol.2011.12.013 2-s2.0-84866556522
-
(2012)
Experimental Neurology
, vol.238
, Issue.1
, pp. 1-11
-
-
Arrasate, M.1
Finkbeiner, S.2
-
160
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
2-s2.0-0031838352
-
Cummings C. J., Mancini M. A., Antalffy B., DeFranco D. B., Orr H. T., Zoghbi H. Y., Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nature Genetics 1998 19 2 148 154 10.1038/502 2-s2.0-0031838352
-
(1998)
Nature Genetics
, vol.19
, Issue.2
, 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
-
161
-
-
0036198110
-
Protein surveillance machinery in brains with spinocerebellar ataxia type 3: Redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions
-
2-s2.0-0036198110
-
Schmidt T., Lindenberg K. S., Krebs A., Schöls L., Laccone F., Herms J., Rechsteiner M., Riess O., Landwehrmeyer B., Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions. Annals of Neurology 2002 51 3 302 310 10.1002/ana.10101 2-s2.0-0036198110
-
(2002)
Annals of Neurology
, vol.51
, Issue.3
, pp. 302-310
-
-
Schmidt, T.1
Lindenberg, K.S.2
Krebs, A.3
Schöls, L.4
Laccone, F.5
Herms, J.6
Rechsteiner, M.7
Riess, O.8
Landwehrmeyer, B.9
-
162
-
-
34547958064
-
Chaperone functions of the E3 ubiquitin ligase CHIP
-
2-s2.0-34547958064
-
Rosser M. F. N., Washburn E., Muchowski P. J., Patterson C., Cyr D. M., Chaperone functions of the E3 ubiquitin ligase CHIP. Journal of Biological Chemistry 2007 282 31 22267 22277 10.1074/jbc.M700513200 2-s2.0-34547958064
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.31
, pp. 22267-22277
-
-
Rosser, M.F.N.1
Washburn, E.2
Muchowski, P.J.3
Patterson, C.4
Cyr, D.M.5
-
163
-
-
15744387323
-
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes
-
2-s2.0-15744387323
-
Jana N. R., Dikshit P., Goswami A., Kotliarova S., Murata S., Tanaka K., Nukina N., Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. Journal of Biological Chemistry 2005 280 12 11635 11640 10.1074/jbc.M412042200 2-s2.0-15744387323
-
(2005)
Journal of Biological Chemistry
, vol.280
, Issue.12
, pp. 11635-11640
-
-
Jana, N.R.1
Dikshit, P.2
Goswami, A.3
Kotliarova, S.4
Murata, S.5
Tanaka, K.6
Nukina, N.7
-
164
-
-
33748741301
-
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation
-
2-s2.0-33748741301
-
Al-Ramahi I., Lam Y. C., Chen H.-K., De Gouyon B., Zhang M., Pérez A. M., Branco J., De Haro M., Patterson C., Zoghbi H. Y., Botas J., CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. Journal of Biological Chemistry 2006 281 36 26714 26724 10.1074/jbc.M601603200 2-s2.0-33748741301
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.36
, pp. 26714-26724
-
-
Al-Ramahi, I.1
Lam, Y.C.2
Chen, H.-K.3
De Gouyon, B.4
Zhang, M.5
Pérez, A.M.6
Branco, J.7
De Haro, M.8
Patterson, C.9
Zoghbi, H.Y.10
Botas, J.11
-
165
-
-
60849119525
-
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis
-
2-s2.0-60849119525
-
Williams A. J., Knutson T. M., Colomer Gould V. F., Paulson H. L., In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiology of Disease 2009 33 3 342 353 10.1016/j.nbd.2008.10.016 2-s2.0-60849119525
-
(2009)
Neurobiology of Disease
, vol.33
, Issue.3
, pp. 342-353
-
-
Williams, A.J.1
Knutson, T.M.2
Colomer Gould, V.F.3
Paulson, H.L.4
-
166
-
-
33845864451
-
Co-chaperone CHIP promotes aggregation of ataxin-1
-
2-s2.0-33845864451
-
Choi J. Y., Ryu J. H., Kim H.-S., Park S. G., Bae K.-H., Kang S., Myung P. K., Cho S., Park B. C., Lee D. H., Co-chaperone CHIP promotes aggregation of ataxin-1. Molecular and Cellular Neuroscience 2007 34 1 69 79 10.1016/j.mcn.2006.10.002 2-s2.0-33845864451
-
(2007)
Molecular and Cellular Neuroscience
, vol.34
, Issue.1
, pp. 69-79
-
-
Choi, J.Y.1
Ryu, J.H.2
Kim, H.-S.3
Park, S.G.4
Bae, K.-H.5
Kang, S.6
Myung, P.K.7
Cho, S.8
Park, B.C.9
Lee, D.H.10
-
167
-
-
57049117002
-
CHIP deletion reveals functional redundancy of E3 ligases in promoting degradation of both signaling proteins and expanded glutamine proteins
-
2-s2.0-57049117002
-
Morishima Y., Wang A. M., Yu Z., Pratt W. B., Osawa Y., Lieberman A. P., CHIP deletion reveals functional redundancy of E3 ligases in promoting degradation of both signaling proteins and expanded glutamine proteins. Human Molecular Genetics 2008 17 24 3942 3952 10.1093/hmg/ddn296 2-s2.0-57049117002
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.24
, pp. 3942-3952
-
-
Morishima, Y.1
Wang, A.M.2
Yu, Z.3
Pratt, W.B.4
Osawa, Y.5
Lieberman, A.P.6
-
168
-
-
0038159253
-
Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function
-
2-s2.0-0038159253
-
Tsai Y. C., Fishman P. S., Thakor N. V., Oyler G. A., Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function. Journal of Biological Chemistry 2003 278 24 22044 22055 10.1074/jbc.M212235200 2-s2.0-0038159253
-
(2003)
Journal of Biological Chemistry
, vol.278
, Issue.24
, pp. 22044-22055
-
-
Tsai, Y.C.1
Fishman, P.S.2
Thakor, N.V.3
Oyler, G.A.4
-
169
-
-
33846545106
-
Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death
-
2-s2.0-33846545106
-
Huynh D. P., Nguyen D. T., Pulst-Korenberg J. B., Brice A., Pulst S.-M., Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death. Experimental Neurology 2007 203 2 531 541 10.1016/j.expneurol.2006.09.009 2-s2.0-33846545106
-
(2007)
Experimental Neurology
, vol.203
, Issue.2
, pp. 531-541
-
-
Huynh, D.P.1
Nguyen, D.T.2
Pulst-Korenberg, J.B.3
Brice, A.4
Pulst, S.-M.5
-
170
-
-
79955964218
-
The machado-joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability
-
2-s2.0-79955964218
-
Durcan T. M., Kontogiannea M., Thorarinsdottir T., Fallon L., Williams A. J., Djarmati A., Fantaneanu T., Paulson H. L., Fon E. A., The machado-joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Human Molecular Genetics 2011 20 1 141 154 10.1093/hmg/ddq452 2-s2.0-79955964218
-
(2011)
Human Molecular Genetics
, vol.20
, Issue.1
, pp. 141-154
-
-
Durcan, T.M.1
Kontogiannea, M.2
Thorarinsdottir, T.3
Fallon, L.4
Williams, A.J.5
Djarmati, A.6
Fantaneanu, T.7
Paulson, H.L.8
Fon, E.A.9
-
171
-
-
84855287943
-
Ataxin-3 deubiquitination is coupled to parkin ubiquitination via E2 ubiquitin-conjugating enzyme
-
2-s2.0-84855287943
-
Durcan T. M., Kontogiannea M., Bedard N., Wing S. S., Fon E. A., Ataxin-3 deubiquitination is coupled to parkin ubiquitination via E2 ubiquitin-conjugating enzyme. Journal of Biological Chemistry 2012 287 1 531 541 10.1074/jbc.M111.288449 2-s2.0-84855287943
-
(2012)
Journal of Biological Chemistry
, vol.287
, Issue.1
, pp. 531-541
-
-
Durcan, T.M.1
Kontogiannea, M.2
Bedard, N.3
Wing, S.S.4
Fon, E.A.5
-
172
-
-
67649411510
-
Effects of partial suppression of parkin on huntingtin mutant R6/1 mice
-
2-s2.0-67649411510
-
Rubio I., Rodríguez-Navarro J. A., Tomás-Zapico C., Ruíz C., Casarejos M. J., Perucho J., Gómez A., Rodal I., Lucas J. J., Mena M. A., de Yébenes J. G., Effects of partial suppression of parkin on huntingtin mutant R6/1 mice. Brain Research 2009 1281 91 100 10.1016/j.brainres.2009.05.039 2-s2.0-67649411510
-
(2009)
Brain Research
, vol.1281
, pp. 91-100
-
-
Rubio, I.1
Rodríguez-Navarro, J.A.2
Tomás-Zapico, C.3
Ruíz, C.4
Casarejos, M.J.5
Perucho, J.6
Gómez, A.7
Rodal, I.8
Lucas, J.J.9
Mena, M.A.10
De Yébenes, J.G.11
-
173
-
-
9444239187
-
Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme
-
2-s2.0-9444239187
-
Kalchman M. A., Graham R. K., Xia G., Brook Koide H., Graeme Hodgson J., Graham K. C., Paul Goldberg Y., Dan Gietz R., Pickart C. M., Hayden M. R., Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. Journal of Biological Chemistry 1996 271 32 19385 19394 10.1074/jbc.271.32.19385 2-s2.0-9444239187
-
(1996)
Journal of Biological Chemistry
, vol.271
, Issue.32
, pp. 19385-19394
-
-
Kalchman, M.A.1
Graham, R.K.2
Xia, G.3
Brook Koide, H.4
Graeme Hodgson, J.5
Graham, K.C.6
Paul Goldberg, Y.7
Dan Gietz, R.8
Pickart, C.M.9
Hayden, M.R.10
-
174
-
-
33845898194
-
Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases
-
2-s2.0-33845898194
-
de Pril R., Fischer D. F., Roos R. A. C., van Leeuwen F. W., Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases. Molecular and Cellular Neuroscience 2007 34 1 10 19 10.1016/j.mcn.2006.09.006 2-s2.0-33845898194
-
(2007)
Molecular and Cellular Neuroscience
, vol.34
, Issue.1
, pp. 10-19
-
-
De Pril, R.1
Fischer, D.F.2
Roos, R.A.C.3
Van Leeuwen, F.W.4
-
175
-
-
0037108725
-
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins
-
2-s2.0-0037108725
-
Weinhofer I., Forss-Petter S., Žigman M., Berger J., Aggregate formation inhibits proteasomal degradation of polyglutamine proteins. Human Molecular Genetics 2002 11 22 2689 2700 10.1093/hmg/11.22.2689 2-s2.0-0037108725
-
(2002)
Human Molecular Genetics
, vol.11
, Issue.22
, pp. 2689-2700
-
-
Weinhofer, I.1
Forss-Petter, S.2
Žigman, M.3
Berger, J.4
-
176
-
-
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
-
2-s2.0-0035336658
-
Jana N. R., Zemskov E. A., Wang G.-H., Nukina N., Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Human Molecular Genetics 2001 10 10 1049 1059 10.1093/hmg/10.10.1049 2-s2.0-0035336658
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.10
, pp. 1049-1059
-
-
Jana, N.R.1
Zemskov, E.A.2
Wang, G.-H.3
Nukina, N.4
-
177
-
-
4444303263
-
Generalized brain and skin proteasome inhibition in Huntington's disease
-
2-s2.0-4444303263
-
Seo H., Sonntag K.-C., Isacson O., Generalized brain and skin proteasome inhibition in Huntington's disease. Annals of Neurology 2004 56 3 319 328 10.1002/ana.20207 2-s2.0-4444303263
-
(2004)
Annals of Neurology
, vol.56
, Issue.3
, pp. 319-328
-
-
Seo, H.1
Sonntag, K.-C.2
Isacson, O.3
-
178
-
-
22844451581
-
Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product
-
2-s2.0-22844451581
-
Park Y., Hong S., Kim S.-J., Kang S., Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product. Molecules and Cells 2005 19 1 23 30 2-s2.0-22844451581
-
(2005)
Molecules and Cells
, vol.19
, Issue.1
, pp. 23-30
-
-
Park, Y.1
Hong, S.2
Kim, S.-J.3
Kang, S.4
-
179
-
-
33746932115
-
Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain
-
2-s2.0-33746932115
-
Díaz-Hernández M., Valera A. G., Morán M. A., Gómez-Ramos P., Alvarez-Castelao B., Castaño J. G., Hernández F., Lucas J. J., Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain. Journal of Neurochemistry 2006 98 5 1585 1596 10.1111/j.1471-4159.2006.03968.x 2-s2.0-33746932115
-
(2006)
Journal of Neurochemistry
, vol.98
, Issue.5
, pp. 1585-1596
-
-
Díaz-Hernández, M.1
Valera, A.G.2
Morán, M.A.3
Gómez-Ramos, P.4
Alvarez-Castelao, B.5
Castaño, J.G.6
Hernández, F.7
Lucas, J.J.8
-
180
-
-
33746487396
-
Expanded polyglutamines impair synaptic transmission and ubiquitin-proteasome system in Caenorhabditis elegans
-
2-s2.0-33746487396
-
Khan L. A., Bauer P. O., Miyazaki H., Lindenberg K. S., Landwehrmeyer B. G., Nukina N., Expanded polyglutamines impair synaptic transmission and ubiquitin-proteasome system in Caenorhabditis elegans. Journal of Neurochemistry 2006 98 2 576 587 10.1111/j.1471-4159.2006.03895.x 2-s2.0-33746487396
-
(2006)
Journal of Neurochemistry
, vol.98
, Issue.2
, pp. 576-587
-
-
Khan, L.A.1
Bauer, P.O.2
Miyazaki, H.3
Lindenberg, K.S.4
Landwehrmeyer, B.G.5
Nukina, N.6
-
181
-
-
33846220180
-
Polyglutamine-expanded ataxin-7 decreases nuclear translocation of NF- B p65 and impairs NF- B activity by inhibiting proteasome activity of cerebellar neurons
-
2-s2.0-33846220180
-
Wang H.-L., He C.-Y., Chou A.-H., Yeh T.-H., Chen Y.-L., Li A. H., Polyglutamine-expanded ataxin-7 decreases nuclear translocation of NF- B p65 and impairs NF- B activity by inhibiting proteasome activity of cerebellar neurons. Cellular Signalling 2007 19 3 573 581 10.1016/j.cellsig.2006.08.006 2-s2.0-33846220180
-
(2007)
Cellular Signalling
, vol.19
, Issue.3
, pp. 573-581
-
-
Wang, H.-L.1
He, C.-Y.2
Chou, A.-H.3
Yeh, T.-H.4
Chen, Y.-L.5
Li, A.H.6
-
182
-
-
84892768191
-
Age-dependent decrease in chaperone activity impairs MANF expression, leading to Purkinje cell degeneration in inducible SCA17 mice
-
2-s2.0-84892768191
-
Yang S., Huang S., Gaertig M. A., Li X.-J., Li S., Age-dependent decrease in chaperone activity impairs MANF expression, leading to Purkinje cell degeneration in inducible SCA17 mice. Neuron 2014 81 2 349 365 10.1016/j.neuron.2013.12.002 2-s2.0-84892768191
-
(2014)
Neuron
, vol.81
, Issue.2
, pp. 349-365
-
-
Yang, S.1
Huang, S.2
Gaertig, M.A.3
Li, X.-J.4
Li, S.5
-
183
-
-
14644419638
-
Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation
-
2-s2.0-14644419638
-
Bowman A. B., Yoo S.-Y., Dantuma N. P., Zoghbi H. Y., Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Human Molecular Genetics 2005 14 5 679 691 10.1093/hmg/ddi064 2-s2.0-14644419638
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.5
, pp. 679-691
-
-
Bowman, A.B.1
Yoo, S.-Y.2
Dantuma, N.P.3
Zoghbi, H.Y.4
-
184
-
-
84884567375
-
Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes
-
2-s2.0-84884567375
-
Juenemann K., Schipper-Krom S., Wiemhoefer A., Kloss A., Sanz A. S., Reits E. A. J., Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes. Journal of Biological Chemistry 2013 288 38 27068 27084 10.1074/jbc.M113.486076 2-s2.0-84884567375
-
(2013)
Journal of Biological Chemistry
, vol.288
, Issue.38
, pp. 27068-27084
-
-
Juenemann, K.1
Schipper-Krom, S.2
Wiemhoefer, A.3
Kloss, A.4
Sanz, A.S.5
Reits, E.A.J.6
-
185
-
-
84890988864
-
Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies
-
2-s2.0-84890988864
-
Schipper-Krom S., Juenemann K., Jansen A. H., Wiemhoefer A., Van Den Nieuwendijk R., Smith D. L., Hink M. A., Bates G. P., Overkleeft H., Ovaa H., Reits E., Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies. The FEBS Letters 2014 588 1 151 159 10.1016/j.febslet.2013.11.023 2-s2.0-84890988864
-
(2014)
The FEBS Letters
, vol.588
, Issue.1
, pp. 151-159
-
-
Schipper-Krom, S.1
Juenemann, K.2
Jansen, A.H.3
Wiemhoefer, A.4
Van Den Nieuwendijk, R.5
Smith, D.L.6
Hink, M.A.7
Bates, G.P.8
Overkleeft, H.9
Ovaa, H.10
Reits, E.11
-
186
-
-
0034646426
-
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
-
2-s2.0-0034646426
-
Wyttenbach A., Carmichael J., Swartz J., Furlong R. A., Narain Y., Rankin J., Rubinsztein D. C., Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America 2000 97 6 2898 2903 10.1073/pnas.97.6.2898 2-s2.0-0034646426
-
(2000)
Proceedings of the National Academy of Sciences of the United States of America
, vol.97
, Issue.6
, 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
-
187
-
-
34249807884
-
P45, an ATPase subunit of the 19S proteasome, targets the polyglutamine disease protein ataxin-3 to the proteasome
-
2-s2.0-34249807884
-
Wang H., Jia N., Fei E., Wang Z., Liu C., Zhang T., Fan J., Wu M., Chen L., Nukina N., Zhou J., Wang G., p45, an ATPase subunit of the 19S proteasome, targets the polyglutamine disease protein ataxin-3 to the proteasome. Journal of Neurochemistry 2007 101 6 1651 1661 10.1111/j.1471-4159.2007.04460.x 2-s2.0-34249807884
-
(2007)
Journal of Neurochemistry
, vol.101
, Issue.6
, pp. 1651-1661
-
-
Wang, H.1
Jia, N.2
Fei, E.3
Wang, Z.4
Liu, C.5
Zhang, T.6
Fan, J.7
Wu, M.8
Chen, L.9
Nukina, N.10
Zhou, J.11
Wang, G.12
-
188
-
-
1842766144
-
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
-
2-s2.0-1842766144
-
Venkatraman P., Wetzel R., Tanaka M., Nukina N., Goldberg A. L., Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins. Molecular Cell 2004 14 1 95 104 10.1016/S1097-2765(04)00151-0 2-s2.0-1842766144
-
(2004)
Molecular Cell
, vol.14
, Issue.1
, pp. 95-104
-
-
Venkatraman, P.1
Wetzel, R.2
Tanaka, M.3
Nukina, N.4
Goldberg, A.L.5
-
189
-
-
9144223729
-
Inefficient degradation of truncated polyglutamine proteins by the proteasome
-
2-s2.0-9144223729
-
Holmberg C. I., Staniszewski K. E., Mensah K. N., Matouschek A., Morimoto R. I., Inefficient degradation of truncated polyglutamine proteins by the proteasome. The EMBO Journal 2004 23 21 4307 4318 10.1038/sj.emboj.7600426 2-s2.0-9144223729
-
(2004)
The EMBO Journal
, vol.23
, Issue.21
, pp. 4307-4318
-
-
Holmberg, C.I.1
Staniszewski, K.E.2
Mensah, K.N.3
Matouschek, A.4
Morimoto, R.I.5
-
190
-
-
45149115090
-
Proteasomes cleave at multiple sites within polyglutamine tracts: Activation by PA28 γ (K188E)
-
2-s2.0-45149115090
-
Pratt G., Rechsteiner M., Proteasomes cleave at multiple sites within polyglutamine tracts: activation by PA28 γ (K188E). Journal of Biological Chemistry 2008 283 19 12919 12925 10.1074/jbc.M709347200 2-s2.0-45149115090
-
(2008)
Journal of Biological Chemistry
, vol.283
, Issue.19
, pp. 12919-12925
-
-
Pratt, G.1
Rechsteiner, M.2
-
191
-
-
0036566266
-
Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy
-
2-s2.0-0036566266
-
Ravikumar B., Duden R., Rubinsztein D. C., Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy. Human Molecular Genetics 2002 11 9 1107 1117 10.1093/hmg/11.9.1107 2-s2.0-0036566266
-
(2002)
Human Molecular Genetics
, vol.11
, Issue.9
, pp. 1107-1117
-
-
Ravikumar, B.1
Duden, R.2
Rubinsztein, D.C.3
-
192
-
-
72849148037
-
Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment
-
2-s2.0-72849148037
-
Mookerjee S., Papanikolaou T., Guyenet S. J., Sampath V., Lin A., Vitelli C., DeGiacomo F., Sopher B. L., Chen S. F., La Spada A. R., Ellerby L. M., Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment. Journal of Neuroscience 2009 29 48 15134 15144 10.1523/JNEUROSCI.4720-09.2009 2-s2.0-72849148037
-
(2009)
Journal of Neuroscience
, vol.29
, Issue.48
, pp. 15134-15144
-
-
Mookerjee, S.1
Papanikolaou, T.2
Guyenet, S.J.3
Sampath, V.4
Lin, A.5
Vitelli, C.6
Degiacomo, F.7
Sopher, B.L.8
Chen, S.F.9
La Spada, A.R.10
Ellerby, L.M.11
-
193
-
-
77957565855
-
Age at onset in Huntington's disease is modified by the autophagy pathway: Implication of the V471A polymorphism in Atg7
-
2-s2.0-77957565855
-
Metzger S., Saukko M., Van Che H., Tong L., Puder Y., Riess O., Nguyen H. P., Age at onset in Huntington's disease is modified by the autophagy pathway: implication of the V471A polymorphism in Atg7. Human Genetics 2010 128 4 453 459 10.1007/s00439-010-0873-9 2-s2.0-77957565855
-
(2010)
Human Genetics
, vol.128
, Issue.4
, pp. 453-459
-
-
Metzger, S.1
Saukko, M.2
Van Che, H.3
Tong, L.4
Puder, Y.5
Riess, O.6
Nguyen, H.P.7
-
194
-
-
84880667166
-
The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients
-
2-s2.0-84880667166
-
Metzger S., Walter C., REGISTRY Investigators of the European Huntington's Disease Network, Riess O., Roos R. A. C., Nielsen J. E., Craufurd D., Nguyen H. P., The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients. PLoS ONE 2013 8 7 e68951 10.1371/journal.pone.0068951 2-s2.0-84880667166
-
(2013)
PLoS ONE
, vol.8
, Issue.7
, pp. e68951
-
-
Metzger, S.1
Walter, C.2
Riess, O.3
Roos, R.A.C.4
Nielsen, J.E.5
Craufurd, D.6
Nguyen, H.P.7
-
195
-
-
24944482408
-
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation
-
2-s2.0-24944482408
-
Iwata A., Christianson J. C., Bucci M., Ellerby L. M., Nukina N., Forno L. S., Kopito R. R., Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation. Proceedings of the National Academy of Sciences of the United States of America 2005 102 37 13135 13140 10.1073/pnas.0505801102 2-s2.0-24944482408
-
(2005)
Proceedings of the National Academy of Sciences of the United States of America
, vol.102
, Issue.37
, pp. 13135-13140
-
-
Iwata, A.1
Christianson, J.C.2
Bucci, M.3
Ellerby, L.M.4
Nukina, N.5
Forno, L.S.6
Kopito, R.R.7
-
196
-
-
77953489549
-
Autophagy: PolyQ toxic fragment turnover
-
2-s2.0-77953489549
-
Duncan C., Papanikolaou T., Ellerby L. M., Autophagy: polyQ toxic fragment turnover. Autophagy 2010 6 2 312 314 10.4161/auto.6.2.11139 2-s2.0-77953489549
-
(2010)
Autophagy
, vol.6
, Issue.2
, pp. 312-314
-
-
Duncan, C.1
Papanikolaou, T.2
Ellerby, L.M.3
-
197
-
-
84863870150
-
Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 model
-
2-s2.0-84863870150
-
Yu X., Ajayi A., Boga N. R., Ström A.-L., Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 model. Journal of Molecular Neuroscience 2012 47 2 219 233 10.1007/s12031-012-9722-8 2-s2.0-84863870150
-
(2012)
Journal of Molecular Neuroscience
, vol.47
, Issue.2
, pp. 219-233
-
-
Yu, X.1
Ajayi, A.2
Boga, N.R.3
Ström, A.-L.4
-
198
-
-
84867908880
-
Development of Purkinje cell degeneration in a knockin mouse model reveals lysosomal involvement in the pathogenesis of SCA6
-
2-s2.0-84867908880
-
Unno T., Wakamori M., Koike M., Uchiyama Y., Ishikawa K., Kubota H., Yoshida T., Sasakawa H., Peters C., Mizusawa H., Watase K., Development of Purkinje cell degeneration in a knockin mouse model reveals lysosomal involvement in the pathogenesis of SCA6. Proceedings of the National Academy of Sciences of the United States of America 2012 109 43 17693 17698 10.1073/pnas.1212786109 2-s2.0-84867908880
-
(2012)
Proceedings of the National Academy of Sciences of the United States of America
, vol.109
, Issue.43
, pp. 17693-17698
-
-
Unno, T.1
Wakamori, M.2
Koike, M.3
Uchiyama, Y.4
Ishikawa, K.5
Kubota, H.6
Yoshida, T.7
Sasakawa, H.8
Peters, C.9
Mizusawa, H.10
Watase, K.11
-
199
-
-
79957458183
-
Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease
-
2-s2.0-79957458183
-
Nascimento-Ferreira I., Santos-Ferreira T., Sousa-Ferreira L., Auregan G., Onofre I., Alves S., Dufour N., Colomer Gould V. F., Koeppen A., Déglon N., Pereira De Almeida L., Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease. Brain 2011 134 5 1400 1415 10.1093/brain/awr047 2-s2.0-79957458183
-
(2011)
Brain
, vol.134
, Issue.5
, pp. 1400-1415
-
-
Nascimento-Ferreira, I.1
Santos-Ferreira, T.2
Sousa-Ferreira, L.3
Auregan, G.4
Onofre, I.5
Alves, S.6
Dufour, N.7
Colomer Gould, V.F.8
Koeppen, A.9
Déglon, N.10
Pereira De Almeida, L.11
-
200
-
-
84879899999
-
Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease
-
2-s2.0-84879899999
-
Nascimento-Ferreira I., Nóbrega C., Vasconcelos-Ferreira A., Onofre I., Albuquerque D., Aveleira C., Hirai H., Déglon N., Pereira De Almeida L., Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease. Brain 2013 136 7 2173 2188 10.1093/brain/awt144 2-s2.0-84879899999
-
(2013)
Brain
, vol.136
, Issue.7
, pp. 2173-2188
-
-
Nascimento-Ferreira, I.1
Nóbrega, C.2
Vasconcelos-Ferreira, A.3
Onofre, I.4
Albuquerque, D.5
Aveleira, C.6
Hirai, H.7
Déglon, N.8
Pereira De Almeida, L.9
-
201
-
-
0030771894
-
Huntingtin localization in brains of normal and Huntington's disease patients
-
2-s2.0-0030771894
-
Sapp E., Schwarz C., Chase K., Bhide P. G., Young A. B., Penney J., Vonsattel J. P., Aronin N., DiFiglia M., Huntingtin localization in brains of normal and Huntington's disease patients. Annals of Neurology 1997 42 4 604 612 10.1002/ana.410420411 2-s2.0-0030771894
-
(1997)
Annals of Neurology
, vol.42
, Issue.4
, pp. 604-612
-
-
Sapp, E.1
Schwarz, C.2
Chase, K.3
Bhide, P.G.4
Young, A.B.5
Penney, J.6
Vonsattel, J.P.7
Aronin, N.8
Difiglia, M.9
-
202
-
-
0035364748
-
Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration
-
2-s2.0-0035364748
-
Petersén Å., Larsen K. E., Behr G. G., Romero N., Przedborski S., Brundin P., Sulzer D., Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration. Human Molecular Genetics 2001 10 12 1243 1254 10.1093/hmg/10.12.1243 2-s2.0-0035364748
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.12
, pp. 1243-1254
-
-
Petersén Å.1
Larsen, K.E.2
Behr, G.G.3
Romero, N.4
Przedborski, S.5
Brundin, P.6
Sulzer, D.7
-
203
-
-
2942527227
-
Autophagosome-like vacuole formation in Huntington's disease lymphoblasts
-
2-s2.0-2942527227
-
Nagata E., Sawa A., Ross C. A., Snyder S. H., Autophagosome-like vacuole formation in Huntington's disease lymphoblasts. NeuroReport 2004 15 8 1325 1328 10.1097/01.wnr.0000127073.66692.8f 2-s2.0-2942527227
-
(2004)
NeuroReport
, vol.15
, Issue.8
, pp. 1325-1328
-
-
Nagata, E.1
Sawa, A.2
Ross, C.A.3
Snyder, S.H.4
-
204
-
-
69549088145
-
Bergmann glial S100B activates myo-inositol monophosphatase 1 and Co-localizes to Purkinje cell vacuoles in SCA1 transgenic mice
-
2-s2.0-69549088145
-
Vig P. J. S., Shao Q., Subramony S. H., Lopez M. E., Safaya E., Bergmann glial S100B activates myo-inositol monophosphatase 1 and Co-localizes to Purkinje cell vacuoles in SCA1 transgenic mice. Cerebellum 2009 8 3 231 244 10.1007/s12311-009-0125-5 2-s2.0-69549088145
-
(2009)
Cerebellum
, vol.8
, Issue.3
, pp. 231-244
-
-
Vig, P.J.S.1
Shao, Q.2
Subramony, S.H.3
Lopez, M.E.4
Safaya, E.5
-
205
-
-
84878934829
-
Inhibition of autophagy via p53-mediated disruption of ULK1 in a SCA7 polyglutamine disease model
-
2-s2.0-84878934829
-
Yu X., Muñoz-Alarcón A., Ajayi A., Webling K. E., Steinhof A., Langel Ü., Ström A.-L., Inhibition of autophagy via p53-mediated disruption of ULK1 in a SCA7 polyglutamine disease model. Journal of Molecular Neuroscience 2013 50 3 586 599 10.1007/s12031-013-0012-x 2-s2.0-84878934829
-
(2013)
Journal of Molecular Neuroscience
, vol.50
, Issue.3
, pp. 586-599
-
-
Yu, X.1
Muñoz-Alarcón, A.2
Ajayi, A.3
Webling, K.E.4
Steinhof, A.5
Langel Ü.6
Ström, A.-L.7
-
206
-
-
34548259958
-
P62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy
-
2-s2.0-34548259958
-
Pankiv S., Clausen T. H., Lamark T., Brech A., Bruun J.-A., Outzen H., Øvervatn A., Bjørkøy G., Johansen T., p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. Journal of Biological Chemistry 2007 282 33 24131 24145 10.1074/jbc.M702824200 2-s2.0-34548259958
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.33
, pp. 24131-24145
-
-
Pankiv, S.1
Clausen, T.H.2
Lamark, T.3
Brech, A.4
Bruun, J.-A.5
Outzen, H.6
Øvervatn, A.7
Bjørkøy, G.8
Johansen, T.9
-
207
-
-
4744349602
-
Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions
-
2-s2.0-4744349602
-
Nagaoka U., Kim K., Nihar R. J., Doi H., Maruyama M., Mitsui K., Oyama F., Nukina N., Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. Journal of Neurochemistry 2004 91 1 57 68 10.1111/j.1471-4159.2004.02692.x 2-s2.0-4744349602
-
(2004)
Journal of Neurochemistry
, vol.91
, Issue.1
, pp. 57-68
-
-
Nagaoka, U.1
Kim, K.2
Nihar, R.J.3
Doi, H.4
Maruyama, M.5
Mitsui, K.6
Oyama, F.7
Nukina, N.8
-
208
-
-
77956183748
-
Axonal inclusions in spinocerebellar ataxia type 3
-
2-s2.0-77956183748
-
Seidel K., Den Dunnen W. F. A., Schultz C., Paulson H., Frank S., De Vos R. A., Brunt E. R., Deller T., Kampinga H. H., Rüb U., Axonal inclusions in spinocerebellar ataxia type 3. Acta Neuropathologica 2010 120 4 449 460 10.1007/s00401-010-0717-7 2-s2.0-77956183748
-
(2010)
Acta Neuropathologica
, vol.120
, Issue.4
, pp. 449-460
-
-
Seidel, K.1
Den Dunnen, W.F.A.2
Schultz, C.3
Paulson, H.4
Frank, S.5
De Vos, R.A.6
Brunt, E.R.7
Deller, T.8
Kampinga, H.H.9
Rüb, U.10
-
209
-
-
77949324195
-
Nucleocytoplasmic shuttling of p62/SQSTM1 and its role in recruitment of nuclear polyubiquitinated proteins to promyelocytic leukemia bodies
-
2-s2.0-77949324195
-
Pankiv S., Lamark T., Bruun J.-A., Øvervatn A., Bjørkøy G., Johansen T., Nucleocytoplasmic shuttling of p62/SQSTM1 and its role in recruitment of nuclear polyubiquitinated proteins to promyelocytic leukemia bodies. Journal of Biological Chemistry 2010 285 8 5941 5953 10.1074/jbc.M109.039925 2-s2.0-77949324195
-
(2010)
Journal of Biological Chemistry
, vol.285
, Issue.8
, pp. 5941-5953
-
-
Pankiv, S.1
Lamark, T.2
Bruun, J.-A.3
Øvervatn, A.4
Bjørkøy, G.5
Johansen, T.6
-
210
-
-
33947164372
-
Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation
-
2-s2.0-33947164372
-
Bhutani N., Venkatraman P., Goldberg A. L., Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation. The EMBO Journal 2007 26 5 1385 1396 10.1038/sj.emboj.7601592 2-s2.0-33947164372
-
(2007)
The EMBO Journal
, vol.26
, Issue.5
, pp. 1385-1396
-
-
Bhutani, N.1
Venkatraman, P.2
Goldberg, A.L.3
-
211
-
-
78149277418
-
Puromycin-sensitive aminopeptidase protects against aggregation-prone proteins via autophagy
-
2-s2.0-78149277418
-
Menzies F. M., Hourez R., Imarisio S., Raspe M., Sadiq O., Chandraratna D., O'Kane C., Rock K. L., Reits E., Goldberg A. L., Rubinsztein D. C., Puromycin-sensitive aminopeptidase protects against aggregation-prone proteins via autophagy. Human Molecular Genetics 2010 19 23 4573 4586 10.1093/hmg/ddq385 2-s2.0-78149277418
-
(2010)
Human Molecular Genetics
, vol.19
, Issue.23
, pp. 4573-4586
-
-
Menzies, F.M.1
Hourez, R.2
Imarisio, S.3
Raspe, M.4
Sadiq, O.5
Chandraratna, D.6
O'Kane, C.7
Rock, K.L.8
Reits, E.9
Goldberg, A.L.10
Rubinsztein, D.C.11
-
212
-
-
0034608868
-
Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
-
2-s2.0-0034608868
-
Muchowski P. J., Schaffar G., Sittler A., Wanker E. E., Hayer-Hartl M. K., Hartl F. U., Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proceedings of the National Academy of Sciences of the United States of America 2000 97 14 7841 7846 10.1073/pnas.140202897 2-s2.0-0034608868
-
(2000)
Proceedings of the National Academy of Sciences of the United States of America
, vol.97
, Issue.14
, pp. 7841-7846
-
-
Muchowski, P.J.1
Schaffar, G.2
Sittler, A.3
Wanker, E.E.4
Hayer-Hartl, M.K.5
Hartl, F.U.6
-
213
-
-
70350432944
-
Activation of gene transcription by heat shock protein 27 may contribute to its neuronal protection
-
2-s2.0-70350432944
-
Friedman M. J., Li S., Li X.-J., Activation of gene transcription by heat shock protein 27 may contribute to its neuronal protection. Journal of Biological Chemistry 2009 284 41 27944 27951 10.1074/jbc.M109.037937 2-s2.0-70350432944
-
(2009)
Journal of Biological Chemistry
, vol.284
, Issue.41
, pp. 27944-27951
-
-
Friedman, M.J.1
Li, S.2
Li, X.-J.3
-
214
-
-
0034652127
-
Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
-
2-s2.0-0034652127
-
Krobitsch S., Lindquist S., Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins. Proceedings of the National Academy of Sciences of the United States of America 2000 97 4 1589 1594 10.1073/pnas.97.4.1589 2-s2.0-0034652127
-
(2000)
Proceedings of the National Academy of Sciences of the United States of America
, vol.97
, Issue.4
, pp. 1589-1594
-
-
Krobitsch, S.1
Lindquist, S.2
-
215
-
-
0034705224
-
Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans
-
2-s2.0-0034705224
-
Satyal S. H., Schmidt E., Kitagawa K., Sondheimer N., Lindquist S., Kramer J. M., Morimoto R. I., Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proceedings of the National Academy of Sciences of the United States of America 2000 97 11 5750 5755 10.1073/pnas.100107297 2-s2.0-0034705224
-
(2000)
Proceedings of the National Academy of Sciences of the United States of America
, vol.97
, Issue.11
, pp. 5750-5755
-
-
Satyal, S.H.1
Schmidt, E.2
Kitagawa, K.3
Sondheimer, N.4
Lindquist, S.5
Kramer, J.M.6
Morimoto, R.I.7
-
216
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
-
2-s2.0-0032727617
-
Warrick J. M., Chan H. Y. E., Gray-Board G. L., Chai Y., Paulson H. L., Bonini N. M., Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nature Genetics 1999 23 4 425 428 10.1038/70532 2-s2.0-0032727617
-
(1999)
Nature Genetics
, vol.23
, Issue.4
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.E.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
217
-
-
0034641589
-
Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
-
2-s2.0-0034641589
-
Jana N. R., Tanaka M., Wang G.-H., Nukina N., Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity. Human Molecular Genetics 2000 9 13 2009 2018 10.1093/hmg/9.13.2009 2-s2.0-0034641589
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.13
, pp. 2009-2018
-
-
Jana, N.R.1
Tanaka, M.2
Wang, G.-H.3
Nukina, N.4
-
218
-
-
0035930598
-
Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation
-
2-s2.0-0035930598
-
Zhou H., Li S.-H., Li X.-J., Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation. Journal of Biological Chemistry 2001 276 51 48417 48424 2-s2.0-0035930598
-
(2001)
Journal of Biological Chemistry
, vol.276
, Issue.51
, pp. 48417-48424
-
-
Zhou, H.1
Li, S.-H.2
Li, X.-J.3
-
219
-
-
0036566675
-
Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin
-
2-s2.0-0036566675
-
Wyttenbach A., Sauvageot O., Carmichael J., Diaz-Latoud C., Arrigo A.-P., Rubinsztein D. C., Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin. Human Molecular Genetics 2002 11 9 1137 1151 10.1093/hmg/11.9.1137 2-s2.0-0036566675
-
(2002)
Human Molecular Genetics
, vol.11
, Issue.9
, pp. 1137-1151
-
-
Wyttenbach, A.1
Sauvageot, O.2
Carmichael, J.3
Diaz-Latoud, C.4
Arrigo, A.-P.5
Rubinsztein, D.C.6
-
220
-
-
0035394668
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
-
2-s2.0-0035394668
-
Cummings C. J., Sun Y., Opal P., Antalffy B., Mestril R., Orr H. T., Dillmann W. H., Zoghbi H. Y., Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Human Molecular Genetics 2001 10 14 1511 1518 10.1093/hmg/10.14.1511 2-s2.0-0035394668
-
(2001)
Human Molecular Genetics
, vol.10
, Issue.14
, pp. 1511-1518
-
-
Cummings, C.J.1
Sun, Y.2
Opal, P.3
Antalffy, B.4
Mestril, R.5
Orr, H.T.6
Dillmann, W.H.7
Zoghbi, H.Y.8
-
221
-
-
0347928859
-
Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression
-
2-s2.0-0347928859
-
Hansson O., Nylandsted J., Castilho R. F., Leist M., Jäättelä M., Brundin P., Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression. Brain Research 2003 970 1-2 47 57 10.1016/S0006-8993(02)04275-0 2-s2.0-0347928859
-
(2003)
Brain Research
, vol.970
, Issue.1-2
, pp. 47-57
-
-
Hansson, O.1
Nylandsted, J.2
Castilho, R.F.3
Leist, M.4
Jäättelä, M.5
Brundin, P.6
-
222
-
-
3242695184
-
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
-
2-s2.0-3242695184
-
Hay D. G., Sathasivam K., Tobaben S., Stahl B., Marber M., Mestril R., Mahal A., Smith D. L., Woodman B., Bates G. P., Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Human Molecular Genetics 2004 13 13 1389 1405 10.1093/hmg/ddh144 2-s2.0-3242695184
-
(2004)
Human Molecular Genetics
, vol.13
, Issue.13
, pp. 1389-1405
-
-
Hay, D.G.1
Sathasivam, K.2
Tobaben, S.3
Stahl, B.4
Marber, M.5
Mestril, R.6
Mahal, A.7
Smith, D.L.8
Woodman, B.9
Bates, G.P.10
-
223
-
-
27144524290
-
Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models
-
2-s2.0-27144524290
-
Fujimoto M., Takaki E., Hayashi T., Kitaura Y., Tanaka Y., Inouye S., Nakai A., Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models. Journal of Biological Chemistry 2005 280 41 34908 34916 10.1074/jbc.M506288200 2-s2.0-27144524290
-
(2005)
Journal of Biological Chemistry
, vol.280
, Issue.41
, pp. 34908-34916
-
-
Fujimoto, M.1
Takaki, E.2
Hayashi, T.3
Kitaura, Y.4
Tanaka, Y.5
Inouye, S.6
Nakai, A.7
-
224
-
-
27944499891
-
Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease
-
2-s2.0-27944499891
-
Vacher C., Garcia-Oroz L., Rubinsztein D. C., Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease. Human Molecular Genetics 2005 14 22 3425 3433 10.1093/hmg/ddi372 2-s2.0-27944499891
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.22
, pp. 3425-3433
-
-
Vacher, C.1
Garcia-Oroz, L.2
Rubinsztein, D.C.3
-
225
-
-
34247245632
-
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease
-
2-s2.0-34247245632
-
Perrin V., Régulier E., Abbas-Terki T., Hassig R., Brouillet E., Aebischer P., Luthi-Carter R., Déglon N., Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Molecular Therapy 2007 15 5 903 911 10.1038/mt.sj.6300141 2-s2.0-34247245632
-
(2007)
Molecular Therapy
, vol.15
, Issue.5
, pp. 903-911
-
-
Perrin, V.1
Régulier, E.2
Abbas-Terki, T.3
Hassig, R.4
Brouillet, E.5
Aebischer, P.6
Luthi-Carter, R.7
Déglon, N.8
-
226
-
-
34447331291
-
Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: Chronic neurodegeneration does not induce Hsp27 activation
-
2-s2.0-34447331291
-
Zourlidou A., Gidalevitz T., Kristiansen M., Landles C., Woodman B., Wells D. J., Latchman D. S., de Belleroche J., Tabrizi S. J., Morimoto R. I., Bates G. P., Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: Chronic neurodegeneration does not induce Hsp27 activation. Human Molecular Genetics 2007 16 9 1078 1090 10.1093/hmg/ddm057 2-s2.0-34447331291
-
(2007)
Human Molecular Genetics
, vol.16
, Issue.9
, pp. 1078-1090
-
-
Zourlidou, A.1
Gidalevitz, T.2
Kristiansen, M.3
Landles, C.4
Woodman, B.5
Wells, D.J.6
Latchman, D.S.7
De Belleroche, J.8
Tabrizi, S.J.9
Morimoto, R.I.10
Bates, G.P.11
-
227
-
-
54449101793
-
Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones
-
2-s2.0-54449101793
-
Fujikake N., Nagai Y., Popiel H. A., Okamoto Y., Yamaguchi M., Toda T., Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones. Journal of Biological Chemistry 2008 283 38 26188 26197 10.1074/jbc.M710521200 2-s2.0-54449101793
-
(2008)
Journal of Biological Chemistry
, vol.283
, Issue.38
, pp. 26188-26197
-
-
Fujikake, N.1
Nagai, Y.2
Popiel, H.A.3
Okamoto, Y.4
Yamaguchi, M.5
Toda, T.6
-
228
-
-
84873874848
-
Sodium valproate alleviates neurodegeneration in SCA3/MJD via suppressing apoptosis and rescuing the hypoacetylation levels of histone H3 and H4
-
2-s2.0-84873874848
-
Yi J., Zhang L., Tang B., Han W., Zhou Y., Chen Z., Jia D., Jiang H., Sodium valproate alleviates neurodegeneration in SCA3/MJD via suppressing apoptosis and rescuing the hypoacetylation levels of histone H3 and H4. PLoS ONE 2013 8 1 e54792 10.1371/journal.pone.0054792 2-s2.0-84873874848
-
(2013)
PLoS ONE
, vol.8
, Issue.1
, pp. e54792
-
-
Yi, J.1
Zhang, L.2
Tang, B.3
Han, W.4
Zhou, Y.5
Chen, Z.6
Jia, D.7
Jiang, H.8
-
229
-
-
70349195820
-
Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease
-
2-s2.0-70349195820
-
Zádori D., Geisz A., Vámos E., Vécsei L., Klivényi P., Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease. Pharmacology Biochemistry and Behavior 2009 94 1 148 153 10.1016/j.pbb.2009.08.001 2-s2.0-70349195820
-
(2009)
Pharmacology Biochemistry and Behavior
, vol.94
, Issue.1
, pp. 148-153
-
-
Zádori, D.1
Geisz, A.2
Vámos, E.3
Vécsei, L.4
Klivényi, P.5
-
230
-
-
80054772539
-
Combined treatment with the mood stabilizers lithium and valproate produces multiple beneficial effects in transgenic mouse models of huntington's disease
-
2-s2.0-80054772539
-
Chiu C.-T., Liu G., Leeds P., Chuang D.-M., Combined treatment with the mood stabilizers lithium and valproate produces multiple beneficial effects in transgenic mouse models of huntington's disease. Neuropsychopharmacology 2011 36 12 2406 2421 10.1038/npp.2011.128 2-s2.0-80054772539
-
(2011)
Neuropsychopharmacology
, vol.36
, Issue.12
, pp. 2406-2421
-
-
Chiu, C.-T.1
Liu, G.2
Leeds, P.3
Chuang, D.-M.4
-
231
-
-
84911948943
-
Q09 & Valproic Acid for the treatment of aggressiveness in Huntington's disease: 1-year follow-up
-
Unti E., Mazzucchi S., Kiferle L., Bonuccelli U., Ceravolo R., Q09 & Valproic Acid for the treatment of aggressiveness in Huntington's disease: 1-year follow-up. Journal of Neurology, Neurosurgery & Psychiatry 2012 83 A57 10.1136/jnnp-2012-303524.179
-
(2012)
Journal of Neurology, Neurosurgery &; Psychiatry
, vol.83
, pp. A57
-
-
Unti, E.1
Mazzucchi, S.2
Kiferle, L.3
Bonuccelli, U.4
Ceravolo, R.5
-
232
-
-
61449125123
-
Pisa syndrome and Parkinson secondary to valproic acid in Huntington's disease
-
2-s2.0-61449125123
-
Salazar Z., Tschopp L., Calandra C., Micheli F., Pisa syndrome and Parkinson secondary to valproic acid in Huntington's disease. Movement Disorders 2008 23 16 2430 2431 10.1002/mds.22328 2-s2.0-61449125123
-
(2008)
Movement Disorders
, vol.23
, Issue.16
, pp. 2430-2431
-
-
Salazar, Z.1
Tschopp, L.2
Calandra, C.3
Micheli, F.4
-
233
-
-
78650613146
-
HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3
-
2-s2.0-78650613146
-
Chou A.-H., Chen S.-Y., Yeh T.-H., Weng Y.-H., Wang H.-L., HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3. Neurobiology of Disease 2011 41 2 481 488 10.1016/j.nbd.2010.10.019 2-s2.0-78650613146
-
(2011)
Neurobiology of Disease
, vol.41
, Issue.2
, pp. 481-488
-
-
Chou, A.-H.1
Chen, S.-Y.2
Yeh, T.-H.3
Weng, Y.-H.4
Wang, H.-L.5
-
234
-
-
19944431703
-
Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease
-
2-s2.0-19944431703
-
Gardian G., Browne S. E., Choi D.-K., Klivenyi P., Gregorio J., Kubilus J. K., Ryu H., Langley B., Ratan R. R., Ferrante R. J., Beal M. F., Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease. Journal of Biological Chemistry 2005 280 1 556 563 10.1074/jbc.M410210200 2-s2.0-19944431703
-
(2005)
Journal of Biological Chemistry
, vol.280
, Issue.1
, pp. 556-563
-
-
Gardian, G.1
Browne, S.E.2
Choi, D.-K.3
Klivenyi, P.4
Gregorio, J.5
Kubilus, J.K.6
Ryu, H.7
Langley, B.8
Ratan, R.R.9
Ferrante, R.J.10
Beal, M.F.11
-
235
-
-
36248970599
-
Sodium phenylbutyrate in Huntington's disease: A dose-finding study
-
2-s2.0-36248970599
-
Hogarth P., Lovrecic L., Krainc D., Sodium phenylbutyrate in Huntington's disease: a dose-finding study. Movement Disorders 2007 22 13 1962 1964 10.1002/mds.21632 2-s2.0-36248970599
-
(2007)
Movement Disorders
, vol.22
, Issue.13
, pp. 1962-1964
-
-
Hogarth, P.1
Lovrecic, L.2
Krainc, D.3
-
236
-
-
84865114423
-
Polyglutamine disease: From pathogenesis to therapy
-
2-s2.0-84865114423
-
Watson L. M., Scholefield J., Jacquie Greenberg L., Wood M. J. A., Polyglutamine disease: from pathogenesis to therapy. South African Medical Journal 2012 102 6 481 484 2-s2.0-84865114423
-
(2012)
South African Medical Journal
, vol.102
, Issue.6
, pp. 481-484
-
-
Watson, L.M.1
Scholefield, J.2
Jacquie Greenberg, L.3
Wood, M.J.A.4
-
237
-
-
84899493975
-
Chronic Treatment with 17-DMAG Improves Balance and Coordination in A New Mouse Model of Machado-Joseph Disease
-
2-s2.0-84899493975
-
Silva-Fernandes A., Duarte-Silva S., Neves-Carvalho A., Amorim M., Soares-Cunha C., Oliveira P., Thirstrup K., Teixeira-Castro A., Maciel P., Chronic Treatment with 17-DMAG Improves Balance and Coordination in A New Mouse Model of Machado-Joseph Disease. Neurotherapeutics 2014 11 2 433 449 10.1007/s13311-013-0255-9 2-s2.0-84899493975
-
(2014)
Neurotherapeutics
, vol.11
, Issue.2
, pp. 433-449
-
-
Silva-Fernandes, A.1
Duarte-Silva, S.2
Neves-Carvalho, A.3
Amorim, M.4
Soares-Cunha, C.5
Oliveira, P.6
Thirstrup, K.7
Teixeira-Castro, A.8
MacIel, P.9
-
238
-
-
84862547657
-
The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1
-
2-s2.0-84862547657
-
Wu J.-C., Qi L., Wang Y., Kegel K. B., Yoder J., Difiglia M., Qin Z.-H., Lin F., The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1. Acta Pharmacologica Sinica 2012 33 6 743 751 10.1038/aps.2012.14 2-s2.0-84862547657
-
(2012)
Acta Pharmacologica Sinica
, vol.33
, Issue.6
, pp. 743-751
-
-
Wu, J.-C.1
Qi, L.2
Wang, Y.3
Kegel, K.B.4
Yoder, J.5
Difiglia, M.6
Qin, Z.-H.7
Lin, F.8
-
239
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
2-s2.0-2642586352
-
Ravikumar B., Vacher C., Berger Z., Davies J. E., Luo S., Oroz L. G., Scaravilli F., Easton D. F., Duden R., O'Kane C. J., Rubinsztein D. C., Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nature Genetics 2004 36 6 585 595 10.1038/ng1362 2-s2.0-2642586352
-
(2004)
Nature Genetics
, vol.36
, Issue.6
, pp. 585-595
-
-
Ravikumar, B.1
Vacher, C.2
Berger, Z.3
Davies, J.E.4
Luo, S.5
Oroz, L.G.6
Scaravilli, F.7
Easton, D.F.8
Duden, R.9
O'Kane, C.J.10
Rubinsztein, D.C.11
-
240
-
-
27544497472
-
Lithium rescues toxicity of aggregate-prone proteins in Drosophila by perturbing Wnt pathway
-
2-s2.0-27544497472
-
Berger Z., Ttofi E. K., Michel C. H., Pasco M. Y., Tenant S., Rubinsztein D. C., O'Kane C. J., Lithium rescues toxicity of aggregate-prone proteins in Drosophila by perturbing Wnt pathway. Human Molecular Genetics 2005 14 20 3003 3011 10.1093/hmg/ddi331 2-s2.0-27544497472
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.20
, pp. 3003-3011
-
-
Berger, Z.1
Ttofi, E.K.2
Michel, C.H.3
Pasco, M.Y.4
Tenant, S.5
Rubinsztein, D.C.6
O'Kane, C.J.7
-
241
-
-
74249103961
-
Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3
-
2-s2.0-74249103961
-
Menzies F. M., Huebener J., Renna M., Bonin M., Riess O., Rubinsztein D. C., Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3. Brain 2010 133 1 93 104 10.1093/brain/awp292 2-s2.0-74249103961
-
(2010)
Brain
, vol.133
, Issue.1
, pp. 93-104
-
-
Menzies, F.M.1
Huebener, J.2
Renna, M.3
Bonin, M.4
Riess, O.5
Rubinsztein, D.C.6
-
242
-
-
33645916698
-
Inositol and IP3 levels regulate autophagy: Biology and therapeutic speculations
-
2-s2.0-33645916698
-
Sarkar S., Rubinsztein D. C., Inositol and IP3 levels regulate autophagy: biology and therapeutic speculations. Autophagy 2006 2 2 132 134 10.4161/auto.2387 2-s2.0-33645916698
-
(2006)
Autophagy
, vol.2
, Issue.2
, pp. 132-134
-
-
Sarkar, S.1
Rubinsztein, D.C.2
-
243
-
-
0037072807
-
Glycogen synthase kinase-3 β inhibitors prevent cellular polyglutamine toxicity caused by the Huntington's disease mutation
-
2-s2.0-0037072807
-
Carmichael J., Sugars K. L., Bao Y. P., Rubinsztein D. C., Glycogen synthase kinase-3 β inhibitors prevent cellular polyglutamine toxicity caused by the Huntington's disease mutation. Journal of Biological Chemistry 2002 277 37 33791 33798 10.1074/jbc.M204861200 2-s2.0-0037072807
-
(2002)
Journal of Biological Chemistry
, vol.277
, Issue.37
, pp. 33791-33798
-
-
Carmichael, J.1
Sugars, K.L.2
Bao, Y.P.3
Rubinsztein, D.C.4
-
244
-
-
34249675397
-
Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model
-
2-s2.0-34249675397
-
Watase K., Gatchel J. R., Sun Y., Emamian E., Atkinson R., Richman R., Mizusawa H., Orr H. T., Shaw C., Zoghbi H. Y., Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model. PLoS Medicine 2007 4 5, article e182 0836 0847 10.1371/journal.pmed.0040182 2-s2.0-34249675397
-
(2007)
PLoS Medicine
, vol.4
, Issue.5 ARTICLE E182
, pp. 0836-0847
-
-
Watase, K.1
Gatchel, J.R.2
Sun, Y.3
Emamian, E.4
Atkinson, R.5
Richman, R.6
Mizusawa, H.7
Orr, H.T.8
Shaw, C.9
Zoghbi, H.Y.10
-
245
-
-
84892431753
-
Lithium chloride alleviates neurodegeneration partly by inhibiting activity of GSK3 β in a SCA3 drosophila model
-
2-s2.0-84892431753
-
Jia D.-D., Zhang L., Chen Z., Wang C.-R., Huang F.-Z., Duan R.-H., Xia K., Tang B.-S., Jiang H., Lithium chloride alleviates neurodegeneration partly by inhibiting activity of GSK3 β in a SCA3 drosophila model. Cerebellum 2013 12 6 892 901 10.1007/s12311-013-0498-3 2-s2.0-84892431753
-
(2013)
Cerebellum
, vol.12
, Issue.6
, pp. 892-901
-
-
Jia, D.-D.1
Zhang, L.2
Chen, Z.3
Wang, C.-R.4
Huang, F.-Z.5
Duan, R.-H.6
Xia, K.7
Tang, B.-S.8
Jiang, H.9
-
246
-
-
84894355071
-
Lithium down-regulates histone deacetylase 1 (HDAC1) and induces degradation of mutant huntingtin
-
2-s2.0-84894355071
-
Wu S., Zheng S.-D., Huang H.-L., Yan L.-C., Yin X.-F., Xu H.-N., Zhang K.-J., Gui J.-H., Chu L., Liu X.-Y., Lithium down-regulates histone deacetylase 1 (HDAC1) and induces degradation of mutant huntingtin. Journal of Biological Chemistry 2013 288 49 35500 35510 10.1074/jbc.M113.479865 2-s2.0-84894355071
-
(2013)
Journal of Biological Chemistry
, vol.288
, Issue.49
, pp. 35500-35510
-
-
Wu, S.1
Zheng, S.-D.2
Huang, H.-L.3
Yan, L.-C.4
Yin, X.-F.5
Xu, H.-N.6
Zhang, K.-J.7
Gui, J.-H.8
Chu, L.9
Liu, X.-Y.10
-
247
-
-
37849042536
-
A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin
-
2-s2.0-37849042536
-
Sarkar S., Krishna G., Imarisio S., Saiki S., O'Kane C. J., Rubinsztein D. C., A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin. Human Molecular Genetics 2008 17 2 170 178 10.1093/hmg/ddm294 2-s2.0-37849042536
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.2
, pp. 170-178
-
-
Sarkar, S.1
Krishna, G.2
Imarisio, S.3
Saiki, S.4
O'Kane, C.J.5
Rubinsztein, D.C.6
-
248
-
-
77953486943
-
Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease
-
2-s2.0-77953486943
-
Rose C., Menzies F. M., Renna M., Acevedo-Arozena A., Corrochano S., Sadiq O., Brown S. D., Rubinsztein D. C., Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease. Human Molecular Genetics 2010 19 11 2144 2153 10.1093/hmg/ddq093 2-s2.0-77953486943
-
(2010)
Human Molecular Genetics
, vol.19
, Issue.11
, pp. 2144-2153
-
-
Rose, C.1
Menzies, F.M.2
Renna, M.3
Acevedo-Arozena, A.4
Corrochano, S.5
Sadiq, O.6
Brown, S.D.7
Rubinsztein, D.C.8
-
249
-
-
34247161367
-
Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and &-synuclein
-
2-s2.0-34247161367
-
Sarkar S., Davies J. E., Huang Z., Tunnacliffe A., Rubinsztein D. C., Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and &-synuclein. Journal of Biological Chemistry 2007 282 8 5641 5652 10.1074/jbc.M609532200 2-s2.0-34247161367
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.8
, pp. 5641-5652
-
-
Sarkar, S.1
Davies, J.E.2
Huang, Z.3
Tunnacliffe, A.4
Rubinsztein, D.C.5
-
250
-
-
84887620690
-
Accelerating the clearance of mutant huntingtin protein aggregates through autophagy induction by europium hydroxide nanorods
-
2-s2.0-84887620690
-
Wei P.-F., Zhang L., Nethi S. K., Barui A. K., Lin J., Zhou W., Shen Y., Man N., Zhang Y.-J., Xu J., Patra C. R., Wen L.-P., Accelerating the clearance of mutant huntingtin protein aggregates through autophagy induction by europium hydroxide nanorods. Biomaterials 2014 35 3 899 907 10.1016/j.biomaterials.2013.10.024 2-s2.0-84887620690
-
(2014)
Biomaterials
, vol.35
, Issue.3
, pp. 899-907
-
-
Wei, P.-F.1
Zhang, L.2
Nethi, S.K.3
Barui, A.K.4
Lin, J.5
Zhou, W.6
Shen, Y.7
Man, N.8
Zhang, Y.-J.9
Xu, J.10
Patra, C.R.11
Wen, L.-P.12
-
251
-
-
77953500851
-
Mitochondrial dysfunction in neurodegenerative diseases and cancer
-
2-s2.0-77953500851
-
Moura M. B., dos Santos L. S., van Houten B., Mitochondrial dysfunction in neurodegenerative diseases and cancer. Environmental and Molecular Mutagenesis 2010 51 5 391 405 10.1002/em.20575 2-s2.0-77953500851
-
(2010)
Environmental and Molecular Mutagenesis
, vol.51
, Issue.5
, pp. 391-405
-
-
Moura, M.B.1
Dos Santos, L.S.2
Van Houten, B.3
-
252
-
-
84903316334
-
Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis
-
2-s2.0-84895856178
-
Tan W., Pasinelli P., Trotti D., Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis. Biochimica et Biophysica Acta-Molecular Basis of Disease 2014 1842 8 1295 1301 10.1016/j.bbadis.2014.02.009 2-s2.0-84895856178
-
(2014)
Biochimica et Biophysica Acta - Molecular Basis of Disease
, vol.1842
, Issue.8
, pp. 1295-1301
-
-
Tan, W.1
Pasinelli, P.2
Trotti, D.3
-
253
-
-
0022946353
-
Brain energy metabolism and dopaminergic function in Huntington's disease measured in vivo using positron emission tomography
-
2-s2.0-0022946353
-
Leenders K. L., Frackowiak R. S., Quinn N., Marsden C. D., Brain energy metabolism and dopaminergic function in Huntington's disease measured in vivo using positron emission tomography. Movement Disorders 1986 1 1 69 77 10.1002/mds.870010110 2-s2.0-0022946353
-
(1986)
Movement Disorders
, vol.1
, Issue.1
, pp. 69-77
-
-
Leenders, K.L.1
Frackowiak, R.S.2
Quinn, N.3
Marsden, C.D.4
-
254
-
-
0037069280
-
Weight loss in early stage of Huntington's disease
-
2-s2.0-0037069280
-
Djoussé L., Knowlton B., Cupples L. A., Marder K., Shoulson I., Myers R. H., Weight loss in early stage of Huntington's disease. Neurology 2002 59 9 1325 1330 10.1212/01.WNL.0000031791.10922.CF 2-s2.0-0037069280
-
(2002)
Neurology
, vol.59
, Issue.9
, pp. 1325-1330
-
-
Djoussé, L.1
Knowlton, B.2
Cupples, L.A.3
Marder, K.4
Shoulson, I.5
Myers, R.H.6
-
255
-
-
84904598489
-
Increased catabolic state in spinocerebellar ataxia type 1 patients
-
2-s2.0-84894780640
-
Mähler A., Steiniger J., Endres M., Paul F., Boschmann M., Doss S., Increased catabolic state in spinocerebellar ataxia type 1 patients. Cerebellum 2014 1 7 10.1007/s12311-014-0555-6 2-s2.0-84894780640
-
(2014)
Cerebellum
, pp. 1-7
-
-
Mähler, A.1
Steiniger, J.2
Endres, M.3
Paul, F.4
Boschmann, M.5
Doss, S.6
-
256
-
-
84864717629
-
Body mass index is inversely correlated with the expanded CAG repeat length in SCA3/MJD patients
-
2-s2.0-84864717629
-
Saute J. A. M., da Silva A. C. F., Souza G. N., Russo A. D., Donis K. C., Vedolin L., Saraiva-Pereira M. L., Portela L. V. C., Jardim L. B., Body mass index is inversely correlated with the expanded CAG repeat length in SCA3/MJD patients. Cerebellum 2012 11 3 771 774 10.1007/s12311-011-0326-6 2-s2.0-84864717629
-
(2012)
Cerebellum
, vol.11
, Issue.3
, pp. 771-774
-
-
Saute, J.A.M.1
Da Silva, A.C.F.2
Souza, G.N.3
Russo, A.D.4
Donis, K.C.5
Vedolin, L.6
Saraiva-Pereira, M.L.7
Portela, L.V.C.8
Jardim, L.B.9
-
257
-
-
79952168998
-
Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3 ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration
-
2-s2.0-79952168998
-
Jafar-Nejad P., Ward C. S., Richman R., Orr H. T., Zoghbi H. Y., Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3 ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America 2011 108 5 2142 2147 10.1073/pnas.1018748108 2-s2.0-79952168998
-
(2011)
Proceedings of the National Academy of Sciences of the United States of America
, vol.108
, Issue.5
, pp. 2142-2147
-
-
Jafar-Nejad, P.1
Ward, C.S.2
Richman, R.3
Orr, H.T.4
Zoghbi, H.Y.5
-
258
-
-
56149125562
-
Weight loss in Huntington disease increases with higher CAG repeat number
-
2-s2.0-56149125562
-
Aziz N. A., Van Der Burg J. M. M., Landwehrmeyer G. B., Brundin P., Stijnen T., Roos R. A. C., Weight loss in Huntington disease increases with higher CAG repeat number. Neurology 2008 71 19 1505 1513 2-s2.0-56149125562
-
(2008)
Neurology
, vol.71
, Issue.19
, pp. 1505-1513
-
-
Aziz, N.A.1
Van Der Burg, J.M.M.2
Landwehrmeyer, G.B.3
Brundin, P.4
Stijnen, T.5
Roos, R.A.C.6
-
259
-
-
0027741301
-
Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy
-
2-s2.0-0027741301
-
Jenkins B. G., Koroshetz W. J., Beal M. F., Rosen B. R., Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy. Neurology 1993 43 12 2689 2695 10.1212/WNL.43.12.2689 2-s2.0-0027741301
-
(1993)
Neurology
, vol.43
, Issue.12
, pp. 2689-2695
-
-
Jenkins, B.G.1
Koroshetz, W.J.2
Beal, M.F.3
Rosen, B.R.4
-
260
-
-
4043130355
-
Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study
-
2-s2.0-4043130355
-
Guerrini L., Lolli F., Ginestroni A., Belli G., Della Nave R., Tessa C., Foresti S., Cosottini M., Piacentini S., Salvi F., Plasmati R., De Grandis D., Siciliano G., Filla A., Mascalchi M., Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study. Brain 2004 127 8 1785 1795 10.1093/brain/awh201 2-s2.0-4043130355
-
(2004)
Brain
, vol.127
, Issue.8
, pp. 1785-1795
-
-
Guerrini, L.1
Lolli, F.2
Ginestroni, A.3
Belli, G.4
Della Nave, R.5
Tessa, C.6
Foresti, S.7
Cosottini, M.8
Piacentini, S.9
Salvi, F.10
Plasmati, R.11
De Grandis, D.12
Siciliano, G.13
Filla, A.14
Mascalchi, M.15
-
261
-
-
77949371220
-
Noninvasive detection of presymptomatic and progressive neurodegeneration in a mouse model of spinocerebellar ataxia type 1
-
2-s2.0-77949371220
-
Öz G., Nelson C. D., Koski D. M., Henry P.-G., Marjańska M., Deelchand D. K., Shanley R., Eberly L. E., Orr H. T., Clark H. B., Noninvasive detection of presymptomatic and progressive neurodegeneration in a mouse model of spinocerebellar ataxia type 1. Journal of Neuroscience 2010 30 10 3831 3838 10.1523/JNEUROSCI.5612-09.2010 2-s2.0-77949371220
-
(2010)
Journal of Neuroscience
, vol.30
, Issue.10
, pp. 3831-3838
-
-
Öz, G.1
Nelson, C.D.2
Koski, D.M.3
Henry, P.-G.4
Marjańska, M.5
Deelchand, D.K.6
Shanley, R.7
Eberly, L.E.8
Orr, H.T.9
Clark, H.B.10
-
262
-
-
84887605878
-
Non-invasive detection of neurochemical changes prior to overt pathology in a mouse model of spinocerebellar ataxia type 1
-
2-s2.0-84887605878
-
Emir U. E., Brent Clark H., Vollmers M. L., Eberly L. E., Öz G., Non-invasive detection of neurochemical changes prior to overt pathology in a mouse model of spinocerebellar ataxia type 1. Journal of Neurochemistry 2013 127 5 660 668 10.1111/jnc.12435 2-s2.0-84887605878
-
(2013)
Journal of Neurochemistry
, vol.127
, Issue.5
, pp. 660-668
-
-
Emir, U.E.1
Brent Clark, H.2
Vollmers, M.L.3
Eberly, L.E.4
Öz, G.5
-
263
-
-
84859904873
-
Shaping the role of mitochondria in the pathogenesis of Huntington's disease
-
2-s2.0-84859904873
-
Costa V., Scorrano L., Shaping the role of mitochondria in the pathogenesis of Huntington's disease. The EMBO Journal 2012 31 8 1853 1864 10.1038/emboj.2012.65 2-s2.0-84859904873
-
(2012)
The EMBO Journal
, vol.31
, Issue.8
, pp. 1853-1864
-
-
Costa, V.1
Scorrano, L.2
-
264
-
-
34447267684
-
Mitochondrial complex i gene variant associated with early age at onset in spinocerebellar ataxia type 2
-
2-s2.0-34447267684
-
Simon D. K., Zheng K., Velázquez L., Santos N., Almaguer L., Figueroa K. P., Pulst S.-M., Mitochondrial complex I gene variant associated with early age at onset in spinocerebellar ataxia type 2. Archives of Neurology 2007 64 7 1042 1044 10.1001/archneur.64.7.1042 2-s2.0-34447267684
-
(2007)
Archives of Neurology
, vol.64
, Issue.7
, pp. 1042-1044
-
-
Simon, D.K.1
Zheng, K.2
Velázquez, L.3
Santos, N.4
Almaguer, L.5
Figueroa, K.P.6
Pulst, S.-M.7
-
265
-
-
60249095382
-
The spinocerebellar ataxia 12 gene product and protein phosphatase 2A regulatory subunit B β 2 antagonizes neuronal survival by promoting mitochondrial fission
-
2-s2.0-60249095382
-
Dagda R. K., Merrill R. A., Cribbs J. T., Chen Y., Hell J. W., Usachev Y. M., Strack S., The spinocerebellar ataxia 12 gene product and protein phosphatase 2A regulatory subunit B β 2 antagonizes neuronal survival by promoting mitochondrial fission. Journal of Biological Chemistry 2008 283 52 36241 36248 10.1074/jbc.M800989200 2-s2.0-60249095382
-
(2008)
Journal of Biological Chemistry
, vol.283
, Issue.52
, pp. 36241-36248
-
-
Dagda, R.K.1
Merrill, R.A.2
Cribbs, J.T.3
Chen, Y.4
Hell, J.W.5
Usachev, Y.M.6
Strack, S.7
-
266
-
-
66049150300
-
Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease
-
2-s2.0-66049150300
-
Yu Y.-C., Kuo C.-L., Cheng W.-L., Liu C.-S., Hsieh M., Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease. Journal of Neuroscience Research 2009 87 8 1884 1891 10.1002/jnr.22011 2-s2.0-66049150300
-
(2009)
Journal of Neuroscience Research
, vol.87
, Issue.8
, pp. 1884-1891
-
-
Yu, Y.-C.1
Kuo, C.-L.2
Cheng, W.-L.3
Liu, C.-S.4
Hsieh, M.5
-
267
-
-
79958723738
-
Mitochondrial dysfunction and oxidative stress contribute to the pathogenesis of spinocerebellar ataxia type 12 (SCA12)
-
2-s2.0-79958723738
-
Wang Y.-C., Lee C.-M., Lee L.-C., Tung L.-C., Hsieh-Li H.-M., Lee-Chen G.-J., Su M.-T., Mitochondrial dysfunction and oxidative stress contribute to the pathogenesis of spinocerebellar ataxia type 12 (SCA12). Journal of Biological Chemistry 2011 286 24 21742 21754 10.1074/jbc.M110.160697 2-s2.0-79958723738
-
(2011)
Journal of Biological Chemistry
, vol.286
, Issue.24
, pp. 21742-21754
-
-
Wang, Y.-C.1
Lee, C.-M.2
Lee, L.-C.3
Tung, L.-C.4
Hsieh-Li, H.-M.5
Lee-Chen, G.-J.6
Su, M.-T.7
-
268
-
-
34548361756
-
BH3-only proteins Bid and BimEL are differentially involved in neuronal dysfunction in mouse models of Huntington's disease
-
2-s2.0-34548361756
-
García-Martínez J. M., Pérez-Navarro E., Xifró X., Canals J. M., Díaz-Hernández M., Trioulier Y., Brouillet E., Lucas J. J., Alberch J., BH3-only proteins Bid and BimEL are differentially involved in neuronal dysfunction in mouse models of Huntington's disease. Journal of Neuroscience Research 2007 85 12 2756 2769 10.1002/jnr.21258 2-s2.0-34548361756
-
(2007)
Journal of Neuroscience Research
, vol.85
, Issue.12
, pp. 2756-2769
-
-
García-Martínez, J.M.1
Pérez-Navarro, E.2
Xifró, X.3
Canals, J.M.4
Díaz-Hernández, M.5
Trioulier, Y.6
Brouillet, E.7
Lucas, J.J.8
Alberch, J.9
-
269
-
-
83155185511
-
Compromised mitochondrial complex II in models of Machado-Joseph disease
-
2-s2.0-83155185511
-
Laço M. N., Oliveira C. R., Paulson H. L., Rego A. C., Compromised mitochondrial complex II in models of Machado-Joseph disease. Acta Biochimica et Biophysica Sinica 2012 1822 2 139 149 10.1016/j.bbadis.2011.10.010 2-s2.0-83155185511
-
(2012)
Acta Biochimica et Biophysica Sinica
, vol.1822
, Issue.2
, pp. 139-149
-
-
Laço, M.N.1
Oliveira, C.R.2
Paulson, H.L.3
Rego, A.C.4
-
270
-
-
84885850806
-
Association between Machado-Joseph disease and oxidative stress biomarkers
-
2-s2.0-84885850806
-
Pacheco L. S., da Silveira A. F., Trott A., Houenou L. J., Algarve T. D., Belló C., Lenz A. F., Mânica-Cattani M. F., da Cruz I. B. M., Association between Machado-Joseph disease and oxidative stress biomarkers. Mutation Research-Genetic Toxicology and Environmental Mutagenesis 2013 757 2 99 103 10.1016/j.mrgentox.2013.06.023 2-s2.0-84885850806
-
(2013)
Mutation Research - Genetic Toxicology and Environmental Mutagenesis
, vol.757
, Issue.2
, pp. 99-103
-
-
Pacheco, L.S.1
Da Silveira, A.F.2
Trott, A.3
Houenou, L.J.4
Algarve, T.D.5
Belló, C.6
Lenz, A.F.7
Mânica-Cattani, M.F.8
Da Cruz, I.B.M.9
-
271
-
-
84896891344
-
Aqueous extract of Gardenia jasminoides targeting oxidative stress to reduce polyQ aggregation in cell models of spinocerebellar ataxia 3
-
2-s2.0-84896891344
-
Chang K.-H., Chen W.-L., Wu Y.-R., Lin T.-H., Wu Y.-C., Chao C.-Y., Lin J.-Y., Lee L.-C., Chen Y.-C., Lee-Chen G.-J., Chen C.-M., Aqueous extract of Gardenia jasminoides targeting oxidative stress to reduce polyQ aggregation in cell models of spinocerebellar ataxia 3. Neuropharmacology 2014 81 166 175 10.1016/j.neuropharm.2014.01.032 2-s2.0-84896891344
-
(2014)
Neuropharmacology
, vol.81
, pp. 166-175
-
-
Chang, K.-H.1
Chen, W.-L.2
Wu, Y.-R.3
Lin, T.-H.4
Wu, Y.-C.5
Chao, C.-Y.6
Lin, J.-Y.7
Lee, L.-C.8
Chen, Y.-C.9
Lee-Chen, G.-J.10
Chen, C.-M.11
-
272
-
-
78650007530
-
A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein
-
2-s2.0-78650007530
-
Sugiura A., Yonashiro R., Fukuda T., Matsushita N., Nagashima S., Inatome R., Yanagi S., A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein. Mitochondrion 2011 11 1 139 146 10.1016/j.mito.2010.09.001 2-s2.0-78650007530
-
(2011)
Mitochondrion
, vol.11
, Issue.1
, pp. 139-146
-
-
Sugiura, A.1
Yonashiro, R.2
Fukuda, T.3
Matsushita, N.4
Nagashima, S.5
Inatome, R.6
Yanagi, S.7
-
273
-
-
0036327065
-
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines
-
2-s2.0-0036327065
-
Panov A. V., Gutekunst C.-A., Leavitt B. R., Hayden M. R., Burke J. R., Strittmatter W. J., Greenamyre J. T., Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nature Neuroscience 2002 5 8 731 736 10.1038/nn884 2-s2.0-0036327065
-
(2002)
Nature Neuroscience
, vol.5
, Issue.8
, pp. 731-736
-
-
Panov, A.V.1
Gutekunst, C.-A.2
Leavitt, B.R.3
Hayden, M.R.4
Burke, J.R.5
Strittmatter, W.J.6
Greenamyre, J.T.7
-
274
-
-
29044445801
-
Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-xL
-
2-s2.0-29044445801
-
Wang H.-L., Yeh T.-H., Chou A.-H., Kuo Y.-L., Luo L.-J., He C.-Y., Huang P.-C., Li A. H., Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-xL. Cellular Signalling 2006 18 4 541 552 10.1016/j.cellsig.2005.05.024 2-s2.0-29044445801
-
(2006)
Cellular Signalling
, vol.18
, Issue.4
, pp. 541-552
-
-
Wang, H.-L.1
Yeh, T.-H.2
Chou, A.-H.3
Kuo, Y.-L.4
Luo, L.-J.5
He, C.-Y.6
Huang, P.-C.7
Li, A.H.8
-
275
-
-
77954659300
-
Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons
-
2-s2.0-77954659300
-
Wang H.-L., Chou A.-H., Lin A.-C., Chen S.-Y., Weng Y.-H., Yeh T.-H., Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons. Experimental Neurology 2010 224 2 486 494 10.1016/j.expneurol.2010.05.011 2-s2.0-77954659300
-
(2010)
Experimental Neurology
, vol.224
, Issue.2
, pp. 486-494
-
-
Wang, H.-L.1
Chou, A.-H.2
Lin, A.-C.3
Chen, S.-Y.4
Weng, Y.-H.5
Yeh, T.-H.6
-
276
-
-
78651254160
-
P53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons
-
2-s2.0-78651254160
-
Chou A.-H., Lin A.-C., Hong K.-Y., Hu S.-H., Chen Y.-L., Chen J.-Y., Wang H.-L., P53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons. Neurochemistry International 2011 58 2 145 152 10.1016/j.neuint.2010.11.005 2-s2.0-78651254160
-
(2011)
Neurochemistry International
, vol.58
, Issue.2
, pp. 145-152
-
-
Chou, A.-H.1
Lin, A.-C.2
Hong, K.-Y.3
Hu, S.-H.4
Chen, Y.-L.5
Chen, J.-Y.6
Wang, H.-L.7
-
277
-
-
21544450545
-
P53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
-
2-s2.0-21544450545
-
Bae B.-I., Xu H., Igarashi S., Fujimuro M., Agrawal N., Taya Y., Hayward S. D., Moran T. H., Montell C., Ross C. A., Snyder S. H., Sawa A., p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron 2005 47 1 29 41 10.1016/j.neuron.2005.06.005 2-s2.0-21544450545
-
(2005)
Neuron
, vol.47
, Issue.1
, pp. 29-41
-
-
Bae, B.-I.1
Xu, H.2
Igarashi, S.3
Fujimuro, M.4
Agrawal, N.5
Taya, Y.6
Hayward, S.D.7
Moran, T.H.8
Montell, C.9
Ross, C.A.10
Snyder, S.H.11
Sawa, A.12
-
278
-
-
23844440646
-
Expression pattern of apoptosis-related markers in Huntington's disease
-
2-s2.0-23844440646
-
Vis J. C., Schipper E., de Boer-van H., Verbeek M. M., de Waal R. M. W., Wesseling P., ten Donkelaar H. J., Kremer B., Expression pattern of apoptosis-related markers in Huntington's disease. Acta Neuropathologica 2005 109 3 321 328 10.1007/s00401-004-0957-5 2-s2.0-23844440646
-
(2005)
Acta Neuropathologica
, vol.109
, Issue.3
, pp. 321-328
-
-
Vis, J.C.1
Schipper, E.2
De Boer-Van, H.3
Verbeek, M.M.4
De Waal, R.M.W.5
Wesseling, P.6
Ten Donkelaar, H.J.7
Kremer, B.8
-
279
-
-
14344262672
-
Full-length expanded ataxin-3 enhances mitochondrial-mediated cell death and decreases Bcl-2 expression in human neuroblastoma cells
-
2-s2.0-14344262672
-
Tsai H.-F., Tsai H.-J., Hsieh M., Full-length expanded ataxin-3 enhances mitochondrial-mediated cell death and decreases Bcl-2 expression in human neuroblastoma cells. Biochemical and Biophysical Research Communications 2004 324 4 1274 1282 10.1016/j.bbrc.2004.09.192 2-s2.0-14344262672
-
(2004)
Biochemical and Biophysical Research Communications
, vol.324
, Issue.4
, pp. 1274-1282
-
-
Tsai, H.-F.1
Tsai, H.-J.2
Hsieh, M.3
-
280
-
-
36248946819
-
The polyglutamine-expanded protein ataxin-3 decreases bcl-2 mRNA stability
-
2-s2.0-36248946819
-
Tien C.-L., Wen F.-C., Hsieh M., The polyglutamine-expanded protein ataxin-3 decreases bcl-2 mRNA stability. Biochemical and Biophysical Research Communications 2008 365 2 232 238 10.1016/j.bbrc.2007.10.162 2-s2.0-36248946819
-
(2008)
Biochemical and Biophysical Research Communications
, vol.365
, Issue.2
, pp. 232-238
-
-
Tien, C.-L.1
Wen, F.-C.2
Hsieh, M.3
-
281
-
-
43649106471
-
Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease
-
2-s2.0-43649106471
-
Duan W., Peng Q., Masuda N., Ford E., Tryggestad E., Ladenheim B., Zhao M., Cadet J. L., Wong J., Ross C. A., Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease. Neurobiology of Disease 2008 30 3 312 322 10.1016/j.nbd.2008.01.015 2-s2.0-43649106471
-
(2008)
Neurobiology of Disease
, vol.30
, Issue.3
, pp. 312-322
-
-
Duan, W.1
Peng, Q.2
Masuda, N.3
Ford, E.4
Tryggestad, E.5
Ladenheim, B.6
Zhao, M.7
Cadet, J.L.8
Wong, J.9
Ross, C.A.10
-
282
-
-
79961116788
-
Nuclear translocation of AMPK-&1 potentiates striatal neurodegeneration in Huntington's disease
-
2-s2.0-79961116788
-
Ju T.-C., Chen H.-M., Lin J.-T., Chang C.-P., Chang W.-C., Kang J.-J., Sun C.-P., Tao M.-H., Tu P.-H., Dickson D. W., Chern Y., Nuclear translocation of AMPK-&1 potentiates striatal neurodegeneration in Huntington's disease. The Journal of Cell Biology 2011 194 2 209 227 10.1083/jcb.201105010 2-s2.0-79961116788
-
(2011)
The Journal of Cell Biology
, vol.194
, Issue.2
, pp. 209-227
-
-
Ju, T.-C.1
Chen, H.-M.2
Lin, J.-T.3
Chang, C.-P.4
Chang, W.-C.5
Kang, J.-J.6
Sun, C.-P.7
Tao, M.-H.8
Tu, P.-H.9
Dickson, D.W.10
Chern, Y.11
-
283
-
-
84861233461
-
The dopaminergic stabilizer, (-)-OSU6162, rescues striatal neurons with normal and expanded polyglutamine chains in huntingtin protein from exposure to free radicals and mitochondrial toxins
-
2-s2.0-84861233461
-
Ruiz C., Casarejos M. J., Rubio I., Gines S., Puigdellivol M., Alberch J., Mena M. A., de Yebenes J. G., The dopaminergic stabilizer, (-)-OSU6162, rescues striatal neurons with normal and expanded polyglutamine chains in huntingtin protein from exposure to free radicals and mitochondrial toxins. Brain Research 2012 1459 100 112 10.1016/j.brainres.2012.04.021 2-s2.0-84861233461
-
(2012)
Brain Research
, vol.1459
, pp. 100-112
-
-
Ruiz, C.1
Casarejos, M.J.2
Rubio, I.3
Gines, S.4
Puigdellivol, M.5
Alberch, J.6
Mena, M.A.7
De Yebenes, J.G.8
-
284
-
-
43549102381
-
Increase in bax expression and apoptosis are associated in Huntington's disease progression
-
2-s2.0-43549102381
-
Teles A. V. F., Rosenstock T. R., Okuno C. S., Lopes G. S., Bertoncini C. R., Smaili S. S., Increase in bax expression and apoptosis are associated in Huntington's disease progression. Neuroscience Letters 2008 438 1 59 63 10.1016/j.neulet.2008.03.062 2-s2.0-43549102381
-
(2008)
Neuroscience Letters
, vol.438
, Issue.1
, pp. 59-63
-
-
Teles, A.V.F.1
Rosenstock, T.R.2
Okuno, C.S.3
Lopes, G.S.4
Bertoncini, C.R.5
Smaili, S.S.6
-
285
-
-
0032549811
-
A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis
-
2-s2.0-0032549811
-
Puigserver P., Wu Z., Park C. W., Graves R., Wright M., Spiegelman B. M., A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis. Cell 1998 92 6 829 839 10.1016/S0092-8674(00)81410-5 2-s2.0-0032549811
-
(1998)
Cell
, vol.92
, Issue.6
, pp. 829-839
-
-
Puigserver, P.1
Wu, Z.2
Park, C.W.3
Graves, R.4
Wright, M.5
Spiegelman, B.M.6
-
286
-
-
1542373685
-
Transcriptional regulatory circuits controlling mitochondrial biogenesis and function
-
2-s2.0-1542373685
-
Kelly D. P., Scarpulla R. C., Transcriptional regulatory circuits controlling mitochondrial biogenesis and function. Genes &; Development 2004 18 4 357 368 10.1101/gad.1177604 2-s2.0-1542373685
-
(2004)
Genes &; Development
, vol.18
, Issue.4
, pp. 357-368
-
-
Kelly, D.P.1
Scarpulla, R.C.2
-
287
-
-
33845596500
-
Peroxisome proliferator-activated receptor γ coactivator 1 coactivators, energy homeostasis, and metabolism
-
2-s2.0-33845596500
-
Handschin C., Spiegelman B. M., Peroxisome proliferator-activated receptor γ coactivator 1 coactivators, energy homeostasis, and metabolism. Endocrine Reviews 2006 27 7 728 735 10.1210/er.2006-0037 2-s2.0-33845596500
-
(2006)
Endocrine Reviews
, vol.27
, Issue.7
, pp. 728-735
-
-
Handschin, C.1
Spiegelman, B.M.2
-
288
-
-
33749042331
-
Transcriptional repression of PGC-1& by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
-
2-s2.0-33749042331
-
Cui L., Jeong H., Borovecki F., Parkhurst C. N., Tanese N., Krainc D., Transcriptional repression of PGC-1& by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006 127 1 59 69 10.1016/j.cell.2006.09.015 2-s2.0-33749042331
-
(2006)
Cell
, vol.127
, Issue.1
, pp. 59-69
-
-
Cui, L.1
Jeong, H.2
Borovecki, F.3
Parkhurst, C.N.4
Tanese, N.5
Krainc, D.6
-
289
-
-
33750437278
-
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1& in Huntington's disease neurodegeneration
-
2-s2.0-33750437278
-
Weydt P., Pineda V. V., Torrence A. E., Libby R. T., Satterfield T. F., Lazarowski E., Gilbert M. L., Morton G. J., Bammler T. K., Strand A. D., Cui L., Beyer R. P., Easley C. N., Smith A. C., Krainc D., Luquet S., Sweet I., Schwartz M. W., La Spada A. R., Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1& in Huntington's disease neurodegeneration. Cell Metabolism 2006 4 5 349 362 10.1016/j.cmet.2006.10.004 2-s2.0-33750437278
-
(2006)
Cell Metabolism
, vol.4
, Issue.5
, pp. 349-362
-
-
Weydt, P.1
Pineda, V.V.2
Torrence, A.E.3
Libby, R.T.4
Satterfield, T.F.5
Lazarowski, E.6
Gilbert, M.L.7
Morton, G.J.8
Bammler, T.K.9
Strand, A.D.10
Cui, L.11
Beyer, R.P.12
Easley, C.N.13
Smith, A.C.14
Krainc, D.15
Luquet, S.16
Sweet, I.17
Schwartz, M.W.18
La Spada, A.R.19
-
290
-
-
58649094617
-
The gene coding for PGC-1& modifies age at onset in Huntington's disease
-
2-s2.0-58649094617
-
Weydt P., Soyal S. M., Gellera C., Didonato S., Weidinger C., Oberkofler H., Landwehrmeyer G. B., Patsch W., The gene coding for PGC-1& modifies age at onset in Huntington's disease. Molecular Neurodegeneration 2009 4, article 3 10.1186/1750-1326-4-3 2-s2.0-58649094617
-
(2009)
Molecular Neurodegeneration
, vol.43
-
-
Weydt, P.1
Soyal, S.M.2
Gellera, C.3
Didonato, S.4
Weidinger, C.5
Oberkofler, H.6
Landwehrmeyer, G.B.7
Patsch, W.8
-
291
-
-
84863011541
-
Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease
-
2-s2.0-84863011541
-
Johri A., Calingasan N. Y., Hennessey T. M., Sharma A., Yang L., Wille E., Chandra A., Beal M. F., Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease. Human Molecular Genetics 2012 21 5 1124 1137 10.1093/hmg/ddr541 2-s2.0-84863011541
-
(2012)
Human Molecular Genetics
, vol.21
, Issue.5
, pp. 1124-1137
-
-
Johri, A.1
Calingasan, N.Y.2
Hennessey, T.M.3
Sharma, A.4
Yang, L.5
Wille, E.6
Chandra, A.7
Beal, M.F.8
-
292
-
-
33646136884
-
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons
-
2-s2.0-33646136884
-
Chang D. T., Rintoul G. L., Pandipati S., Reynolds I. J., Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiology of Disease 2006 22 2 388 400 10.1016/j.envpol.2005.09.025 2-s2.0-33646136884
-
(2006)
Neurobiology of Disease
, vol.22
, Issue.2
, pp. 388-400
-
-
Chang, D.T.1
Rintoul, G.L.2
Pandipati, S.3
Reynolds, I.J.4
-
293
-
-
40849147435
-
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking
-
2-s2.0-40849147435
-
Orr A. L., Li S., Wang C.-E., Li H., Wang J., Rong J., Xu X., Mastroberardino P. G., Greenamyre J. T., Li X.-J., N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. The Journal of Neuroscience 2008 28 11 2783 2792 10.1523/JNEUROSCI.0106-08.2008 2-s2.0-40849147435
-
(2008)
The Journal of Neuroscience
, vol.28
, Issue.11
, pp. 2783-2792
-
-
Orr, A.L.1
Li, S.2
Wang, C.-E.3
Li, H.4
Wang, J.5
Rong, J.6
Xu, X.7
Mastroberardino, P.G.8
Greenamyre, J.T.9
Li, X.-J.10
-
294
-
-
78650284389
-
Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli
-
2-s2.0-78650284389
-
Costa V., Giacomello M., Hudec R., Lopreiato R., Ermak G., Lim D., Malorni W., Davies K. J. A., Carafoli E., Scorrano L., Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli. EMBO Molecular Medicine 2010 2 12 490 503 10.1002/emmm.201000102 2-s2.0-78650284389
-
(2010)
EMBO Molecular Medicine
, vol.2
, Issue.12
, pp. 490-503
-
-
Costa, V.1
Giacomello, M.2
Hudec, R.3
Lopreiato, R.4
Ermak, G.5
Lim, D.6
Malorni, W.7
Davies, K.J.A.8
Carafoli, E.9
Scorrano, L.10
-
295
-
-
79952585486
-
Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: Implications for selective neuronal damage
-
2-s2.0-79952585486
-
Shirendeb U., Reddy A. P., Manczak M., Calkins M. J., Mao P., Tagle D. A., Reddy P. H., Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Human Molecular Genetics 2011 20 7 1438 1455 10.1093/hmg/ddr024 2-s2.0-79952585486
-
(2011)
Human Molecular Genetics
, vol.20
, Issue.7
, pp. 1438-1455
-
-
Shirendeb, U.1
Reddy, A.P.2
Manczak, M.3
Calkins, M.J.4
Mao, P.5
Tagle, D.A.6
Reddy, P.H.7
|