-
1
-
-
0034329159
-
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
-
10.1038/35039051. 11252773
-
Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. JF Gusella ME MacDonald, Nat Rev Neurosci 2000 1 2 109 115 10.1038/35039051 11252773
-
(2000)
Nat Rev Neurosci
, vol.1
, Issue.2
, pp. 109-115
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
2
-
-
0142181243
-
How does the Huntington's disease mutation damage cells?
-
10.1126/sageke.2003.37.pe26. 13679594
-
How does the Huntington's disease mutation damage cells? DC Rubinsztein, Sci Aging Knowledge Environ 2003 2003 37 PE26 10.1126/sageke.2003.37.pe26 13679594
-
(2003)
Sci Aging Knowledge Environ
, vol.2003
, Issue.37
, pp. 26
-
-
Rubinsztein, D.C.1
-
3
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
10.1126/science.277.5334.1990. 9302293
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. M DiFiglia E Sapp KO Chase SW Davies GP Bates JP Vonsattel N Aronin, Science 1997 277 5334 1990 1993 10.1126/science.277.5334. 1990 9302293
-
(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
-
4
-
-
0030850412
-
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
-
10.1016/S0896-6273(00)80943-5. 9292723
-
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. HL Paulson MK Perez Y Trottier JQ Trojanowski SH Subramony SS Das P Vig JL Mandel KH Fischbeck RN Pittman, Neuron 1997 19 2 333 344 10.1016/S0896-6273(00)80943-5 9292723
-
(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
-
5
-
-
0037077040
-
Toxic proteins in neurodegenerative disease
-
10.1126/science.1067122. 12065827
-
Toxic proteins in neurodegenerative disease. JP Taylor J Hardy KH Fischbeck, Science 2002 296 5575 1991 1995 10.1126/science.1067122 12065827
-
(2002)
Science
, vol.296
, Issue.5575
, pp. 1991-1995
-
-
Taylor, J.P.1
Hardy, J.2
Fischbeck, K.H.3
-
6
-
-
33745545413
-
Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8
-
10.1038/ng1827. 16804541
-
Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8. ML Moseley T Zu Y Ikeda W Gao AK Mosemiller RS Daughters G Chen MR Weatherspoon HB Clark TJ Ebner, Nat Genet 2006 38 7 758 769 10.1038/ng1827 16804541
-
(2006)
Nat Genet
, vol.38
, Issue.7
, pp. 758-769
-
-
Moseley, M.L.1
Zu, T.2
Ikeda, Y.3
Gao, W.4
Mosemiller, A.K.5
Daughters, R.S.6
Chen, G.7
Weatherspoon, M.R.8
Clark, H.B.9
Ebner, T.J.10
-
7
-
-
0037408279
-
Transcriptional abnormalities in Huntington disease
-
10.1016/S0168-9525(03)00074-X. 12711212
-
Transcriptional abnormalities in Huntington disease. KL Sugars DC Rubinsztein, Trends Genet 2003 19 5 233 238 10.1016/S0168-9525(03)00074-X 12711212
-
(2003)
Trends Genet
, vol.19
, Issue.5
, pp. 233-238
-
-
Sugars, K.L.1
Rubinsztein, D.C.2
-
8
-
-
0035919701
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
-
10.1126/science.1059581. 11408619
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. C Zuccato A Ciammola D Rigamonti BR Leavitt D Goffredo L Conti ME MacDonald RM Friedlander V Silani MR Hayden, Science 2001 293 5529 493 498 10.1126/science.1059581 11408619
-
(2001)
Science
, vol.293
, Issue.5529
, pp. 493-498
-
-
Zuccato, C.1
Ciammola, A.2
Rigamonti, D.3
Leavitt, B.R.4
Goffredo, D.5
Conti, L.6
MacDonald, M.E.7
Friedlander, R.M.8
Silani, V.9
Hayden, M.R.10
-
9
-
-
0041353535
-
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes
-
10.1038/ng1219. 12881722
-
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. C Zuccato M Tartari A Crotti D Goffredo M Valenza L Conti T Cataudella BR Leavitt MR Hayden T Timmusk, Nat Genet 2003 35 1 76 83 10.1038/ng1219 12881722
-
(2003)
Nat Genet
, vol.35
, Issue.1
, pp. 76-83
-
-
Zuccato, C.1
Tartari, M.2
Crotti, A.3
Goffredo, D.4
Valenza, M.5
Conti, L.6
Cataudella, T.7
Leavitt, B.R.8
Hayden, M.R.9
Timmusk, T.10
-
10
-
-
25444474703
-
Mitochondria take center stage in aging and neurodegeneration
-
10.1002/ana.20624. 16178023
-
Mitochondria take center stage in aging and neurodegeneration. MF Beal, Ann Neurol 2005 58 4 495 505 10.1002/ana.20624 16178023
-
(2005)
Ann Neurol
, vol.58
, Issue.4
, pp. 495-505
-
-
Beal, M.F.1
-
11
-
-
0141750470
-
Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila
-
10.1016/S0896-6273(03)00594-4. 14527431
-
Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. S Gunawardena LS Her RG Brusch RA Laymon IR Niesman B Gordesky-Gold L Sintasath NM Bonini LS Goldstein, Neuron 2003 40 1 25 40 10.1016/S0896-6273(03)00594-4 14527431
-
(2003)
Neuron
, vol.40
, Issue.1
, pp. 25-40
-
-
Gunawardena, S.1
Her, L.S.2
Brusch, R.G.3
Laymon, R.A.4
Niesman, I.R.5
Gordesky-Gold, B.6
Sintasath, L.7
Bonini, N.M.8
Goldstein, L.S.9
-
12
-
-
0036850524
-
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis
-
10.1093/hmg/11.23.2815. 12393793
-
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. RR Singaraja S Hadano M Metzler S Givan CL Wellington S Warby A Yanai CA Gutekunst BR Leavitt H Yi, Hum Mol Genet 2002 11 23 2815 2828 10.1093/hmg/11.23.2815 12393793
-
(2002)
Hum Mol Genet
, vol.11
, Issue.23
, pp. 2815-2828
-
-
Singaraja, R.R.1
Hadano, S.2
Metzler, M.3
Givan, S.4
Wellington, C.L.5
Warby, S.6
Yanai, A.7
Gutekunst, C.A.8
Leavitt, B.R.9
Yi, H.10
-
13
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
10.1126/science.292.5521.1552. 11375494
-
Impairment of the ubiquitin-proteasome system by protein aggregation. NF Bence RM Sampat RR Kopito, Science 2001 292 5521 1552 1555 10.1126/science.292. 5521.1552 11375494
-
(2001)
Science
, vol.292
, Issue.5521
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
14
-
-
13244258435
-
Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation
-
10.1016/j.molcel.2004.12.021. 15694337
-
Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. EJ Bennett NF Bence R Jayakumar RR Kopito, Mol Cell 2005 17 3 351 365 10.1016/j.molcel.2004. 12.021 15694337
-
(2005)
Mol Cell
, vol.17
, Issue.3
, pp. 351-365
-
-
Bennett, E.J.1
Bence, N.F.2
Jayakumar, R.3
Kopito, R.R.4
-
15
-
-
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
-
10.1093/hmg/10.10.1049. 11331615
-
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. NR Jana EA Zemskov G Wang N Nukina, Hum Mol Genet 2001 10 10 1049 1059 10.1093/hmg/10.10. 1049 11331615
-
(2001)
Hum Mol Genet
, vol.10
, Issue.10
, pp. 1049-1059
-
-
Jana, N.R.1
Zemskov, E.A.2
Wang, G.3
Nukina, N.4
-
16
-
-
33745088678
-
Molecular pathogenesis of spinocerebellar ataxias
-
10.1093/brain/awl081. 16613893
-
Molecular pathogenesis of spinocerebellar ataxias. AM Duenas R Goold P Giunti, Brain 2006 129 Pt 6 1357 1370 10.1093/brain/awl081 16613893
-
(2006)
Brain
, vol.129
, Issue.PART 6
, pp. 1357-1370
-
-
Duenas, A.M.1
Goold, R.2
Giunti, P.3
-
17
-
-
33745879143
-
Intracellular protein degradation: From a vague idea thru the lysosome and the ubiquitin-proteasome system and onto human diseases and drug targeting
-
16816126
-
Intracellular protein degradation: from a vague idea thru the lysosome and the ubiquitin-proteasome system and onto human diseases and drug targeting. A Ciechanover, Exp Biol Med (Maywood) 2006 231 7 1197 1211 16816126
-
(2006)
Exp Biol Med (Maywood)
, vol.231
, Issue.7
, pp. 1197-1211
-
-
Ciechanover, A.1
-
18
-
-
0346727127
-
Protein degradation and protection against misfolded or damaged proteins
-
10.1038/nature02263. 14685250
-
Protein degradation and protection against misfolded or damaged proteins. AL Goldberg, Nature 2003 426 6968 895 899 10.1038/nature02263 14685250
-
(2003)
Nature
, vol.426
, Issue.6968
, pp. 895-899
-
-
Goldberg, A.L.1
-
19
-
-
33745593049
-
Activity-dependent dynamics and sequestration of proteasomes in dendritic spines
-
10.1038/nature04769. 16810255
-
Activity-dependent dynamics and sequestration of proteasomes in dendritic spines. B Bingol EM Schuman, Nature 2006 441 7097 1144 1148 10.1038/nature04769 16810255
-
(2006)
Nature
, vol.441
, Issue.7097
, pp. 1144-1148
-
-
Bingol, B.1
Schuman, E.M.2
-
20
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
10.1038/502. 9620770
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. CJ Cummings MA Mancini B Antalffy DB DeFranco HT Orr HY Zoghbi, Nat Genet 1998 19 2 148 154 10.1038/502 9620770
-
(1998)
Nat Genet
, 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
-
21
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
10.1016/S0092-8674(00)80513-9. 9267033
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. SW Davies M Turmaine BA Cozens M DiFiglia AH Sharp CA Ross E Scherzinger EE Wanker L Mangiarini GP Bates, Cell 1997 90 3 537 548 10.1016/S0092-8674(00)80513-9 9267033
-
(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
-
23
-
-
9144223729
-
Inefficient degradation of truncated polyglutamine proteins by the proteasome
-
15470501. 10.1038/sj.emboj.7600426
-
Inefficient degradation of truncated polyglutamine proteins by the proteasome. CI Holmberg KE Staniszewski KN Mensah A Matouschek RI Morimoto, Embo J 2004 23 21 4307 4318 15470501 10.1038/sj.emboj.7600426
-
(2004)
Embo J
, 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
-
24
-
-
1842766144
-
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
-
10.1016/S1097-2765(04)00151-0. 15068806
-
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins. P Venkatraman R Wetzel M Tanaka N Nukina AL Goldberg, Mol Cell 2004 14 1 95 104 10.1016/S1097-2765(04)00151-0 15068806
-
(2004)
Mol Cell
, vol.14
, Issue.1
, pp. 95-104
-
-
Venkatraman, P.1
Wetzel, R.2
Tanaka, M.3
Nukina, N.4
Goldberg, A.L.5
-
26
-
-
0035891863
-
Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex
-
10.1093/hmg/10.24.2821. 11734547
-
Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex. A Matilla C Gorbea DD Einum J Townsend A Michalik C van Broeckhoven CC Jensen KJ Murphy LJ Ptacek YH Fu, Hum Mol Genet 2001 10 24 2821 2831 10.1093/hmg/10.24.2821 11734547
-
(2001)
Hum Mol Genet
, 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
Ptacek, L.J.9
Fu, Y.H.10
-
27
-
-
0034703397
-
Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein
-
11001934
-
Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. JD Davidson B Riley EN Burright LA Duvick HY Zoghbi HT Orr, Hum Mol Genet 2000 9 15 2305 2312 11001934
-
(2000)
Hum Mol Genet
, 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
-
28
-
-
23044515498
-
The solution structure of the Josephin domain of ataxin-3: Structural determinants for molecular recognition
-
16020535. 10.1073/pnas.0501732102
-
The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition. G Nicastro RP Menon L Masino PP Knowles NQ McDonald A Pastore, Proc Natl Acad Sci U S A 2005 102 30 10493 10498 16020535 10.1073/pnas.0501732102
-
(2005)
Proc Natl Acad Sci U S a
, vol.102
, Issue.30
, pp. 10493-10498
-
-
Nicastro, G.1
Menon, R.P.2
Masino, L.3
Knowles, P.P.4
McDonald, N.Q.5
Pastore, A.6
-
29
-
-
22844451581
-
Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product
-
15750336
-
Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product. Y Park S Hong SJ Kim S Kang, Mol Cells 2005 19 1 23 30 15750336
-
(2005)
Mol Cells
, vol.19
, Issue.1
, pp. 23-30
-
-
Park, Y.1
Hong, S.2
Kim, S.J.3
Kang, S.4
-
30
-
-
4444303263
-
Generalized brain and skin proteasome inhibition in Huntington's disease
-
10.1002/ana.20207. 15349858
-
Generalized brain and skin proteasome inhibition in Huntington's disease. H Seo KC Sonntag O Isacson, Ann Neurol 2004 56 3 319 328 10.1002/ana.20207 15349858
-
(2004)
Ann Neurol
, vol.56
, Issue.3
, pp. 319-328
-
-
Seo, H.1
Sonntag, K.C.2
Isacson, O.3
-
31
-
-
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
-
10.1093/hmg/ddi064. 15661755
-
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. AB Bowman SY Yoo NP Dantuma HY Zoghbi, Hum Mol Genet 2005 14 5 679 691 10.1093/hmg/ddi064 15661755
-
(2005)
Hum Mol Genet
, vol.14
, Issue.5
, pp. 679-691
-
-
Bowman, A.B.1
Yoo, S.Y.2
Dantuma, N.P.3
Zoghbi, H.Y.4
-
32
-
-
0037134460
-
Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival
-
10.1074/jbc.M107706200. 11782460
-
Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival. Q Ding JJ Lewis KM Strum E Dimayuga AJ Bruce-Keller JC Dunn JN Keller, J Biol Chem 2002 277 16 13935 13942 10.1074/jbc.M107706200 11782460
-
(2002)
J Biol Chem
, vol.277
, Issue.16
, pp. 13935-13942
-
-
Ding, Q.1
Lewis, J.J.2
Strum, K.M.3
Dimayuga, E.4
Bruce-Keller, A.J.5
Dunn, J.C.6
Keller, J.N.7
-
33
-
-
0347064159
-
Neuronal induction of the immunoproteasome in Huntington's disease
-
14684867
-
Neuronal induction of the immunoproteasome in Huntington's disease. M Diaz-Hernandez F Hernandez E Martin-Aparicio P Gomez-Ramos MA Moran JG Castano I Ferrer J Avila JJ Lucas, J Neurosci 2003 23 37 11653 11661 14684867
-
(2003)
J Neurosci
, vol.23
, Issue.37
, pp. 11653-11661
-
-
Diaz-Hernandez, M.1
Hernandez, F.2
Martin-Aparicio, E.3
Gomez-Ramos, P.4
Moran, M.A.5
Castano, J.G.6
Ferrer, I.7
Avila, J.8
Lucas, J.J.9
-
34
-
-
29644433718
-
Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: Exclusion of proteasome activator REGgamma as a therapeutic target
-
10.1093/hmg/ddi423. 16311253
-
Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target. JS Bett GM Goellner B Woodman G Pratt M Rechsteiner GP Bates, Hum Mol Genet 2006 15 1 33 44 10.1093/hmg/ddi423 16311253
-
(2006)
Hum Mol Genet
, vol.15
, Issue.1
, pp. 33-44
-
-
Bett, J.S.1
Goellner, G.M.2
Woodman, B.3
Pratt, G.4
Rechsteiner, M.5
Bates, G.P.6
-
36
-
-
0141650794
-
Monitoring the ubiquitin/proteasome system in conformational diseases
-
Good review of methods to study UPS activity. 10.1016/S1568-1637(03) 00031-X. 14522245
-
Monitoring the ubiquitin/proteasome system in conformational diseases. K Lindsten NP Dantuma, Ageing Res Rev 2003 2 4 433 449 Good review of methods to study UPS activity 10.1016/S1568-1637(03)00031-X 14522245
-
(2003)
Ageing Res Rev
, vol.2
, Issue.4
, pp. 433-449
-
-
Lindsten, K.1
Dantuma, N.P.2
-
37
-
-
33746487396
-
Expanded polyglutamines impair synaptic transmission and ubiquitin-proteasome system in Caenorhabditis elegans
-
10.1111/j.1471-4159.2006.03895.x. 16805848
-
Expanded polyglutamines impair synaptic transmission and ubiquitin-proteasome system in Caenorhabditis elegans. LA Khan PO Bauer H Miyazaki KS Lindenberg BG Landwehrmeyer N Nukina, J Neurochem 2006 98 2 576 587 10.1111/j.1471-4159.2006.03895.x 16805848
-
(2006)
J Neurochem
, 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
-
38
-
-
0043208904
-
A transgenic mouse model of the ubiquitin/proteasome system
-
10.1038/nbt851. 12872133
-
A transgenic mouse model of the ubiquitin/proteasome system. K Lindsten V Menendez-Benito MG Masucci NP Dantuma, Nat Biotechnol 2003 21 8 897 902 10.1038/nbt851 12872133
-
(2003)
Nat Biotechnol
, vol.21
, Issue.8
, pp. 897-902
-
-
Lindsten, K.1
Menendez-Benito, V.2
Masucci, M.G.3
Dantuma, N.P.4
-
39
-
-
0034646426
-
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
-
10717003. 10.1073/pnas.97.6.2898
-
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease. A Wyttenbach J Carmichael J Swartz RA Furlong Y Narain J Rankin DC Rubinsztein, Proc Natl Acad Sci U S A 2000 97 6 2898 2903 10717003 10.1073/pnas.97.6.2898
-
(2000)
Proc Natl Acad Sci U S a
, 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
-
40
-
-
0037422010
-
Parkin is a component of an SCF-like ubiquitin ligase complex and protects postmitotic neurons from kainate excitotoxicity
-
10.1016/S0896-6273(03)00084-9. 12628165
-
Parkin is a component of an SCF-like ubiquitin ligase complex and protects postmitotic neurons from kainate excitotoxicity. JF Staropoli C McDermott C Martinat B Schulman E Demireva A Abeliovich, Neuron 2003 37 5 735 749 10.1016/S0896-6273(03)00084-9 12628165
-
(2003)
Neuron
, vol.37
, Issue.5
, pp. 735-749
-
-
Staropoli, J.F.1
McDermott, C.2
Martinat, C.3
Schulman, B.4
Demireva, E.5
Abeliovich, A.6
-
41
-
-
0038159253
-
Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function
-
10.1074/jbc.M212235200. 12676955
-
Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function. YC Tsai PS Fishman NV Thakor GA Oyler, J Biol Chem 2003 278 24 22044 22055 10.1074/jbc.M212235200 12676955
-
(2003)
J Biol Chem
, vol.278
, Issue.24
, pp. 22044-22055
-
-
Tsai, Y.C.1
Fishman, P.S.2
Thakor, N.V.3
Oyler, G.A.4
-
42
-
-
0348017149
-
The proteasome of Mycobacterium tuberculosis is required for resistance to nitric oxide
-
10.1126/science.1091176. 14671303
-
The proteasome of Mycobacterium tuberculosis is required for resistance to nitric oxide. KH Darwin S Ehrt JC Gutierrez-Ramos N Weich CF Nathan, Science 2003 302 5652 1963 1966 10.1126/science.1091176 14671303
-
(2003)
Science
, vol.302
, Issue.5652
, pp. 1963-1966
-
-
Darwin, K.H.1
Ehrt, S.2
Gutierrez-Ramos, J.C.3
Weich, N.4
Nathan, C.F.5
-
43
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
10.1038/ng1362. 15146184
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. B Ravikumar C Vacher Z Berger JE Davies S Luo LG Oroz F Scaravilli DF Easton R Duden CJ O'Kane, Nat Genet 2004 36 6 585 595 10.1038/ng1362 15146184
-
(2004)
Nat Genet
, 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
-
44
-
-
31544454404
-
Rapamycin alleviates toxicity of different aggregate-prone proteins
-
10.1093/hmg/ddi458. 16368705
-
Rapamycin alleviates toxicity of different aggregate-prone proteins. Z Berger B Ravikumar FM Menzies LG Oroz BR Underwood MN Pangalos I Schmitt U Wullner BO Evert CJ O'Kane, Hum Mol Genet 2006 15 3 433 442 10.1093/hmg/ddi458 16368705
-
(2006)
Hum Mol Genet
, vol.15
, Issue.3
, pp. 433-442
-
-
Berger, Z.1
Ravikumar, B.2
Menzies, F.M.3
Oroz, L.G.4
Underwood, B.R.5
Pangalos, M.N.6
Schmitt, I.7
Wullner, U.8
Evert, B.O.9
O'Kane, C.J.10
-
46
-
-
9444239187
-
Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme
-
10.1074/jbc.271.32.19385. 8702625
-
Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. MA Kalchman RK Graham G Xia HB Koide JG Hodgson KC Graham YP Goldberg RD Gietz CM Pickart MR Hayden, J Biol Chem 1996 271 32 19385 19394 10.1074/jbc.271.32.19385 8702625
-
(1996)
J Biol Chem
, vol.271
, Issue.32
, pp. 19385-19394
-
-
Kalchman, M.A.1
Graham, R.K.2
Xia, G.3
Koide, H.B.4
Hodgson, J.G.5
Graham, K.C.6
Goldberg, Y.P.7
Gietz, R.D.8
Pickart, C.M.9
Hayden, M.R.10
-
47
-
-
0035889973
-
Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease
-
11698589
-
Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease. E Martin-Aparicio A Yamamoto F Hernandez R Hen J Avila JJ Lucas, J Neurosci 2001 21 22 8772 8781 11698589
-
(2001)
J Neurosci
, vol.21
, Issue.22
, pp. 8772-8781
-
-
Martin-Aparicio, E.1
Yamamoto, A.2
Hernandez, F.3
Hen, R.4
Avila, J.5
Lucas, J.J.6
-
48
-
-
0026065338
-
Lactacystin, a novel microbial metabolite, induces neuritogenesis of neuroblastoma cells
-
1848215
-
Lactacystin, a novel microbial metabolite, induces neuritogenesis of neuroblastoma cells. S Omura T Fujimoto K Otoguro K Matsuzaki R Moriguchi H Tanaka Y Sasaki, J Antibiot (Tokyo) 1991 44 1 113 116 1848215
-
(1991)
J Antibiot (Tokyo)
, vol.44
, Issue.1
, pp. 113-116
-
-
Omura, S.1
Fujimoto, T.2
Otoguro, K.3
Matsuzaki, K.4
Moriguchi, R.5
Tanaka, H.6
Sasaki, Y.7
-
49
-
-
0029033981
-
Inhibition of proteasome activities and subunit-specific amino-terminal threonine modification by lactacystin
-
10.1126/science.7732382. 7732382
-
Inhibition of proteasome activities and subunit-specific amino-terminal threonine modification by lactacystin. G Fenteany RF Standaert WS Lane S Choi EJ Corey SL Schreiber, Science 1995 268 5211 726 731 10.1126/science.7732382 7732382
-
(1995)
Science
, vol.268
, Issue.5211
, pp. 726-731
-
-
Fenteany, G.1
Standaert, R.F.2
Lane, W.S.3
Choi, S.4
Corey, E.J.5
Schreiber, S.L.6
-
50
-
-
0027050714
-
Epoxomicin, a new antitumor agent of microbial origin
-
1468981
-
Epoxomicin, a new antitumor agent of microbial origin. M Hanada K Sugawara K Kaneta S Toda Y Nishiyama K Tomita H Yamamoto M Konishi T Oki, J Antibiot (Tokyo) 1992 45 11 1746 1752 1468981
-
(1992)
J Antibiot (Tokyo)
, vol.45
, Issue.11
, pp. 1746-1752
-
-
Hanada, M.1
Sugawara, K.2
Kaneta, K.3
Toda, S.4
Nishiyama, Y.5
Tomita, K.6
Yamamoto, H.7
Konishi, M.8
Oki, T.9
-
51
-
-
0033517032
-
Total synthesis of the potent proteasome inhibitor epoxomicin: A useful tool for understanding proteasome biology
-
10.1016/S0960-894X(99)00376-5. 10465562
-
Total synthesis of the potent proteasome inhibitor epoxomicin: a useful tool for understanding proteasome biology. N Sin KB Kim M Elofsson L Meng H Auth BH Kwok CM Crews, Bioorg Med Chem Lett 1999 9 15 2283 2288 10.1016/S0960- 894X(99)00376-5 10465562
-
(1999)
Bioorg Med Chem Lett
, vol.9
, Issue.15
, pp. 2283-2288
-
-
Sin, N.1
Kim, K.B.2
Elofsson, M.3
Meng, L.4
Auth, H.5
Kwok, B.H.6
Crews, C.M.7
-
52
-
-
0036566266
-
Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy
-
10.1093/hmg/11.9.1107. 11978769
-
Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy. B Ravikumar R Duden DC Rubinsztein, Hum Mol Genet 2002 11 9 1107 1117 10.1093/hmg/11.9.1107 11978769
-
(2002)
Hum Mol Genet
, vol.11
, Issue.9
, pp. 1107-1117
-
-
Ravikumar, B.1
Duden, R.2
Rubinsztein, D.C.3
-
53
-
-
0034754875
-
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
-
11359930
-
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. S Waelter A Boeddrich R Lurz E Scherzinger G Lueder H Lehrach EE Wanker, Mol Biol Cell 2001 12 5 1393 1407 11359930
-
(2001)
Mol Biol 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
-
54
-
-
0033030565
-
Evidence for proteasome involvement in polyglutamine disease: Localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
-
10.1093/hmg/8.4.673. 10072437
-
Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Y Chai SL Koppenhafer SJ Shoesmith MK Perez HL Paulson, Hum Mol Genet 1999 8 4 673 682 10.1093/hmg/8.4.673 10072437
-
(1999)
Hum Mol Genet
, vol.8
, Issue.4
, pp. 673-682
-
-
Chai, Y.1
Koppenhafer, S.L.2
Shoesmith, S.J.3
Perez, M.K.4
Paulson, H.L.5
-
55
-
-
0033391428
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
-
10.1016/S0896-6273(00)81035-1. 10624951
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. CJ Cummings E Reinstein Y Sun B Antalffy Y Jiang A Ciechanover HT Orr AL Beaudet HY Zoghbi, Neuron 1999 24 4 879 892 10.1016/S0896-6273(00)81035-1 10624951
-
(1999)
Neuron
, vol.24
, Issue.4
, pp. 879-892
-
-
Cummings, C.J.1
Reinstein, E.2
Sun, Y.3
Antalffy, B.4
Jiang, Y.5
Ciechanover, A.6
Orr, H.T.7
Beaudet, A.L.8
Zoghbi, H.Y.9
-
56
-
-
0034597833
-
Identification of genes that modify ataxin-1-induced neurodegeneration
-
10.1038/35040584. 11081516
-
Identification of genes that modify ataxin-1-induced neurodegeneration. P Fernandez-Funez ML Nino-Rosales B de Gouyon WC She JM Luchak P Martinez E Turiegano J Benito M Capovilla PJ Skinner, Nature 2000 408 6808 101 106 10.1038/35040584 11081516
-
(2000)
Nature
, vol.408
, Issue.6808
, pp. 101-106
-
-
Fernandez-Funez, P.1
Nino-Rosales, M.L.2
De Gouyon, B.3
She, W.C.4
Luchak, J.M.5
Martinez, P.6
Turiegano, E.7
Benito, J.8
Capovilla, M.9
Skinner, P.J.10
-
57
-
-
0037108725
-
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins
-
10.1093/hmg/11.22.2689. 12374759
-
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins. LG Verhoef K Lindsten MG Masucci NP Dantuma, Hum Mol Genet 2002 11 22 2689 2700 10.1093/hmg/11.22.2689 12374759
-
(2002)
Hum Mol Genet
, vol.11
, Issue.22
, pp. 2689-2700
-
-
Verhoef, L.G.1
Lindsten, K.2
Masucci, M.G.3
Dantuma, N.P.4
-
58
-
-
29644437591
-
Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy
-
10.1093/hmg/ddi422. 16311254
-
Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy. JE Davies S Sarkar DC Rubinsztein, Hum Mol Genet 2006 15 1 23 31 10.1093/hmg/ddi422 16311254
-
(2006)
Hum Mol Genet
, vol.15
, Issue.1
, pp. 23-31
-
-
Davies, J.E.1
Sarkar, S.2
Rubinsztein, D.C.3
-
59
-
-
0037461730
-
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders
-
10.1038/nature01301. 12540902
-
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. I Sanchez C Mahlke J Yuan, Nature 2003 421 6921 373 379 10.1038/nature01301 12540902
-
(2003)
Nature
, vol.421
, Issue.6921
, pp. 373-379
-
-
Sanchez, I.1
Mahlke, C.2
Yuan, J.3
-
60
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
10.1016/S0092-8674(00)81369-0. 8898202
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. L Mangiarini K Sathasivam M Seller B Cozens A Harper C Hetherington M Lawton Y Trottier H Lehrach SW Davies, Cell 1996 87 3 493 506 10.1016/S0092-8674(00)81369-0 8898202
-
(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
-
61
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
10.1016/S0092-8674(00)80623-6. 10778856
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. A Yamamoto JJ Lucas R Hen, Cell 2000 101 1 57 66 10.1016/S0092-8674(00)80623-6 10778856
-
(2000)
Cell
, vol.101
, Issue.1
, pp. 57-66
-
-
Yamamoto, A.1
Lucas, J.J.2
Hen, R.3
-
62
-
-
0037421691
-
SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity
-
10.1016/S0896-6273(02)01190-X. 12575948
-
SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity. SY Yoo ME Pennesi EJ Weeber B Xu R Atkinson S Chen DL Armstrong SM Wu JD Sweatt HY Zoghbi, Neuron 2003 37 3 383 401 10.1016/S0896-6273(02)01190-X 12575948
-
(2003)
Neuron
, vol.37
, Issue.3
, pp. 383-401
-
-
Yoo, S.Y.1
Pennesi, M.E.2
Weeber, E.J.3
Xu, B.4
Atkinson, R.5
Chen, S.6
Armstrong, D.L.7
Wu, S.M.8
Sweatt, J.D.9
Zoghbi, H.Y.10
-
63
-
-
33947164372
-
Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation
-
10.1038/sj.emboj.7601592. 17318184
-
Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation. NP Bhutani, EMBO J 2007 26 5 1385 1396 10.1038/sj.emboj.7601592 17318184
-
(2007)
EMBO J
, vol.26
, Issue.5
, pp. 1385-1396
-
-
Bhutani, N.P.1
-
64
-
-
34547807613
-
Global changes to the ubiquitin system in Huntington's disease
-
10.1038/nature06022. 17687326
-
Global changes to the ubiquitin system in Huntington's disease. EJ Bennett, Nature 2007 448 7154 704 708 10.1038/nature06022 17687326
-
(2007)
Nature
, vol.448
, Issue.7154
, pp. 704-708
-
-
Bennett, E.J.1
|