1 |
The Huntington' Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington' disease chromosomes. Cell 72, 971-983
DOI
ScienceOn
|
2 |
Conway, K. A., Lee, S. J., Rochet, J. C., Ding, T. T., Harper, J. D., et al. (2000) Accelerated oligomerization by Parkinson's disease linked alpha-synuclein mutants. Ann. N. Y. Acad. Sci. 920, 42-45
|
3 |
Cummings, C. J., Orr, H. T., and Zoghbi, H. Y. (1999) Progress in pathogenesis studies of spinocerebellar ataxia type 1. Philos. Trans. R Soc. Lond. B Biol. Sci. 354, 1079-1081
DOI
ScienceOn
|
4 |
Cummings, C. J., Sun, Y., Opal, P., Antalffy, B., Mestril, R., et al. (2001) Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet. 10, 1511-1518
DOI
ScienceOn
|
5 |
Emamian, E. S., Kaytor, M. D., Duvick, L. A., Zu, T., Tousey, S. K., et al. (2003) Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron 38, 375-387
DOI
ScienceOn
|
6 |
Hong, S., Kim, S. J., Ka, S., Choi, I., and Kang, S. (2002) USP7, a ubiquitin-specific protease, interacts with ataxin-1, the SCA1 gene product. Mol. Cell. Neurosci. 20, 298-306
DOI
ScienceOn
|
7 |
Kayed, R., Head, E., Thompson, J. L., McIntire, T. M., Milton, S. C., et al. (2003) Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis. Science 300, 486-489
DOI
ScienceOn
|
8 |
Koide, R., Ikeuchi, T., Onodera, O., Tanaka, H., Igarashi, S., et al. (1994) Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nat. Genet. 6, 9-13
DOI
ScienceOn
|
9 |
McNaught, K. S., Mytilineou, C., Jnobaptiste, R., Yabut, J., Shashidharan, P., et al. (2002) Impairment of the ubiquitinproteasome system causes dopaminergic cell death and inclusion body formation in ventral mesencephalic cultures. J. Neurochem. 81, 301-306
DOI
ScienceOn
|
10 |
Nahreini, P., Andreatta, C., and Prasad, K. N. (2001) Proteasome activity is critical for the cAMP-induced differentiation of neuroblastoma cells. Cell. Mol. Neurobiol. 21, 509-521
DOI
ScienceOn
|
11 |
Nishitoh, H., Matsuzawa, A., Tobiume, K., Saegusa, K., Takeda, K., et al. (2002) ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats. Genes Dev. 16, 1345-1355
DOI
ScienceOn
|
12 |
Sanchez, I., Mahlke, C., and Yuan, J. (2003) Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373-379
DOI
ScienceOn
|
13 |
Thompson, M. G., Thom, A., Partridge, K., Garden, K., Campbell, G. P., et al. (1999) Stimulation of myofibrillar protein degradation and expression of mRNA encoding the ubiquitinproteasome system in C(2)C(12) myotubes by dexamethasone:effect of the proteasome inhibitor MG-132. J. Cell. Physiol. 181, 455-461
DOI
ScienceOn
|
14 |
Yasuda, S., Inoue, K., Hirabayashi, M., Higashiyama, H., Yamamoto, Y., et al. (1999) Triggering of neuronal cell death by accumulation of activated SEK1 on nuclear polyglutamine aggregations in PML bodies. Genes Cells 4, 743-756
DOI
ScienceOn
|
15 |
Zhang, M., Pickart, C. M., and Coffino, P. (2003) Determinants of proteasome recognition of ornithine decarboxylase, a ubiquitin-independent substrate. EMBO J. 22, 1488-1496
DOI
ScienceOn
|
16 |
Andreatta, C., Nahreini, P., Hovland, A. R., Kumar, B., Edwards-Prasad, J., et al. (2001) Use of short-lived green fluorescent protein for the detection of proteasome inhibition. Biotechniques 30, 656−660
|
17 |
McNaught, K. S., Olanow, C. W., Halliwell, B., Isacson, O., and Jenner, P. (2001) Failure of the ubiquitin-proteasome system in Parkinson' disease. Nat. Rev. Neurosci. 2, 589-594
DOI
ScienceOn
|
18 |
Cummings, C. J., Mancini, M. A., Antalffy, B., DeFranco, D. B., Orr, H. T., et al. (1998) Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19, 148-154
DOI
ScienceOn
|
19 |
Yokota, T., Sugawara, K., Ito, K., Takahashi, R., Ariga, H., et al. (2003) Down regulation of DJ-1 enhances cell death by oxidative stress, ER stress, and proteasome inhibition. Biochem. Biophys. Res. Commun. 312, 1342-1348
DOI
ScienceOn
|
20 |
DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., et al. (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993
DOI
|
21 |
Klement, I. A., Skinner, P. J., Kaytor, M. D., Yi, H., Hersch, S. M., et al. (1998) Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41-53
DOI
ScienceOn
|
22 |
David, G., Abbas, N., Stevanin, G., Durr, A., Yvert, G., et al. (1997) Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion. Nat. Genet. 17, 65-70
DOI
ScienceOn
|
23 |
Pulst, S. M., Nechiporuk, A., Nechiporuk, T., Gispert, S., Chen, X. N., et al. (1996) Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat. Genet. 14, 269-276
DOI
ScienceOn
|
24 |
Shang, F., Nowell, T. R. Jr., and Taylor, A. (2001) Removal of oxidatively damaged proteins from lens cells by the ubiquitin-proteasome pathway. Exp. Eye Res. 73, 229-238
DOI
ScienceOn
|
25 |
Bence, N. F., Sampat, R. M., and Kopito, R. R. (2001) Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292, 1552-1555
DOI
ScienceOn
|
26 |
Klement, I. A., Zoghbi, H. Y., and Orr, H. T. (1999) Pathogenesis of polyglutamine-induced disease: A model for SCA1. Mol. Genet. Metab. 66, 172-178
DOI
ScienceOn
|
27 |
Kouroku, Y., Fujita, E., Jimbo, A., Kikuchi, T., Yamagata, T., et al. (2002) Polyglutamine aggregates stimulate ER stress signals and caspase-12 activation. Hum. Mol. Genet. 11, 1505-1515
DOI
ScienceOn
|
28 |
Skinner, P. J., Vierra-Green, C. A., Emamian, E., Zoghbi, H. Y., and Orr, H. T. (2002) Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice. Neuromolecular Med. 1, 33-42
DOI
ScienceOn
|
29 |
Kim, S.-J., Kim, T.-S., Hong, S., Rhim, H., Kim, I. Y., et al. (2003b) Oxidative stimuli affect polyglutmine aggregation cell death in human mutant ataxin-1-expressing cells. Nueurosci. Lett. 348, 21-24
DOI
ScienceOn
|
30 |
Banerjee, D. and Liefshitz, A. (2001) Potential of the proteasomal inhibitor MG-132 as an anticancer agent, alone and in combination. Anticancer Res. 21, 3941-3947
|
31 |
Morishima-Kawashima, M. and Ihara, Y. (2002) Alzheimer' disease: beta-amyloid protein and tau. J. Neurosci. Res. 70, 392-401
DOI
ScienceOn
|
32 |
Davidson, J. D., Riley, B., Burright, E. N., Duvick, L. A., Zoghbi, H. Y., et al. (2000) Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. Hum. Mol. Genet. 9, 2305-2312
DOI
ScienceOn
|
33 |
Zhuchenko, O., Bailey, J., Bonnen, P., Ashizawa, T., Stockton, D. W., et al. (1997) Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel. Nat. Genet. 15, 62-69
DOI
ScienceOn
|
34 |
Conway, K. A., Harper, J. D., and Lansbury, P. T. (1998) Accelerated in vitro fibril formation by a mutant alpha-synuclein linked to early-onset Parkinson disease. Nat. Med. 4, 1318-1320
DOI
ScienceOn
|
35 |
Brown, R. H. Jr. (1998) SOD1 aggregates in ALS: cause, correlate or consequence? Nat. Med. 4, 1362-1364
DOI
ScienceOn
|
36 |
Kim, S.-J., Kim, T.-S., Kim, I. Y., Hong, S., Rhim, H., et al. (2003a) Polyglutamine-expanded ataxin-1 recruits Cu/Zn superoxide dismutase into the nucleus of HeLa cells. Biochem. Biophys. Res. Commun. 307, 660-665
DOI
ScienceOn
|
37 |
Murakami, Y., Matsufuji, S., Kameji, T., Hayashi, S., Igarashi, K., et al. (1992) Ornithine decarboxylase is degraded by the 26S proteasome without ubiquitination. Nature 360, 597-599
DOI
ScienceOn
|
38 |
Johnston, J. A., Ward, C. L., and Kopito, R. R. (1998) Aggresomes:a cellular response to misfolded proteins. J. Cell Biol. 143, 1883-1898
DOI
|
39 |
30 Proteasome Impairment by Mutant Ataxin-1 Orr, H. T. and Zoghbi, H. Y. (2001) SCA1 molecular genetics: a history of a 13 year collaboration against glutamines. Hum. Mol. Genet. 10, 2307-2311
DOI
ScienceOn
|
40 |
Rideout, H. J., Larsen, K. E., Sulzer, D., and Stefanis, L. (2001) Proteasomal inhibition leads to formation of ubiquitin/alphasynuclein-immunoreactive inclusions in PC12 cells. J. Neurochem. 78, 899-908
DOI
ScienceOn
|
41 |
Keller, J. N., Hanni, K. B., and Markesbery, W. R. (2000) Impaired proteasome function in Alzheimer' disease. J. Neurochem. 75, 436-439
DOI
|
42 |
McNaught, K. S. and Jenner, P. (2001) Proteasomal function is impaired in substantia nigra in Parkinson' disease. Neurosci. Lett. 297, 191-194
DOI
ScienceOn
|
43 |
Chen, H. K., Fernandez-Funez, P., Acevedo, S. F., Lam, Y. C., Kaytor, M. D., et al. (2003) Interaction of akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell 113, 457-468
DOI
ScienceOn
|
44 |
Jana, N. R., Zemskov, E. A., Wang, G., and Nukina, N. (2001) Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cyto- chrome c release. Hum. Mol. Genet. 10, 1049-1059
DOI
ScienceOn
|
45 |
Kawaguchi, Y., Okamoto, T., Taniwaki, M., Aizawa, M., Inoue, M., et al. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet. 8, 221-228
DOI
ScienceOn
|
46 |
Bucciantini, M., Giannoni, E., Chiti, F., Baroni, F., Formigli, L., et al. (2002) Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature 416, 507-511
DOI
ScienceOn
|