-
1
-
-
0035862754
-
Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice
-
Abel, A., Walcott, J., Woods, J., Duda, J., and Merry, D.E. (2001). Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice. Hum. Mol. Genet. 10, 107-116.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 107-116
-
-
Abel, A.1
Walcott, J.2
Woods, J.3
Duda, J.4
Merry, D.E.5
-
2
-
-
18344399938
-
Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death
-
Adachi, H., Kume, A., Li, M., Nakagomi, Y., Niwa, H., Do, J., Sang, C., Kobayashi, Y., Doyu, M., and Sobue, G. (2001). Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death. Hum. Mol. Genet. 10, 1039-1048.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1039-1048
-
-
Adachi, H.1
Kume, A.2
Li, M.3
Nakagomi, Y.4
Niwa, H.5
Do, J.6
Sang, C.7
Kobayashi, Y.8
Doyu, M.9
Sobue, G.10
-
3
-
-
0037444446
-
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein
-
Adachi, H., Katsuno, M., Minamiyama, M., Sang, C., Pagoulatos, G., Angelidis, C., Kusakabe, M., Yoshiki, A., Kobayashi, Y., Doyu, M., et al. (2003). Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J. Neurosci. 23, 2203-2211.
-
(2003)
J. Neurosci.
, vol.23
, pp. 2203-2211
-
-
Adachi, H.1
Katsuno, M.2
Minamiyama, M.3
Sang, C.4
Pagoulatos, G.5
Angelidis, C.6
Kusakabe, M.7
Yoshiki, A.8
Kobayashi, Y.9
Doyu, M.10
-
4
-
-
34248327285
-
CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model
-
Adachi, H., Waza, M., Tokui, K., Katsuno, M., Minamiyama, M., Tanaka, F., Doyu, M., and Sobue, G. (2007). CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. J. Neurosci. 27, 5115-5126.
-
(2007)
J. Neurosci.
, vol.27
, pp. 5115-5126
-
-
Adachi, H.1
Waza, M.2
Tokui, K.3
Katsuno, M.4
Minamiyama, M.5
Tanaka, F.6
Doyu, M.7
Sobue, G.8
-
5
-
-
33748741301
-
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation
-
Al-Ramahi, I., Lam, Y.C., Chen, H.-K., de Gouyon, B., Zhang, M., Perez, A.M., Branco, J., de Haro, M., Patterson, C., Zoghbi, H.Y., et al. (2006). CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J. Biol. Chem. 281, 26714-26724.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 26714-26724
-
-
Al-Ramahi, I.1
Lam, Y.C.2
Chen, H.-K.3
De Gouyon, B.4
Zhang, M.5
Perez, A.M.6
Branco, J.7
De Haro, M.8
Patterson, C.9
Zoghbi, H.Y.10
-
6
-
-
84866556522
-
Protein aggregates in Huntington's disease
-
Arrasate, M., and Finkbeiner, S. (2012). Protein aggregates in Huntington's disease. Exp. Neurol. 238, 1-11.
-
(2012)
Exp. Neurol.
, vol.238
, pp. 1-11
-
-
Arrasate, M.1
Finkbeiner, S.2
-
7
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
Arrasate, M., Mitra, S., Schweitzer, E.S., Segal, M.R., and Finkbeiner, S. (2004). Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431, 805-810.
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
8
-
-
84863178573
-
TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease
-
Baldo, B., Paganetti, P., Grueninger, S., Marcellin, D., Kaltenbach, L.S., Lo, D.C., Semmelroth, M., Zivanovic, A., Abramowski, D., Smith, D., et al. (2012). TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease. Chem. Biol. 19, 264-275.
-
(2012)
Chem. Biol.
, vol.19
, pp. 264-275
-
-
Baldo, B.1
Paganetti, P.2
Grueninger, S.3
Marcellin, D.4
Kaltenbach, L.S.5
Lo, D.C.6
Semmelroth, M.7
Zivanovic, A.8
Abramowski, D.9
Smith, D.10
-
9
-
-
0038701684
-
Huntingtin aggregation and toxicity in Huntington's disease
-
Bates, G. (2003). Huntingtin aggregation and toxicity in Huntington's disease. Lancet 361, 1642-1644.
-
(2003)
Lancet
, vol.361
, pp. 1642-1644
-
-
Bates, G.1
-
10
-
-
69949170793
-
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
-
Bauer, P.O., and Nukina, N. (2009). The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies. J. Neurochem. 110, 1737-1765.
-
(2009)
J. Neurochem.
, vol.110
, pp. 1737-1765
-
-
Bauer, P.O.1
Nukina, N.2
-
11
-
-
77749319356
-
Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein
-
Bauer, P.O., Goswami, A., Wong, H.K., Okuno, M., Kurosawa, M., Yamada, M., Miyazaki, H., Matsumoto, G., Kino, Y., Nagai, Y., et al. (2010). Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein. Nat. Biotechnol. 28, 256-263.
-
(2010)
Nat. Biotechnol.
, vol.28
, pp. 256-263
-
-
Bauer, P.O.1
Goswami, A.2
Wong, H.K.3
Okuno, M.4
Kurosawa, M.5
Yamada, M.6
Miyazaki, H.7
Matsumoto, G.8
Kino, Y.9
Nagai, Y.10
-
12
-
-
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
-
Becher, M.W., Kotzuk, J.A., Sharp, A.H., Davies, S.W., Bates, G.P., Price, D.L., and Ross, C.A. (1998). Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol. Dis. 4, 387-397.
-
(1998)
Neurobiol. Dis.
, vol.4
, 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
-
13
-
-
33748561495
-
Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers
-
Behrends, C., Langer, C.A., Boteva, R., Böttcher, U.M., Stemp, M.J., Schaffar, G., Rao, B.V., Giese, A., Kretzschmar, H., Siegers, K., et al. (2006). Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. Mol. Cell 23, 887-897.
-
(2006)
Mol. Cell
, vol.23
, pp. 887-897
-
-
Behrends, C.1
Langer, C.A.2
Boteva, R.3
Böttcher, U.M.4
Stemp, M.J.5
Schaffar, G.6
Rao, B.V.7
Giese, A.8
Kretzschmar, H.9
Siegers, K.10
-
14
-
-
0037015079
-
A linear lattice model for polyglutamine in CAG-expansion diseases
-
Bennett, M.J., Huey-Tubman, K.E., Herr, A.B., West, A.P., Ross, S.A., and Bjorkman, P.J. (2002). A linear lattice model for polyglutamine in CAG-expansion diseases. Proc. Natl. Acad. Sci. USA 99, 11634-11639.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 11634-11639
-
-
Bennett, M.J.1
Huey-Tubman, K.E.2
Herr, A.B.3
West, A.P.4
Ross, S.A.5
Bjorkman, P.J.6
-
15
-
-
33645235438
-
Pharmacological promotion of inclusion formation: A therapeutic approach for Huntington's and Parkinson's diseases
-
Bodner, R.A., Outeiro, T.F., Altmann, S., Maxwell, M.M., Cho, S.H., Hyman, B.T., McLean, P.J., Young, A.B., Housman, D.E., and Kazantsev, A.G. (2006). Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases. Proc. Natl. Acad. Sci. USA 103, 4246-4251.
-
(2006)
Proc. Natl. Acad. Sci. USA
, vol.103
, pp. 4246-4251
-
-
Bodner, R.A.1
Outeiro, T.F.2
Altmann, S.3
Maxwell, M.M.4
Cho, S.H.5
Hyman, B.T.6
McLean, P.J.7
Young, A.B.8
Housman, D.E.9
Kazantsev, A.G.10
-
16
-
-
0037041420
-
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
-
Bucciantini, M., Giannoni, E., Chiti, F., Baroni, F., Formigli, L., Zurdo, J., Taddei, N., Ramponi, G., Dobson, C.M., and Stefani, M. (2002). Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature 416, 507-511.
-
(2002)
Nature
, vol.416
, pp. 507-511
-
-
Bucciantini, M.1
Giannoni, E.2
Chiti, F.3
Baroni, F.4
Formigli, L.5
Zurdo, J.6
Taddei, N.7
Ramponi, G.8
Dobson, C.M.9
Stefani, M.10
-
17
-
-
79952193876
-
Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy
-
Burke, K.A., Godbey, J., and Legleiter, J. (2011). Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy. Methods 53, 275-284.
-
(2011)
Methods
, vol.53
, pp. 275-284
-
-
Burke, K.A.1
Godbey, J.2
Legleiter, J.3
-
18
-
-
84868142926
-
Pharmacological tuning of heat shock protein 70 modulates polyglutamine toxicity and aggregation
-
Chafekar, S.M., Wisén, S., Thompson, A.D., Echeverria, A., Walter, G.M., Evans, C.G., Makley, L.N., Gestwicki, J.E., and Duennwald, M.L. (2012). Pharmacological tuning of heat shock protein 70 modulates polyglutamine toxicity and aggregation. ACS Chem. Biol. 7, 1556-1564.
-
(2012)
ACS Chem. Biol.
, vol.7
, pp. 1556-1564
-
-
Chafekar, S.M.1
Wisén, S.2
Thompson, A.D.3
Echeverria, A.4
Walter, G.M.5
Evans, C.G.6
Makley, L.N.7
Gestwicki, J.E.8
Duennwald, M.L.9
-
19
-
-
0035800572
-
Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity
-
Chen, S., Berthelier, V., Yang, W., and Wetzel, R. (2001). Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity. J. Mol. Biol. 311, 173-182.
-
(2001)
J. Mol. Biol.
, vol.311
, pp. 173-182
-
-
Chen, S.1
Berthelier, V.2
Yang, W.3
Wetzel, R.4
-
20
-
-
0037015081
-
Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation
-
Chen, S., Ferrone, F.A., and Wetzel, R. (2002). Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation. Proc. Natl. Acad. Sci. USA 99, 11884-11889.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 11884-11889
-
-
Chen, S.1
Ferrone, F.A.2
Wetzel, R.3
-
21
-
-
7144253143
-
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture
-
Cooper, J.K., Schilling, G., Peters, M.F., Herring, W.J., Sharp, A.H., Kaminsky, Z., Masone, J., Khan, F.A., Delanoy, M., Borchelt, D.R., et al. (1998). Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum. Mol. Genet. 7, 783-790.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 783-790
-
-
Cooper, J.K.1
Schilling, G.2
Peters, M.F.3
Herring, W.J.4
Sharp, A.H.5
Kaminsky, Z.6
Masone, J.7
Khan, F.A.8
Delanoy, M.9
Borchelt, D.R.10
-
22
-
-
84857784547
-
Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse
-
Cowin, R.-M., Roscic, A., Bui, N., Graham, D., Paganetti, P., Jankowsky, J.L., Weiss, A., and Paylor, R. (2012). Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse. Behav. Brain Res. 229, 308-319.
-
(2012)
Behav. Brain Res.
, vol.229
, pp. 308-319
-
-
Cowin, R.-M.1
Roscic, A.2
Bui, N.3
Graham, D.4
Paganetti, P.5
Jankowsky, J.L.6
Weiss, A.7
Paylor, R.8
-
23
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
Cummings, C.J., Mancini, M.A., Antalffy, B., DeFranco, D.B., Orr, H.T., and Zoghbi, H.Y. (1998). Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19, 148-154.
-
(1998)
Nat. Genet.
, vol.19
, pp. 148-154
-
-
Cummings, C.J.1
Mancini, M.A.2
Antalffy, B.3
DeFranco, D.B.4
Orr, H.T.5
Zoghbi, H.Y.6
-
24
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies, S.W., Turmaine, M., Cozens, B.A., DiFiglia, M., Sharp, A.H., Ross, C.A., Scherzinger, E., Wanker, E.E., Mangiarini, L., and Bates, G.P. (1997). Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
DiFiglia, M.4
Sharp, A.H.5
Ross, C.A.6
Scherzinger, E.7
Wanker, E.E.8
Mangiarini, L.9
Bates, G.P.10
-
25
-
-
0032501740
-
Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?
-
Davies, S.W., Beardsall, K., Turmaine, M., DiFiglia, M., Aronin, N., and Bates, G.P. (1998). Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet 351, 131-133.
-
(1998)
Lancet
, vol.351
, pp. 131-133
-
-
Davies, S.W.1
Beardsall, K.2
Turmaine, M.3
DiFiglia, M.4
Aronin, N.5
Bates, G.P.6
-
26
-
-
0033614760
-
From neuronal inclusions to neurodegeneration: Neuropathological investigation of a transgenic mouse model of Huntington's disease
-
Davies, S.W., Turmaine, M., Cozens, B.A., Raza, A.S., Mahal, A., Mangiarini, L., and Bates, G.P. (1999). From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease. Philos. Trans. R. Soc. Lond., B, Biol. Sci. 354, 981-989.
-
(1999)
Philos. Trans. R. Soc. Lond., B, Biol. Sci.
, vol.354
, pp. 981-989
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
Raza, A.S.4
Mahal, A.5
Mangiarini, L.6
Bates, G.P.7
-
27
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia, M., Sapp, E., Chase, K.O., Davies, S.W., Bates, G.P., Vonsattel, J.P., and Aronin, N. (1997). Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993.
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
Davies, S.W.4
Bates, G.P.5
Vonsattel, J.P.6
Aronin, N.7
-
28
-
-
84877139577
-
p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model
-
Doi, H., Adachi, H., Katsuno, M., Minamiyama, M., Matsumoto, S., Kondo, N., Miyazaki, Y., Iida, M., Tohnai, G., Qiang, Q., et al. (2013). p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model. J. Neurosci. 33, 7710-7727.
-
(2013)
J. Neurosci.
, vol.33
, pp. 7710-7727
-
-
Doi, H.1
Adachi, H.2
Katsuno, M.3
Minamiyama, M.4
Matsumoto, S.5
Kondo, N.6
Miyazaki, Y.7
Iida, M.8
Tohnai, G.9
Qiang, Q.10
-
29
-
-
60849109193
-
CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse
-
Dragatsis, I., Goldowitz, D., Del Mar, N., Deng, Y.P., Meade, C.A., Liu, L., Sun, Z., Dietrich, P., Yue, J., and Reiner, A. (2009). CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol. Dis. 33, 315-330.
-
(2009)
Neurobiol. Dis.
, vol.33
, pp. 315-330
-
-
Dragatsis, I.1
Goldowitz, D.2
Del Mar, N.3
Deng, Y.P.4
Meade, C.A.5
Liu, L.6
Sun, Z.7
Dietrich, P.8
Yue, J.9
Reiner, A.10
-
30
-
-
33748744375
-
Green tea (-)-epigallocatechingallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models
-
Ehrnhoefer, D.E., Duennwald, M., Markovic, P., Wacker, J.L., Engemann, S., Roark, M., Legleiter, J., Marsh, J.L., Thompson, L.M., Lindquist, S., et al. (2006). Green tea (-)-epigallocatechingallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models. Hum. Mol. Genet. 15, 2743-2751.
-
(2006)
Hum. Mol. Genet.
, vol.15
, pp. 2743-2751
-
-
Ehrnhoefer, D.E.1
Duennwald, M.2
Markovic, P.3
Wacker, J.L.4
Engemann, S.5
Roark, M.6
Legleiter, J.7
Marsh, J.L.8
Thompson, L.M.9
Lindquist, S.10
-
31
-
-
37349007785
-
Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death
-
Gong, B., Lim, M.C.Y., Wanderer, J., Wyttenbach, A., and Morton, A.J. (2008). Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death. Brain Res. Bull. 75, 146-157.
-
(2008)
Brain Res. Bull.
, vol.75
, pp. 146-157
-
-
Gong, B.1
Lim, M.C.Y.2
Wanderer, J.3
Wyttenbach, A.4
Morton, A.J.5
-
32
-
-
0034329159
-
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
-
Gusella, J.F., and MacDonald, M.E. (2000). Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat. Rev. Neurosci. 1, 109-115.
-
(2000)
Nat. Rev. Neurosci.
, vol.1
, pp. 109-115
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
33
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Gutekunst, C.A., Li, S.H., Yi, H., Mulroy, J.S., Kuemmerle, S., Jones, R., Rye, D., Ferrante, R.J., Hersch, S.M., and Li, X.J. (1999). Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci. 19, 2522-2534.
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
Mulroy, J.S.4
Kuemmerle, S.5
Jones, R.6
Rye, D.7
Ferrante, R.J.8
Hersch, S.M.9
Li, X.J.10
-
34
-
-
44949181936
-
Amyloids: Friend or foe?
-
Hammer, N.D., Wang, X., McGuffie, B.A., and Chapman, M.R. (2008). Amyloids: friend or foe? J. Alzheimers Dis. 13, 407-419.
-
(2008)
J. Alzheimers Dis.
, vol.13
, pp. 407-419
-
-
Hammer, N.D.1
Wang, X.2
McGuffie, B.A.3
Chapman, M.R.4
-
35
-
-
77956184558
-
Neurotoxic protein oligomerisation associated with polyglutamine diseases
-
Hands, S.L., and Wyttenbach, A. (2010). Neurotoxic protein oligomerisation associated with polyglutamine diseases. Acta Neuropathol. 120, 419-437.
-
(2010)
Acta Neuropathol.
, vol.120
, pp. 419-437
-
-
Hands, S.L.1
Wyttenbach, A.2
-
36
-
-
7144229376
-
Spinocerebellar ataxia type 7 (SCA7): A neurodegenerative disorder with neuronal intranuclear inclusions
-
Holmberg, M., Duyckaerts, C., Dürr, A., Cancel, G., Gourfinkel-An, I., Damier, P., Faucheux, B., Trottier, Y., Hirsch, E.C., Agid, Y., et al. (1998). Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions. Hum. Mol. Genet. 7, 913-918.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 913-918
-
-
Holmberg, M.1
Duyckaerts, C.2
Dürr, A.3
Cancel, G.4
Gourfinkel-An, I.5
Damier, P.6
Faucheux, B.7
Trottier, Y.8
Hirsch, E.C.9
Agid, Y.10
-
37
-
-
34249286265
-
Hsp40 molecules that target to the ubiquitin-proteasome system decrease inclusion formation in models of polyglutamine disease
-
Howarth, J.L., Kelly, S., Keasey, M.P., Glover, C.P.J., Lee, Y.-B., Mitrophanous, K., Chapple, J.P., Gallo, J.M., Cheetham, M.E., and Uney, J.B. (2007). Hsp40 molecules that target to the ubiquitin-proteasome system decrease inclusion formation in models of polyglutamine disease. Mol. Ther. 15, 1100-1105.
-
(2007)
Mol. Ther.
, vol.15
, pp. 1100-1105
-
-
Howarth, J.L.1
Kelly, S.2
Keasey, M.P.3
Glover, C.P.J.4
Lee, Y.-B.5
Mitrophanous, K.6
Chapple, J.P.7
Gallo, J.M.8
Cheetham, M.E.9
Uney, J.B.10
-
38
-
-
58549105152
-
HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease
-
Howarth, J.L., Glover, C.P.J., and Uney, J.B. (2009). HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease. J. Neurochem. 108, 945-951.
-
(2009)
J. Neurochem.
, vol.108
, pp. 945-951
-
-
Howarth, J.L.1
Glover, C.P.J.2
Uney, J.B.3
-
39
-
-
15744387323
-
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes
-
Jana, N.R., Dikshit, P., Goswami, A., Kotliarova, S., Murata, S., Tanaka, K., and Nukina, N. (2005). Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. J. Biol. Chem. 280, 11635-11640.
-
(2005)
J. Biol. Chem.
, vol.280
, 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
-
40
-
-
84859485246
-
Toxic and non-toxic aggregates from the SBMA and normal forms of androgen receptor have distinct oligomeric structures
-
Jochum, T., Ritz, M.E., Schuster, C., Funderburk, S.F., Jehle, K., Schmitz, K., Brinkmann, F., Hirtz, M., Moss, D., and Cato, A.C.B. (2012). Toxic and non-toxic aggregates from the SBMA and normal forms of androgen receptor have distinct oligomeric structures. Biochim. Biophys. Acta 1822, 1070-1078.
-
(2012)
Biochim. Biophys. Acta
, vol.1822
, pp. 1070-1078
-
-
Jochum, T.1
Ritz, M.E.2
Schuster, C.3
Funderburk, S.F.4
Jehle, K.5
Schmitz, K.6
Brinkmann, F.7
Hirtz, M.8
Moss, D.9
Cato, A.C.B.10
-
41
-
-
84875232151
-
β-hairpin-mediated nucleation of polyglutamine amyloid formation
-
Kar, K., Hoop, C.L., Drombosky, K.W., Baker, M.A., Kodali, R., Arduini, I., van der Wel, P.C.A., Horne, W.S., and Wetzel, R. (2013). β-hairpin-mediated nucleation of polyglutamine amyloid formation. J. Mol. Biol. 425, 1183-1197.
-
(2013)
J. Mol. Biol.
, vol.425
, pp. 1183-1197
-
-
Kar, K.1
Hoop, C.L.2
Drombosky, K.W.3
Baker, M.A.4
Kodali, R.5
Arduini, I.6
Van Der Wel, P.C.A.7
Horne, W.S.8
Wetzel, R.9
-
42
-
-
28044469532
-
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease
-
Katsuno, M., Sang, C., Adachi, H., Minamiyama, M., Waza, M., Tanaka, F., Doyu, M., and Sobue, G. (2005). Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc. Natl. Acad. Sci. USA 102, 16801-16806.
-
(2005)
Proc. Natl. Acad. Sci. USA
, vol.102
, pp. 16801-16806
-
-
Katsuno, M.1
Sang, C.2
Adachi, H.3
Minamiyama, M.4
Waza, M.5
Tanaka, F.6
Doyu, M.7
Sobue, G.8
-
43
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
Kim, M., Lee, H.S., LaForet, G., McIntyre, C., Martin, E.J., Chang, P., Kim, T.W., Williams, M., Reddy, P.H., Tagle, D., et al. (1999). Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J. Neurosci. 19, 964-973.
-
(1999)
J. Neurosci.
, vol.19
, pp. 964-973
-
-
Kim, M.1
Lee, H.S.2
LaForet, G.3
McIntyre, C.4
Martin, E.J.5
Chang, P.6
Kim, T.W.7
Williams, M.8
Reddy, P.H.9
Tagle, D.10
-
44
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
-
Klement, I.A., Skinner, P.J., Kaytor, M.D., Yi, H., Hersch, S.M., Clark, H.B., Zoghbi, H.Y., and Orr, H.T. (1998). Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41-53.
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
Skinner, P.J.2
Kaytor, M.D.3
Yi, H.4
Hersch, S.M.5
Clark, H.B.6
Zoghbi, H.Y.7
Orr, H.T.8
-
45
-
-
84879154600
-
Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration
-
Kondo, N., Katsuno, M., Adachi, H., Minamiyama, M., Doi, H., Matsumoto, S., Miyazaki, Y., Iida, M., Tohnai, G., Nakatsuji, H., et al. (2013). Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration. Nat. Commun. 4, 1405.
-
(2013)
Nat. Commun.
, vol.4
, pp. 1405
-
-
Kondo, N.1
Katsuno, M.2
Adachi, H.3
Minamiyama, M.4
Doi, H.5
Matsumoto, S.6
Miyazaki, Y.7
Iida, M.8
Tohnai, G.9
Nakatsuji, H.10
-
46
-
-
0032840052
-
Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: Triple-labeling immunofluorescent study
-
Koyano, S., Uchihara, T., Fujigasaki, H., Nakamura, A., Yagishita, S., and Iwabuchi, K. (1999). Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study. Neurosci. Lett. 273, 117-120.
-
(1999)
Neurosci. Lett.
, vol.273
, pp. 117-120
-
-
Koyano, S.1
Uchihara, T.2
Fujigasaki, H.3
Nakamura, A.4
Yagishita, S.5
Iwabuchi, K.6
-
47
-
-
0032590053
-
Huntington aggregates may not predict neuronal death in Huntington's disease
-
Kuemmerle, S., Gutekunst, C.A., Klein, A.M., Li, X.J., Li, S.H., Beal, M.F., Hersch, S.M., and Ferrante, R.J. (1999). Huntington aggregates may not predict neuronal death in Huntington's disease. Ann. Neurol. 46, 842-849.
-
(1999)
Ann. Neurol.
, vol.46
, 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
-
48
-
-
66449106372
-
Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity
-
Kvam, E., Nannenga, B.L., Wang, M.S., Jia, Z., Sierks, M.R., and Messer, A. (2009). Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity. PLoS One 4, e5727.
-
(2009)
PLoS One
, vol.4
-
-
Kvam, E.1
Nannenga, B.L.2
Wang, M.S.3
Jia, Z.4
Sierks, M.R.5
Messer, A.6
-
49
-
-
33750361540
-
A century-old debate on protein aggregation and neurodegeneration enters the clinic
-
Lansbury, P.T., and Lashuel, H.A. (2006). A century-old debate on protein aggregation and neurodegeneration enters the clinic. Nature 443, 774-779.
-
(2006)
Nature
, vol.443
, pp. 774-779
-
-
Lansbury, P.T.1
Lashuel, H.A.2
-
50
-
-
1542267796
-
Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease
-
Lee, W.-C.M., Yoshihara, M., and Littleton, J.T. (2004). Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc. Natl. Acad. Sci. USA 101, 3224-3229.
-
(2004)
Proc. Natl. Acad. Sci. USA
, vol.101
, pp. 3224-3229
-
-
Lee, W.-C.M.1
Yoshihara, M.2
Littleton, J.T.3
-
51
-
-
69249139853
-
Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment
-
Legleiter, J., Lotz, G.P., Miller, J., Ko, J., Ng, C., Williams, G.L., Finkbeiner, S., Patterson, P.H., and Muchowski, P.J. (2009). Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment. J. Biol. Chem. 284, 21647-21658.
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 21647-21658
-
-
Legleiter, J.1
Lotz, G.P.2
Miller, J.3
Ko, J.4
Ng, C.5
Williams, G.L.6
Finkbeiner, S.7
Patterson, P.H.8
Muchowski, P.J.9
-
52
-
-
77951988103
-
Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo
-
Legleiter, J., Mitchell, E., Lotz, G.P., Sapp, E., Ng, C., DiFiglia, M., Thompson, L.M., and Muchowski, P.J. (2010). Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. J. Biol. Chem. 285, 14777-14790.
-
(2010)
J. Biol. Chem.
, vol.285
, 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
-
53
-
-
0031945025
-
Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats
-
Li, S.H., and Li, X.J. (1998). Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats. Hum. Mol. Genet. 7, 777-782.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 777-782
-
-
Li, S.H.1
Li, X.J.2
-
54
-
-
15144342225
-
Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy
-
Li, M., Miwa, S., Kobayashi, Y., Merry, D.E., Yamamoto, M., Tanaka, F., Doyu, M., Hashizume, Y., Fischbeck, K.H., and Sobue, G. (1998a). Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy. Ann. Neurol. 44, 249-254.
-
(1998)
Ann. Neurol.
, vol.44
, pp. 249-254
-
-
Li, M.1
Miwa, S.2
Kobayashi, Y.3
Merry, D.E.4
Yamamoto, M.5
Tanaka, F.6
Doyu, M.7
Hashizume, Y.8
Fischbeck, K.H.9
Sobue, G.10
-
55
-
-
0031682209
-
Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy
-
Li, M., Nakagomi, Y., Kobayashi, Y., Merry, D.E., Tanaka, F., Doyu, M., Mitsuma, T., Hashizume, Y., Fischbeck, K.H., and Sobue, G. (1998b). Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy. Am. J. Pathol. 153, 695-701.
-
(1998)
Am. J. Pathol.
, vol.153
, pp. 695-701
-
-
Li, M.1
Nakagomi, Y.2
Kobayashi, Y.3
Merry, D.E.4
Tanaka, F.5
Doyu, M.6
Mitsuma, T.7
Hashizume, Y.8
Fischbeck, K.H.9
Sobue, G.10
-
56
-
-
0032811511
-
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
-
Li, H., Li, S.H., Cheng, A.L., Mangiarini, L., Bates, G.P., and Li, X.J. (1999). Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum. Mol. Genet. 8, 1227-1236.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 1227-1236
-
-
Li, H.1
Li, S.H.2
Cheng, A.L.3
Mangiarini, L.4
Bates, G.P.5
Li, X.J.6
-
57
-
-
78649685457
-
Hsp70 and Hsp40 functionally interact with soluble mutant huntingtin oligomers in a classic ATP-dependent reaction cycle
-
Lotz, G.P., Legleiter, J., Aron, R., Mitchell, E.J., Huang, S.-Y., Ng, C., Glabe, C., Thompson, L.M., and Muchowski, P.J. (2010). Hsp70 and Hsp40 functionally interact with soluble mutant huntingtin oligomers in a classic ATP-dependent reaction cycle. J. Biol. Chem. 285, 38183-38193.
-
(2010)
J. Biol. Chem.
, vol.285
, pp. 38183-38193
-
-
Lotz, G.P.1
Legleiter, J.2
Aron, R.3
Mitchell, E.J.4
Huang, S.-Y.5
Ng, C.6
Glabe, C.7
Thompson, L.M.8
Muchowski, P.J.9
-
58
-
-
84874901074
-
Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: Implications for therapy
-
Malik, B., Nirmalananthan, N., Gray, A.L., La Spada, A.R., Hanna, M.G., and Greensmith, L. (2013). Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy. Brain 136, 926-943.
-
(2013)
Brain
, vol.136
, pp. 926-943
-
-
Malik, B.1
Nirmalananthan, N.2
Gray, A.L.3
La Spada, A.R.4
Hanna, M.G.5
Greensmith, L.6
-
59
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale, D., Hackam, A., Wieczorek, A., Ellerby, L., Wellington, C., McCutcheon, K., Singaraja, R., Kazemi-Esfarjani, P., Devon, R., Kim, S.U., et al. (1998). Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat. Genet. 18, 150-154.
-
(1998)
Nat. Genet.
, vol.18
, pp. 150-154
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
Ellerby, L.4
Wellington, C.5
McCutcheon, K.6
Singaraja, R.7
Kazemi-Esfarjani, P.8
Devon, R.9
Kim, S.U.10
-
60
-
-
0031948607
-
Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy
-
Merry, D.E., Kobayashi, Y., Bailey, C.K., Taye, A.A., and Fischbeck, K.H. (1998). Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy. Hum. Mol. Genet. 7, 693-701.
-
(1998)
Hum. Mol. Genet.
, vol.7
, pp. 693-701
-
-
Merry, D.E.1
Kobayashi, Y.2
Bailey, C.K.3
Taye, A.A.4
Fischbeck, K.H.5
-
61
-
-
0141891215
-
Pathogenesis of polyglutamine disorders: Aggregation revisited
-
Michalik, A., and Van Broeckhoven, C. (2003). Pathogenesis of polyglutamine disorders: aggregation revisited. Hum. Mol. Genet. 12 Spec No 2, R173-R186.
-
(2003)
Hum. Mol. Genet.
, vol.12
, Issue.SPEC NO 2
-
-
Michalik, A.1
Van Broeckhoven, C.2
-
62
-
-
27144558034
-
CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo
-
Miller, V.M., Nelson, R.F., Gouvion, C.M., Williams, A., Rodriguez-Lebron, E., Harper, S.Q., Davidson, B.L., Rebagliati, M.R., and Paulson, H.L. (2005). CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J. Neurosci. 25, 9152-9161.
-
(2005)
J. Neurosci.
, vol.25
, pp. 9152-9161
-
-
Miller, V.M.1
Nelson, R.F.2
Gouvion, C.M.3
Williams, A.4
Rodriguez-Lebron, E.5
Harper, S.Q.6
Davidson, B.L.7
Rebagliati, M.R.8
Paulson, H.L.9
-
63
-
-
81355160169
-
Identifying polyglutamine protein species in situ that best predict neurodegeneration
-
Miller, J., Arrasate, M., Brooks, E., Libeu, C.P., Legleiter, J., Hatters, D., Curtis, J., Cheung, K., Krishnan, P., Mitra, S., et al. (2011). Identifying polyglutamine protein species in situ that best predict neurodegeneration. Nat. Chem. Biol. 7, 925-934.
-
(2011)
Nat. Chem. Biol.
, vol.7
, pp. 925-934
-
-
Miller, J.1
Arrasate, M.2
Brooks, E.3
Libeu, C.P.4
Legleiter, J.5
Hatters, D.6
Curtis, J.7
Cheung, K.8
Krishnan, P.9
Mitra, S.10
-
64
-
-
73449118922
-
Autophagy and access: Understanding the role of androgen receptor subcellular localization in SBMA
-
Montie, H.L., and Merry, D.E. (2009). Autophagy and access: understanding the role of androgen receptor subcellular localization in SBMA. Autophagy 5, 1194-1197.
-
(2009)
Autophagy
, vol.5
, pp. 1194-1197
-
-
Montie, H.L.1
Merry, D.E.2
-
65
-
-
65549163327
-
Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy
-
Montie, H.L., Cho, M.S., Holder, L., Liu, Y., Tsvetkov, A.S., Finkbeiner, S., and Merry, D.E. (2009). Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy. Hum. Mol. Genet. 18, 1937-1950.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 1937-1950
-
-
Montie, H.L.1
Cho, M.S.2
Holder, L.3
Liu, Y.4
Tsvetkov, A.S.5
Finkbeiner, S.6
Merry, D.E.7
-
66
-
-
60849120450
-
Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice
-
Morton, A.J., Glynn, D., Leavens, W., Zheng, Z., Faull, R.L.M., Skepper, J.N., and Wight, J.M. (2009). Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice. Neurobiol. Dis. 33, 331-341.
-
(2009)
Neurobiol. Dis.
, vol.33
, pp. 331-341
-
-
Morton, A.J.1
Glynn, D.2
Leavens, W.3
Zheng, Z.4
Faull, R.L.M.5
Skepper, J.N.6
Wight, J.M.7
-
67
-
-
0034608868
-
Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
-
Muchowski, P.J., Schaffar, G., Sittler, A., Wanker, E.E., Hayer-Hartl, M.K., and Hartl, F.U. (2000). Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proc. Natl. Acad. Sci. USA 97, 7841-7846.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, 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
-
68
-
-
34247247115
-
A toxic monomeric conformer of the polyglutamine protein
-
Nagai, Y., Inui, T., Popiel, H.A., Fujikake, N., Hasegawa, K., Urade, Y., Goto, Y., Naiki, H., and Toda, T. (2007). A toxic monomeric conformer of the polyglutamine protein. Nat. Struct. Mol. Biol. 14, 332-340.
-
(2007)
Nat. Struct. Mol. Biol.
, vol.14
, pp. 332-340
-
-
Nagai, Y.1
Inui, T.2
Popiel, H.A.3
Fujikake, N.4
Hasegawa, K.5
Urade, Y.6
Goto, Y.7
Naiki, H.8
Toda, T.9
-
69
-
-
84876420463
-
Study of the aggregation mechanism of polyglutamine peptides using replica exchange molecular dynamics simulations
-
Nakano, M., Ebina, K., and Tanaka, S. (2013). Study of the aggregation mechanism of polyglutamine peptides using replica exchange molecular dynamics simulations. J. Mol. Model. 19, 1627-1639.
-
(2013)
J. Mol. Model.
, vol.19
, pp. 1627-1639
-
-
Nakano, M.1
Ebina, K.2
Tanaka, S.3
-
70
-
-
67649856863
-
Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity
-
Nekooki-Machida, Y., Kurosawa, M., Nukina, N., Ito, K., Oda, T., and Tanaka, M. (2009). Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity. Proc. Natl. Acad. Sci. USA 106, 9679-9684.
-
(2009)
Proc. Natl. Acad. Sci. USA
, vol.106
, pp. 9679-9684
-
-
Nekooki-Machida, Y.1
Kurosawa, M.2
Nukina, N.3
Ito, K.4
Oda, T.5
Tanaka, M.6
-
71
-
-
0031469707
-
Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse
-
Ordway, J.M., Tallaksen-Greene, S., Gutekunst, C.A., Bernstein, E.M., Cearley, J.A., Wiener, H.W., Dure, L.S., Lindsey, R., Hersch, S.M., Jope, R.S., et al. (1997). Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 91, 753-763.
-
(1997)
Cell
, vol.91
, pp. 753-763
-
-
Ordway, J.M.1
Tallaksen-Greene, S.2
Gutekunst, C.A.3
Bernstein, E.M.4
Cearley, J.A.5
Wiener, H.W.6
Dure, L.S.7
Lindsey, R.8
Hersch, S.M.9
Jope, R.S.10
-
72
-
-
34547692622
-
Trinucleotide repeat disorders
-
Orr, H.T., and Zoghbi, H.Y. (2007). Trinucleotide repeat disorders. Ann. Rev. Neurosci. 30, 575-621.
-
(2007)
Ann. Rev. Neurosci.
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
73
-
-
77953648932
-
B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy
-
Palazzolo, I., Nedelsky, N.B., Askew, C.E., Harmison, G.G., Kasantsev, A.G., Taylor, J.P., Fischbeck, K.H., and Pennuto, M. (2010). B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy. J. Neurosci. Res. 88, 2207-2216.
-
(2010)
J. Neurosci. Res.
, vol.88
, pp. 2207-2216
-
-
Palazzolo, I.1
Nedelsky, N.B.2
Askew, C.E.3
Harmison, G.G.4
Kasantsev, A.G.5
Taylor, J.P.6
Fischbeck, K.H.7
Pennuto, M.8
-
74
-
-
0030850412
-
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
-
Paulson, H.L., Perez, M.K., Trottier, Y., Trojanowski, J.Q., Subramony, S.H., Das, S.S., Vig, P., Mandel, J.L., Fischbeck, K.H., and Pittman, R.N. (1997). Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333-344.
-
(1997)
Neuron
, vol.19
, pp. 333-344
-
-
Paulson, H.L.1
Perez, M.K.2
Trottier, Y.3
Trojanowski, J.Q.4
Subramony, S.H.5
Das, S.S.6
Vig, P.7
Mandel, J.L.8
Fischbeck, K.H.9
Pittman, R.N.10
-
75
-
-
0027988041
-
Polar zippers: Their role in human disease
-
Perutz, M. (1994). Polar zippers: their role in human disease. Protein Sci. 3, 1629-1637.
-
(1994)
Protein Sci.
, vol.3
, pp. 1629-1637
-
-
Perutz, M.1
-
76
-
-
0029401042
-
Glutamine repeats as polar zippers: Their role in inherited neurodegenerative disease
-
Perutz, M.F. (1995). Glutamine repeats as polar zippers: their role in inherited neurodegenerative disease. Mol. Med. 1, 718-721.
-
(1995)
Mol. Med.
, vol.1
, pp. 718-721
-
-
Perutz, M.F.1
-
77
-
-
0028283985
-
Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases
-
Perutz, M.F., Johnson, T., Suzuki, M., and Finch, J.T. (1994). Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc. Natl. Acad. Sci. USA 91, 5355-5358.
-
(1994)
Proc. Natl. Acad. Sci. USA
, vol.91
, pp. 5355-5358
-
-
Perutz, M.F.1
Johnson, T.2
Suzuki, M.3
Finch, J.T.4
-
78
-
-
0037174879
-
Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization
-
Poirier, M.A., Li, H., Macosko, J., Cai, S., Amzel, M., and Ross, C.A. (2002). Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization. J. Biol. Chem. 277, 41032-41037.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 41032-41037
-
-
Poirier, M.A.1
Li, H.2
Macosko, J.3
Cai, S.4
Amzel, M.5
Ross, C.A.6
-
79
-
-
84878948177
-
Inhibition of protein misfolding/aggregation using polyglutamine binding peptide QBP1 as a therapy for the polyglutamine diseases
-
[Epub ahead of print]
-
Popiel, H.A., Takeuchi, T., Burke, J.R., Strittmatter, W.J., Toda, T., Wada, K., and Nagai, Y. (2013). Inhibition of protein misfolding/aggregation using polyglutamine binding peptide QBP1 as a therapy for the polyglutamine diseases. Neurotherapeutics [Epub ahead of print]
-
(2013)
Neurotherapeutics
-
-
Popiel, H.A.1
Takeuchi, T.2
Burke, J.R.3
Strittmatter, W.J.4
Toda, T.5
Wada, K.6
Nagai, Y.7
-
80
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
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., et al. (2004). Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet. 36, 585-595.
-
(2004)
Nat. Genet.
, vol.36
, 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
-
81
-
-
37049029416
-
R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice
-
Reiner, A., Del Mar, N., Deng, Y.-P., Meade, C.A., Sun, Z., and Goldowitz, D. (2007). R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice. J. Comp. Neurol. 505, 603-629.
-
(2007)
J. Comp. Neurol.
, vol.505
, pp. 603-629
-
-
Reiner, A.1
Del Mar, N.2
Deng, Y.-P.3
Meade, C.A.4
Sun, Z.5
Goldowitz, D.6
-
82
-
-
37549004790
-
Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm
-
Romero, E., Cha, G.-H., Verstreken, P., Ly, C.V., Hughes, R.E., Bellen, H.J., and Botas, J. (2008). Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm. Neuron 57, 27-40.
-
(2008)
Neuron
, vol.57
, pp. 27-40
-
-
Romero, E.1
Cha, G.-H.2
Verstreken, P.3
Ly, C.V.4
Hughes, R.E.5
Bellen, H.J.6
Botas, J.7
-
83
-
-
0031446233
-
Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
-
Ross, C.A. (1997). Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? Neuron 19, 1147-1150.
-
(1997)
Neuron
, vol.19
, pp. 1147-1150
-
-
Ross, C.A.1
-
84
-
-
27644596641
-
Opinion: What is the role of protein aggregation in neurodegeneration?
-
Ross, C.A., and Poirier, M.A. (2005). Opinion: What is the role of protein aggregation in neurodegeneration? Nat. Rev. Mol. Cell Biol. 6, 891-898.
-
(2005)
Nat. Rev. Mol. Cell Biol.
, vol.6
, pp. 891-898
-
-
Ross, C.A.1
Poirier, M.A.2
-
85
-
-
78349309239
-
17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy
-
Rusmini, P., Simonini, F., Crippa, V., Bolzoni, E., Onesto, E., Cagnin, M., Sau, D., Ferri, N., and Poletti, A. (2011). 17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy. Neurobiol. Dis. 41, 83-95.
-
(2011)
Neurobiol. Dis.
, vol.41
, pp. 83-95
-
-
Rusmini, P.1
Simonini, F.2
Crippa, V.3
Bolzoni, E.4
Onesto, E.5
Cagnin, M.6
Sau, D.7
Ferri, N.8
Poletti, A.9
-
86
-
-
0032987513
-
Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease
-
Sapp, E., Penney, J., Young, A., Aronin, N., Vonsattel, J.P., and DiFiglia, M. (1999). Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. J. Neuropathol. Exp. Neurol. 58, 165-173.
-
(1999)
J. Neuropathol. Exp. Neurol.
, vol.58
, pp. 165-173
-
-
Sapp, E.1
Penney, J.2
Young, A.3
Aronin, N.4
Vonsattel, J.P.5
DiFiglia, M.6
-
87
-
-
77649297870
-
Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease
-
Sathasivam, K., Lane, A., Legleiter, J., Warley, A., Woodman, B., Finkbeiner, S., Paganetti, P., Muchowski, P.J., Wilson, S., and Bates, G.P. (2010). Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease. Hum. Mol. Genet. 19, 65-78.
-
(2010)
Hum. Mol. Genet.
, vol.19
, 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
-
88
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
Saudou, F., Finkbeiner, S., Devys, D., and Greenberg, M.E. (1998). Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55-66.
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
89
-
-
0037461730
-
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders
-
Sánchez, I., Mahlke, C., and Yuan, J. (2003). Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373-379.
-
(2003)
Nature
, vol.421
, pp. 373-379
-
-
Sánchez, I.1
Mahlke, C.2
Yuan, J.3
-
90
-
-
18544400323
-
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
-
Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G.P., Davies, S.W., Lehrach, H., and Wanker, E.E. (1997). Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549-558.
-
(1997)
Cell
, vol.90
, pp. 549-558
-
-
Scherzinger, E.1
Lurz, R.2
Turmaine, M.3
Mangiarini, L.4
Hollenbach, B.5
Hasenbank, R.6
Bates, G.P.7
Davies, S.W.8
Lehrach, H.9
Wanker, E.E.10
-
91
-
-
37849030901
-
Polyglutamine diseases: Emerging concepts in pathogenesis and therapy
-
Shao, J., and Diamond, M.I. (2007). Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum. Mol. Genet. 16 Spec No. 2, R115-R123.
-
(2007)
Hum. Mol. Genet.
, vol.16
, Issue.SPEC NO. 2
-
-
Shao, J.1
Diamond, M.I.2
-
92
-
-
0034098057
-
Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract
-
Simeoni, S., Mancini, M.A., Stenoien, D.L., Marcelli, M., Weigel, N.L., Zanisi, M., Martini, L., and Poletti, A. (2000). Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract. Hum. Mol. Genet. 9, 133-144.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 133-144
-
-
Simeoni, S.1
Mancini, M.A.2
Stenoien, D.L.3
Marcelli, M.4
Weigel, N.L.5
Zanisi, M.6
Martini, L.7
Poletti, A.8
-
93
-
-
84859486796
-
Ubiquitination is involved in secondary growth, not initial formation of polyglutamine protein aggregates in C. Elegans
-
Skibinski, G.A., and Boyd, L. (2012). Ubiquitination is involved in secondary growth, not initial formation of polyglutamine protein aggregates in C. elegans. BMC Cell Biol. 13, 10.
-
(2012)
BMC Cell Biol.
, vol.13
, pp. 10
-
-
Skibinski, G.A.1
Boyd, L.2
-
94
-
-
0030666001
-
Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures
-
Skinner, P.J., Koshy, B.T., Cummings, C.J., Klement, I.A., Helin, K., Servadio, A., Zoghbi, H.Y., and Orr, H.T. (1997). Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures. Nature 389, 971-974.
-
(1997)
Nature
, vol.389
, 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
-
95
-
-
84864862868
-
Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models
-
Sontag, E.M., Lotz, G.P., Agrawal, N., Tran, A., Aron, R., Yang, G., Necula, M., Lau, A., Finkbeiner, S., Glabe, C., et al. (2012). Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models. J. Neurosci. 32, 11109-11119.
-
(2012)
J. Neurosci.
, vol.32
, pp. 11109-11119
-
-
Sontag, E.M.1
Lotz, G.P.2
Agrawal, N.3
Tran, A.4
Aron, R.5
Yang, G.6
Necula, M.7
Lau, A.8
Finkbeiner, S.9
Glabe, C.10
-
96
-
-
0029059477
-
Incorporation of glutamine repeats makes protein oligomerize: Implications for neurodegenerative diseases
-
Stott, K., Blackburn, J.M., Butler, P.J., and Perutz, M. (1995). Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases. Proc. Natl. Acad. Sci. USA 92, 6509-6513.
-
(1995)
Proc. Natl. Acad. Sci. USA
, vol.92
, pp. 6509-6513
-
-
Stott, K.1
Blackburn, J.M.2
Butler, P.J.3
Perutz, M.4
-
97
-
-
38349158062
-
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
-
Takahashi, T., Kikuchi, S., Katada, S., Nagai, Y., Nishizawa, M., and Onodera, O. (2008). Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum. Mol. Genet. 17, 345-356.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 345-356
-
-
Takahashi, T.1
Kikuchi, S.2
Katada, S.3
Nagai, Y.4
Nishizawa, M.5
Onodera, O.6
-
98
-
-
78049430399
-
Polyglutamine diseases: Where does toxicity come from? What is toxicity? Where are we going?
-
Takahashi, T., Katada, S., and Onodera, O. (2010). Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going? J. Mol. Cell Biol. 2, 180-191.
-
(2010)
J. Mol. Cell Biol.
, vol.2
, pp. 180-191
-
-
Takahashi, T.1
Katada, S.2
Onodera, O.3
-
99
-
-
1642633757
-
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
-
Tanaka, M., Machida, Y., Niu, S., Ikeda, T., Jana, N.R., Doi, H., Kurosawa, M., Nekooki, M., and Nukina, N. (2004). Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med. 10, 148-154.
-
(2004)
Nat. Med.
, vol.10
, pp. 148-154
-
-
Tanaka, M.1
Machida, Y.2
Niu, S.3
Ikeda, T.4
Jana, N.R.5
Doi, H.6
Kurosawa, M.7
Nekooki, M.8
Nukina, N.9
-
100
-
-
0037388418
-
Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
-
Taylor, J.P., Tanaka, F., Robitschek, J., Sandoval, C.M., Taye, A., Markovic-Plese, S., and Fischbeck, K.H. (2003). Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein. Hum. Mol. Genet. 12, 749-757.
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 749-757
-
-
Taylor, J.P.1
Tanaka, F.2
Robitschek, J.3
Sandoval, C.M.4
Taye, A.5
Markovic-Plese, S.6
Fischbeck, K.H.7
-
101
-
-
60549084901
-
17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse
-
Tokui, K., Adachi, H., Waza, M., Katsuno, M., Minamiyama, M., Doi, H., Tanaka, K., Hamazaki, J., Murata, S., Tanaka, F., et al. (2009). 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse. Hum. Mol. Genet. 18, 898-910.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 898-910
-
-
Tokui, K.1
Adachi, H.2
Waza, M.3
Katsuno, M.4
Minamiyama, M.5
Doi, H.6
Tanaka, K.7
Hamazaki, J.8
Murata, S.9
Tanaka, F.10
-
102
-
-
0028972448
-
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias
-
Trottier, Y., Lutz, Y., Stevanin, G., Imbert, G., Devys, D., Cancel, G., Saudou, F., Weber, C., David, G., and Tora, L. (1995). Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 378, 403-406.
-
(1995)
Nature
, vol.378
, pp. 403-406
-
-
Trottier, Y.1
Lutz, Y.2
Stevanin, G.3
Imbert, G.4
Devys, D.5
Cancel, G.6
Saudou, F.7
Weber, C.8
David, G.9
Tora, L.10
-
103
-
-
67650745109
-
Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease
-
Wacker, J.L., Huang, S.-Y., Steele, A.D., Aron, R., Lotz, G.P., Nguyen, Q., Giorgini, F., Roberson, E.D., Lindquist, S., Masliah, E., et al. (2009). Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease. J. Neurosci. 29, 9104-9114.
-
(2009)
J. Neurosci.
, vol.29
, pp. 9104-9114
-
-
Wacker, J.L.1
Huang, S.-Y.2
Steele, A.D.3
Aron, R.4
Lotz, G.P.5
Nguyen, Q.6
Giorgini, F.7
Roberson, E.D.8
Lindquist, S.9
Masliah, E.10
-
104
-
-
77952332824
-
Inhibition of hsp70 by methylene blue affects signaling protein function and ubiquitination and modulates polyglutamine protein degradation
-
Wang, A.M., Morishima, Y., Clapp, K.M., Peng, H.-M., Pratt, W.B., Gestwicki, J.E., Osawa, Y., and Lieberman, A.P. (2010). Inhibition of hsp70 by methylene blue affects signaling protein function and ubiquitination and modulates polyglutamine protein degradation. J. Biol. Chem. 285, 15714-15723.
-
(2010)
J. Biol. Chem.
, vol.285
, pp. 15714-15723
-
-
Wang, A.M.1
Morishima, Y.2
Clapp, K.M.3
Peng, H.-M.4
Pratt, W.B.5
Gestwicki, J.E.6
Osawa, Y.7
Lieberman, A.P.8
-
105
-
-
84865114423
-
Polyglutamine disease: From pathogenesis to therapy
-
Watson, L.M., Scholefield, J., Greenberg, L.J., and Wood, M.J.A. (2012). Polyglutamine disease: from pathogenesis to therapy. S. Afr. Med. J. 102, 481-484.
-
(2012)
S. Afr. Med. J.
, vol.102
, pp. 481-484
-
-
Watson, L.M.1
Scholefield, J.2
Greenberg, L.J.3
Wood, M.J.A.4
-
106
-
-
27144503120
-
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration
-
Waza, M., Adachi, H., Katsuno, M., Minamiyama, M., Sang, C., Tanaka, F., Inukai, A., Doyu, M., and Sobue, G. (2005). 17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat. Med. 11, 1088-1095.
-
(2005)
Nat. Med.
, vol.11
, pp. 1088-1095
-
-
Waza, M.1
Adachi, H.2
Katsuno, M.3
Minamiyama, M.4
Sang, C.5
Tanaka, F.6
Inukai, A.7
Doyu, M.8
Sobue, G.9
-
107
-
-
38049139425
-
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease
-
Weiss, A., Klein, C., Woodman, B., Sathasivam, K., Bibel, M., Régulier, E., Bates, G.P., and Paganetti, P. (2008). Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J. Neurochem. 104, 846-858.
-
(2008)
J. Neurochem.
, vol.104
, 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
-
108
-
-
84863990252
-
Physical chemistry of polyglutamine: Intriguing tales of a monotonous sequence
-
Wetzel, R. (2012). Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence. J. Mol. Biol. 421, 466-490.
-
(2012)
J. Mol. Biol.
, vol.421
, pp. 466-490
-
-
Wetzel, R.1
-
109
-
-
33751034682
-
Aggregate-prone proteins are cleared from the cytosol by autophagy: Therapeutic implications
-
Williams, A., Jahreiss, L., Sarkar, S., Saiki, S., Menzies, F.M., Ravikumar, B., and Rubinsztein, D.C. (2006). Aggregate-prone proteins are cleared from the cytosol by autophagy: therapeutic implications. Curr. Top Dev. Biol. 76, 89-101.
-
(2006)
Curr. Top Dev. Biol.
, vol.76
, pp. 89-101
-
-
Williams, A.1
Jahreiss, L.2
Sarkar, S.3
Saiki, S.4
Menzies, F.M.5
Ravikumar, B.6
Rubinsztein, D.C.7
-
110
-
-
60849119525
-
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis
-
Williams, A.J., Knutson, T.M., Colomer Gould, V.F., and Paulson, H.L. (2009). In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiol. Dis. 33, 342-353.
-
(2009)
Neurobiol. Dis.
, vol.33
, pp. 342-353
-
-
Williams, A.J.1
Knutson, T.M.2
Colomer Gould, V.F.3
Paulson, H.L.4
-
111
-
-
33846295583
-
Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice
-
Wood, N.I., Pallier, P.N., Wanderer, J., and Morton, A.J. (2007). Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice. Neurobiol. Dis. 25, 342-353.
-
(2007)
Neurobiol. Dis.
, vol.25
, pp. 342-353
-
-
Wood, N.I.1
Pallier, P.N.2
Wanderer, J.3
Morton, A.J.4
-
112
-
-
0036850529
-
Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells
-
Yang, W., Dunlap, J.R., Andrews, R.B., and Wetzel, R. (2002). Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells. Hum. Mol. Genet. 11, 2905-2917.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 2905-2917
-
-
Yang, W.1
Dunlap, J.R.2
Andrews, R.B.3
Wetzel, R.4
-
113
-
-
80055089069
-
Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice
-
Yu, Z., Wang, A.M., Adachi, H., Katsuno, M., Sobue, G., Yue, Z., Robins, D.M., and Lieberman, A.P. (2011). Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice. PLoS Genet. 7, e1002321.
-
(2011)
PLoS Genet.
, vol.7
-
-
Yu, Z.1
Wang, A.M.2
Adachi, H.3
Katsuno, M.4
Sobue, G.5
Yue, Z.6
Robins, D.M.7
Lieberman, A.P.8
-
114
-
-
0035930598
-
Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation
-
Zhou, H., Li, S.H., and Li, X.J. (2001). Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation. J. Biol. Chem. 276, 48417-48424.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 48417-48424
-
-
Zhou, H.1
Li, S.H.2
Li, X.J.3
|