-
1
-
-
0034077041
-
Mice lacking alpha-synuclein display functional deficits in the nigrostriatal dopamine system
-
Abeliovich A, Schmitz Y, Farinas I, Choi-Lundberg D, Ho WH, Castillo PE, Shinsky N, Verdugo JM, Armanini M, Ryan A, et al. Mice lacking alpha-synuclein display functional deficits in the nigrostriatal dopamine system. Neuron 2000;25:239-252.
-
(2000)
Neuron
, vol.25
, pp. 239-252
-
-
Abeliovich, A.1
Schmitz, Y.2
Farinas, I.3
Choi-Lundberg, D.4
Ho, W.H.5
Castillo, P.E.6
Shinsky, N.7
Verdugo, J.M.8
Armanini, M.9
Ryan, A.10
-
2
-
-
63049091236
-
A systematic survey identifies prions and illuminates sequence features of prionogenic proteins
-
Alberti S, Halfmann R, King O, Kapila A, Lindquist S. A systematic survey identifies prions and illuminates sequence features of prionogenic proteins. Cell 2009;137:146-158.
-
(2009)
Cell
, vol.137
, pp. 146-158
-
-
Alberti, S.1
Halfmann, R.2
King, O.3
Kapila, A.4
Lindquist, S.5
-
3
-
-
0015859467
-
Principles that govern the folding of protein chains
-
Anfinsen CB. Principles that govern the folding of protein chains. Science 1973; 181:223-230.
-
(1973)
Science
, vol.181
, pp. 223-230
-
-
Anfinsen, C.B.1
-
4
-
-
56949083650
-
Hsp104 targets multiple intermediates on the amyloid pathway and suppresses the seeding capacity of Abeta fibrils and protofibrils
-
Arimon M, Grimminger V, Sanz F, Lashuel HA. Hsp104 targets multiple intermediates on the amyloid pathway and suppresses the seeding capacity of Abeta fibrils and protofibrils. J Mol Biol 2008;384:1157-1173.
-
(2008)
J Mol Biol
, vol.384
, pp. 1157-1173
-
-
Arimon, M.1
Grimminger, V.2
Sanz, F.3
Lashuel, H.A.4
-
5
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004;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
-
6
-
-
44249120315
-
Effect of gene therapy on visual function in Leber's congenital amaurosis
-
Bainbridge JW, Smith AJ, Barker SS, Robbie S, Henderson R, Balaggan K, Viswanathan A, Holder GE, Stockman A, Tyler N, et al. Effect of gene therapy on visual function in Leber's congenital amaurosis. N Engl J Med 2008;358:2231-2239.
-
(2008)
N Engl J Med
, vol.358
, pp. 2231-2239
-
-
Bainbridge, J.W.1
Smith, A.J.2
Barker, S.S.3
Robbie, S.4
Henderson, R.5
Balaggan, K.6
Viswanathan, A.7
Holder, G.E.8
Stockman, A.9
Tyler, N.10
-
8
-
-
0037023781
-
Mammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophy
-
Bao YP, Cook LJ, O'Donovan D, Uyama E, Rubinsztein DC. Mammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophy. J Biol Chem 2002;277:12263-12269.
-
(2002)
J Biol Chem
, vol.277
, pp. 12263-12269
-
-
Bao, Y.P.1
Cook, L.J.2
O'Donovan, D.3
Uyama, E.4
Rubinsztein, D.C.5
-
9
-
-
27144456262
-
The amino-terminal domain of ClpB supports binding to strongly aggregated proteins
-
Barnett ME, Nagy M, Kedzierska S, Zolkiewski M. The amino-terminal domain of ClpB supports binding to strongly aggregated proteins. J Biol Chem 2005; 280:34940-34945.
-
(2005)
J Biol Chem
, vol.280
, pp. 34940-34945
-
-
Barnett, M.E.1
Nagy, M.2
Kedzierska, S.3
Zolkiewski, M.4
-
10
-
-
66849106554
-
An expanding arsenal of experimental methods yields an explosion of insights into protein folding mechanisms
-
Bartlett AI, Radford SE. An expanding arsenal of experimental methods yields an explosion of insights into protein folding mechanisms. Nat Struct Mol Biol 2009;16:582-588.
-
(2009)
Nat Struct Mol Biol
, vol.16
, pp. 582-588
-
-
Bartlett, A.I.1
Radford, S.E.2
-
11
-
-
0037117485
-
Mechanism of inactivation on prion conversion of the Saccharomyces cerevisiae Ure2 protein
-
Baxa U, Speransky V, Steven AC, Wickner RB. Mechanism of inactivation on prion conversion of the Saccharomyces cerevisiae Ure2 protein. Proc Natl Acad Sci USA 2002;99:5253-5260.
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 5253-5260
-
-
Baxa, U.1
Speransky, V.2
Steven, A.C.3
Wickner, R.B.4
-
12
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence NF, Sampat RM, Kopito RR. Impairment of the ubiquitin-proteasome system by protein aggregation. Science 2001;292:1552-1555.
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
13
-
-
0037986392
-
Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis
-
Berson JF, Theos AC, Harper DC, Tenza D, Raposo G, Marks MS. Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis. J Cell Biol 2003;161:521-533.
-
(2003)
J Cell Biol
, vol.161
, pp. 521-533
-
-
Berson, J.F.1
Theos, A.C.2
Harper, D.C.3
Tenza, D.4
Raposo, G.5
Marks, M.S.6
-
14
-
-
70350519391
-
A kinetic assessment of the C
-
Bieschke J, Cohen E, Murray A, Dillin A, Kelly JW. A kinetic assessment of the C. elegans amyloid disaggregation activity enables uncoupling of disassembly and proteolysis. Protein Sci 2009;18:2231-2241.
-
(2009)
Elegans amyloid disaggregation activity enables uncoupling of disassembly and proteolysis. Protein Sci
, vol.18
, pp. 2231-2241
-
-
Bieschke, J.1
Cohen, E.2
Murray, A.3
Dillin, A.4
Kelly, J.W.5
-
15
-
-
31444442284
-
Substrate binding to the molecular chaperone Hsp104 and its regulation by nucleotides
-
Bosl B, Grimminger V, Walter S. Substrate binding to the molecular chaperone Hsp104 and its regulation by nucleotides. J Biol Chem 2005;280:38170-38176.
-
(2005)
J Biol Chem
, vol.280
, pp. 38170-38176
-
-
Bosl, B.1
Grimminger, V.2
Walter, S.3
-
16
-
-
0037333666
-
Staging of brain pathology related to sporadic Parkinson's disease
-
Braak H, Del Tredici K, Rub U, de Vos RA, Jansen Steur EN, Braak E. Staging of brain pathology related to sporadic Parkinson's disease. Neurobiol Aging 2003;24:197-211.
-
(2003)
Neurobiol Aging
, vol.24
, pp. 197-211
-
-
Braak, H.1
Del Tredici, K.2
Rub, U.3
de Vos, R.A.4
Jansen Steur, E.N.5
Braak, E.6
-
17
-
-
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 CM, Stefani M. Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature 2002;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
-
18
-
-
0034662915
-
Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease
-
Carmichael J, Chatellier J, Woolfson A, Milstein C, Fersht AR, Rubinsztein DC. Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease. Proc Natl Acad Sci USA 2000;97:9701-9705.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 9701-9705
-
-
Carmichael, J.1
Chatellier, J.2
Woolfson, A.3
Milstein, C.4
Fersht, A.R.5
Rubinsztein, D.C.6
-
19
-
-
23144459082
-
Molecular recycling within amyloid fibrils
-
Carulla N, Caddy GL, Hall DR, Zurdo J, Gairi M, Feliz M, Giralt E, Robinson CV, Dobson CM. Molecular recycling within amyloid fibrils. Nature 2005;436:554-558.
-
(2005)
Nature
, vol.436
, pp. 554-558
-
-
Carulla, N.1
Caddy, G.L.2
Hall, D.R.3
Zurdo, J.4
Gairi, M.5
Feliz, M.6
Giralt, E.7
Robinson, C.V.8
Dobson, C.M.9
-
20
-
-
21244466032
-
A chaperone pathway in protein disaggregation: Hsp26 alters the nature of protein aggregates to facilitate reactivation by Hsp104
-
Cashikar AG, Duennwald M, Lindquist SL. A chaperone pathway in protein disaggregation: Hsp26 alters the nature of protein aggregates to facilitate reactivation by Hsp104. J Biol Chem 2005;280:23869-23875.
-
(2005)
J Biol Chem
, vol.280
, pp. 23869-23875
-
-
Cashikar, A.G.1
Duennwald, M.2
Lindquist, S.L.3
-
21
-
-
0036238432
-
Defining a pathway of communication from the C-terminal peptide binding domain to the N-terminal ATPase domain in a AAA protein
-
Cashikar AG, Schirmer EC, Hattendorf DA, Glover JR, Ramakrishnan MS, Ware DM, Lindquist SL. Defining a pathway of communication from the C-terminal peptide binding domain to the N-terminal ATPase domain in a AAA protein. Mol Cell 2002;9:751-760.
-
(2002)
Mol Cell
, vol.9
, pp. 751-760
-
-
Cashikar, A.G.1
Schirmer, E.C.2
Hattendorf, D.A.3
Glover, J.R.4
Ramakrishnan, M.S.5
Ware, D.M.6
Lindquist, S.L.7
-
22
-
-
0037551741
-
Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders
-
Caughey B, Lansbury PT. Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders. Annu Rev Neurosci 2003;26:267-298.
-
(2003)
Annu Rev Neurosci
, vol.26
, pp. 267-298
-
-
Caughey, B.1
Lansbury, P.T.2
-
23
-
-
27944450427
-
Intrasarcoplasmic amyloidosis impairs proteolytic function of proteasomes in cardiomyocytes by compromising substrate uptake
-
Chen Q, Liu JB, Horak KM, Zheng H, Kumarapeli AR, Li J, Li F, Gerdes AM, Wawrousek EF, Wang X. Intrasarcoplasmic amyloidosis impairs proteolytic function of proteasomes in cardiomyocytes by compromising substrate uptake. Circ Res 2005;97:1018-1026.
-
(2005)
Circ Res
, vol.97
, pp. 1018-1026
-
-
Chen, Q.1
Liu, J.B.2
Horak, K.M.3
Zheng, H.4
Kumarapeli, A.R.5
Li, J.6
Li, F.7
Gerdes, A.M.8
Wawrousek, E.F.9
Wang, X.10
-
24
-
-
0029052468
-
Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [PSI +]
-
Chernoff YO, Lindquist SL, Ono B, Inge-Vechtomov SG, Liebman SW. Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [PSI +]. Science 1995;268:880-884.
-
(1995)
Science
, vol.268
, pp. 880-884
-
-
Chernoff, Y.O.1
Lindquist, S.L.2
Ono, B.3
Inge-Vechtomov, S.G.4
Liebman, S.W.5
-
25
-
-
0033500152
-
Evidence for a protein mutator in yeast: role of the Hsp70-related chaperone ssb in formation, stability, and toxicity of the [PSI+] prion
-
Chernoff YO, Newnam GP, Kumar J, Allen K, Zink AD. Evidence for a protein mutator in yeast: role of the Hsp70-related chaperone ssb in formation, stability, and toxicity of the [PSI+] prion. Mol Cell Biol 1999;19:8103-8112.
-
(1999)
Mol Cell Biol
, vol.19
, pp. 8103-8112
-
-
Chernoff, Y.O.1
Newnam, G.P.2
Kumar, J.3
Allen, K.4
Zink, A.D.5
-
26
-
-
6344228684
-
Mutation E46K increases phospholipid binding and assembly into filaments of human alphasynuclein
-
Choi W, Zibaee S, Jakes R, Serpell LC, Davletov B, Crowther RA, Goedert M. Mutation E46K increases phospholipid binding and assembly into filaments of human alphasynuclein. FEBS Lett 2004;576:363-368.
-
(2004)
FEBS Lett
, vol.576
, pp. 363-368
-
-
Choi, W.1
Zibaee, S.2
Jakes, R.3
Serpell, L.C.4
Davletov, B.5
Crowther, R.A.6
Goedert, M.7
-
27
-
-
33748792821
-
Opposing activities protect against age-onset proteotoxicity
-
Cohen E, Bieschke J, Perciavalle RM, Kelly JW, Dillin A. Opposing activities protect against age-onset proteotoxicity. Science 2006;313:1604-1610.
-
(2006)
Science
, vol.313
, pp. 1604-1610
-
-
Cohen, E.1
Bieschke, J.2
Perciavalle, R.M.3
Kelly, J.W.4
Dillin, A.5
-
28
-
-
0023579739
-
Purification and characterization of a peptide from amyloid-rich pancreases of type 2 diabetic patients
-
Cooper GJ, Willis AC, Clark A, Turner RC, Sim RB, Reid KB. Purification and characterization of a peptide from amyloid-rich pancreases of type 2 diabetic patients. Proc Natl Acad Sci USA 1987;84:8628-8632.
-
(1987)
Proc Natl Acad Sci USA
, vol.84
, pp. 8628-8632
-
-
Cooper, G.J.1
Willis, A.C.2
Clark, A.3
Turner, R.C.4
Sim, R.B.5
Reid, K.B.6
-
29
-
-
30744451401
-
DNA-induced switch from independent to sequential dTTP hydrolysis in the bacteriophage T7 DNA helicase
-
Crampton DJ, Mukherjee S, Richardson CC. DNA-induced switch from independent to sequential dTTP hydrolysis in the bacteriophage T7 DNA helicase. Mol Cell 2006;21:165-174.
-
(2006)
Mol Cell
, vol.21
, pp. 165-174
-
-
Crampton, D.J.1
Mukherjee, S.2
Richardson, C.C.3
-
30
-
-
0034613450
-
Characterisation of isolated alpha-synuclein filaments from substantia nigra of Parkinson's disease brain
-
Crowther RA, Daniel SE, Goedert M. Characterisation of isolated alpha-synuclein filaments from substantia nigra of Parkinson's disease brain. Neurosci Lett 2000; 292:128-130.
-
(2000)
Neurosci Lett
, vol.292
, pp. 128-130
-
-
Crowther, R.A.1
Daniel, S.E.2
Goedert, M.3
-
31
-
-
56349168452
-
Autophagy and aging: keeping that old broom working
-
Cuervo AM. Autophagy and aging: keeping that old broom working. Trends Genet 2008;24:604-612.
-
(2008)
Trends Genet
, vol.24
, pp. 604-612
-
-
Cuervo, A.M.1
-
32
-
-
77951183978
-
Prion-like disorders: blurring the divide between transmissibility and infectivity
-
Cushman M, Johnson BS, King OD, Gitler AD, Shorter J. Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci 2010;123:1191-1201.
-
(2010)
J Cell Sci
, vol.123
, pp. 1191-1201
-
-
Cushman, M.1
Johnson, B.S.2
King, O.D.3
Gitler, A.D.4
Shorter, J.5
-
33
-
-
33747040086
-
Infection by ME7 prion is not modified in transgenic mice expressing the yeast chaperone Hsp104 in neurons
-
Dandoy-Dron F, Bogdanova A, Beringue V, Bailly Y, Tovey MG, Laude H, Dron M. Infection by ME7 prion is not modified in transgenic mice expressing the yeast chaperone Hsp104 in neurons. Neurosci Lett 2006;405:181-185.
-
(2006)
Neurosci Lett
, vol.405
, pp. 181-185
-
-
Dandoy-Dron, F.1
Bogdanova, A.2
Beringue, V.3
Bailly, Y.4
Tovey, M.G.5
Laude, H.6
Dron, M.7
-
34
-
-
77951540816
-
Caspase activation precedes and leads to tangles
-
de Calignon A, Fox LM, Pitstick R, Carlson GA, Bacskai BJ, Spires-Jones TL, Hyman BT. Caspase activation precedes and leads to tangles. Nature 2010;464:1201-1204.
-
(2010)
Nature
, vol.464
, pp. 1201-1204
-
-
de Calignon, A.1
Fox, L.M.2
Pitstick, R.3
Carlson, G.A.4
Bacskai, B.J.5
Spires-Jones, T.L.6
Hyman, B.T.7
-
35
-
-
0347357617
-
Protein folding and misfolding
-
Dobson CM. Protein folding and misfolding. Nature 2003;426:884-890.
-
(2003)
Nature
, vol.426
, pp. 884-890
-
-
Dobson, C.M.1
-
36
-
-
44449179474
-
Chaperone-dependent amyloid assembly protects cells from prion toxicity
-
Douglas PM, Treusch S, Ren HY, Halfmann R, Duennwald ML, Lindquist S, Cyr DM. Chaperone-dependent amyloid assembly protects cells from prion toxicity. Proc Natl Acad Sci USA 2008;105:7206-7211.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 7206-7211
-
-
Douglas, P.M.1
Treusch, S.2
Ren, H.Y.3
Halfmann, R.4
Duennwald, M.L.5
Lindquist, S.6
Cyr, D.M.7
-
37
-
-
34547455220
-
Inaugural article: collaboration between the ClpB AAA+ remodeling protein and the DnaK chaperone system
-
Doyle SM, Hoskins JR, Wickner S. Inaugural article: collaboration between the ClpB AAA+ remodeling protein and the DnaK chaperone system. Proc Natl Acad Sci USA 2007a;104:11138-11144.
-
(2007)
Proc Natl Acad Sci USA
, vol.104
, pp. 11138-11144
-
-
Doyle, S.M.1
Hoskins, J.R.2
Wickner, S.3
-
38
-
-
33846941900
-
Asymmetric deceleration of ClpB or Hsp104 ATPase activity unleashes protein-remodeling activity
-
Doyle SM, Shorter J, Zolkiewski M, Hoskins JR, Lindquist S, Wickner S. Asymmetric deceleration of ClpB or Hsp104 ATPase activity unleashes protein-remodeling activity. Nat Struct Mol Biol 2007b;14:114-122.
-
(2007)
Nat Struct Mol Biol
, vol.14
, pp. 114-122
-
-
Doyle, S.M.1
Shorter, J.2
Zolkiewski, M.3
Hoskins, J.R.4
Lindquist, S.5
Wickner, S.6
-
39
-
-
58149181486
-
Hsp104 and ClpB: protein disaggregating machines
-
Doyle SM, Wickner S. Hsp104 and ClpB: protein disaggregating machines. Trends Biochem Sci 2009;34:40-48.
-
(2009)
Trends Biochem Sci
, vol.34
, pp. 40-48
-
-
Doyle, S.M.1
Wickner, S.2
-
40
-
-
33749818217
-
The structural biology of protein aggregation diseases: Fundamental questions and some answers
-
Eisenberg D, Nelson R, Sawaya MR, Balbirnie M, Sambashivan S, Ivanova MI, Madsen AO, Riekel C. The structural biology of protein aggregation diseases: Fundamental questions and some answers. Acc Chem Res 2006;39:568-575.
-
(2006)
Acc Chem Res
, vol.39
, pp. 568-575
-
-
Eisenberg, D.1
Nelson, R.2
Sawaya, M.R.3
Balbirnie, M.4
Sambashivan, S.5
Ivanova, M.I.6
Madsen, A.O.7
Riekel, C.8
-
41
-
-
33746099650
-
Protein aggregation in crowded environments
-
Ellis RJ, Minton AP. Protein aggregation in crowded environments. Biol Chem 2006;387:485-497.
-
(2006)
Biol Chem
, vol.387
, pp. 485-497
-
-
Ellis, R.J.1
Minton, A.P.2
-
42
-
-
42449151176
-
Protein folding and misfolding: mechanism and principles
-
Englander SW, Mayne L, Krishna MM. Protein folding and misfolding: mechanism and principles. Q Rev Biophys 2007;40:287-326.
-
(2007)
Q Rev Biophys
, vol.40
, pp. 287-326
-
-
Englander, S.W.1
Mayne, L.2
Krishna, M.M.3
-
43
-
-
33745041480
-
Evolutionary relationships and structural mechanisms of AAA+ proteins
-
Erzberger JP, Berger JM. Evolutionary relationships and structural mechanisms of AAA+ proteins. Annu Rev Biophys Biomol Struct 2006;35:93-114.
-
(2006)
Annu Rev Biophys Biomol Struct
, vol.35
, pp. 93-114
-
-
Erzberger, J.P.1
Berger, J.M.2
-
44
-
-
37349011012
-
Modulation of metabolic brain networks after subthalamic gene therapy for Parkinson's disease
-
Feigin A, Kaplitt MG, Tang C, Lin T, Mattis P, Dhawan V, During MJ, Eidelberg D. Modulation of metabolic brain networks after subthalamic gene therapy for Parkinson's disease. Proc Natl Acad Sci USA 2007;104:19559-19564.
-
(2007)
Proc Natl Acad Sci USA
, vol.104
, pp. 19559-19564
-
-
Feigin, A.1
Kaplitt, M.G.2
Tang, C.3
Lin, T.4
Mattis, P.5
Dhawan, V.6
During, M.J.7
Eidelberg, D.8
-
45
-
-
7044238416
-
Neurodegenerative diseases: a decade of discoveries paves the way for therapeutic breakthroughs
-
Forman MS, Trojanowski JQ, Lee VM. Neurodegenerative diseases: a decade of discoveries paves the way for therapeutic breakthroughs. Nat Med 2004;10: 1055-1063.
-
(2004)
Nat Med
, vol.10
, pp. 1055-1063
-
-
Forman, M.S.1
Trojanowski, J.Q.2
Lee, V.M.3
-
46
-
-
31144460030
-
Functional amyloid formation within mammalian tissue
-
Fowler DM, Koulov AV, Alory-Jost C, Marks MS, Balch WE, Kelly JW. Functional amyloid formation within mammalian tissue. PLoS Biol 2006;4:e6.
-
(2006)
PLoS Biol
, vol.4
-
-
Fowler, D.M.1
Koulov, A.V.2
Alory-Jost, C.3
Marks, M.S.4
Balch, W.E.5
Kelly, J.W.6
-
48
-
-
7444221875
-
Harnessing chaperones to generate small-molecule inhibitors of amyloid beta aggregation
-
Gestwicki JE, Crabtree GR, Graef IA. Harnessing chaperones to generate small-molecule inhibitors of amyloid beta aggregation. Science 2004;306:865-869.
-
(2004)
Science
, vol.306
, pp. 865-869
-
-
Gestwicki, J.E.1
Crabtree, G.R.2
Graef, I.A.3
-
49
-
-
38349160161
-
The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis
-
Gitler AD, Bevis BJ, Shorter J, Strathearn KE, Hamamichi S, Su LJ, Caldwell KA, Caldwell GA, Rochet JC, McCaffery JM, et al. The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis. Proc Natl Acad Sci USA 2008;105:145-150.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 145-150
-
-
Gitler, A.D.1
Bevis, B.J.2
Shorter, J.3
Strathearn, K.E.4
Hamamichi, S.5
Su, L.J.6
Caldwell, K.A.7
Caldwell, G.A.8
Rochet, J.C.9
McCaffery, J.M.10
-
50
-
-
33847773421
-
Prime time for alpha-synuclein
-
Gitler AD, Shorter J. Prime time for alpha-synuclein. J Neurosci 2007;27:2433-2434.
-
(2007)
J Neurosci
, vol.27
, pp. 2433-2434
-
-
Gitler, A.D.1
Shorter, J.2
-
51
-
-
0021256895
-
Alzheimer's disease: initial report of the purification and characterization of a novel cerebrovascular amyloid protein
-
Glenner GG, Wong CW. Alzheimer's disease: initial report of the purification and characterization of a novel cerebrovascular amyloid protein. Biochem Biophys Res Commun 1984;120:885-890.
-
(1984)
Biochem Biophys Res Commun
, vol.120
, pp. 885-890
-
-
Glenner, G.G.1
Wong, C.W.2
-
52
-
-
0032503968
-
Hsp104, Hsp70, and Hsp40: a novel chaperone system that rescues previously aggregated proteins
-
Glover JR, Lindquist S. Hsp104, Hsp70, and Hsp40: a novel chaperone system that rescues previously aggregated proteins. Cell 1998;94:73-82.
-
(1998)
Cell
, vol.94
, pp. 73-82
-
-
Glover, J.R.1
Lindquist, S.2
-
53
-
-
67650162529
-
Remodeling of protein aggregates by Hsp104
-
Glover JR, Lum R. Remodeling of protein aggregates by Hsp104. Protein Pept Lett 2009;16:587-597.
-
(2009)
Protein Pept Lett
, vol.16
, pp. 587-597
-
-
Glover, J.R.1
Lum, R.2
-
54
-
-
0033598703
-
Sequential mechanism of solubilization and refolding of stable protein aggregates by a bichaperone network
-
Goloubinoff P, Mogk A, Zvi AP, Tomoyasu T, Bukau B. Sequential mechanism of solubilization and refolding of stable protein aggregates by a bichaperone network. Proc Natl Acad Sci USA 1999;96:13732-13737.
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 13732-13737
-
-
Goloubinoff, P.1
Mogk, A.2
Zvi, A.P.3
Tomoyasu, T.4
Bukau, B.5
-
55
-
-
14844335694
-
The E46K mutation in alpha-synuclein increases amyloid fibril formation
-
Greenbaum EA, Graves CL, Mishizen-Eberz AJ, Lupoli MA, Lynch DR, Englander SW, Axelsen PH, Giasson BI. The E46K mutation in alpha-synuclein increases amyloid fibril formation. J Biol Chem 2005;280:7800-7807.
-
(2005)
J Biol Chem
, vol.280
, pp. 7800-7807
-
-
Greenbaum, E.A.1
Graves, C.L.2
Mishizen-Eberz, A.J.3
Lupoli, M.A.4
Lynch, D.R.5
Englander, S.W.6
Axelsen, P.H.7
Giasson, B.I.8
-
56
-
-
67651230677
-
Complex adaptations can drive the evolution of the capacitor [PSI +], even with realistic rates of yeast sex
-
Griswold CK, Masel J. Complex adaptations can drive the evolution of the capacitor [PSI +], even with realistic rates of yeast sex. PLoS Genet 2009;5:e1000517.
-
(2009)
PLoS Genet
, vol.5
-
-
Griswold, C.K.1
Masel, J.2
-
57
-
-
0024563160
-
Amyloid protein and neurofibrillary tangles coexist in the same neuron in Alzheimer disease
-
Grundke-Iqbal I, Iqbal K, George L, Tung YC, Kim KS, Wisniewski HM. Amyloid protein and neurofibrillary tangles coexist in the same neuron in Alzheimer disease. Proc Natl Acad Sci USA 1989;86:2853-2857.
-
(1989)
Proc Natl Acad Sci USA
, vol.86
, pp. 2853-2857
-
-
Grundke-Iqbal, I.1
Iqbal, K.2
George, L.3
Tung, Y.C.4
Kim, K.S.5
Wisniewski, H.M.6
-
58
-
-
33847662852
-
Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide
-
Haass C, Selkoe DJ. Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide. Nat Rev Mol Cell Biol 2007;8:101-112.
-
(2007)
Nat Rev Mol Cell Biol
, vol.8
, pp. 101-112
-
-
Haass, C.1
Selkoe, D.J.2
-
59
-
-
0037129435
-
Sustained correction of X-linked severe combined immunodeficiency by ex vivo gene therapy
-
Hacein-Bey-Abina S, Le Deist F, Carlier F, Bouneaud C, Hue C, De Villartay JP, Thrasher AJ, Wulffraat N, Sorensen R, Dupuis-Girod S, et al. Sustained correction of X-linked severe combined immunodeficiency by ex vivo gene therapy. N Engl J Med 2002;346:1185-1193.
-
(2002)
N Engl J Med
, vol.346
, pp. 1185-1193
-
-
Hacein-Bey-Abina, S.1
Le Deist, F.2
Carlier, F.3
Bouneaud, C.4
Hue, C.5
De Villartay, J.P.6
Thrasher, A.J.7
Wulffraat, N.8
Sorensen, R.9
Dupuis-Girod, S.10
-
60
-
-
77249122579
-
Prions, protein homeostasis, and phenotypic diversity
-
Halfmann R, Alberti S, Lindquist S. Prions, protein homeostasis, and phenotypic diversity. Trends Cell Biol 2010;20:125-133.
-
(2010)
Trends Cell Biol
, vol.20
, pp. 125-133
-
-
Halfmann, R.1
Alberti, S.2
Lindquist, S.3
-
61
-
-
44849138934
-
Protein disaggregation by the AAA+ chaperone ClpB involves partial threading of looped polypeptide segments
-
Haslberger T, Zdanowicz A, Brand I, Kirstein J, Turgay K, Mogk A, Bukau B. Protein disaggregation by the AAA+ chaperone ClpB involves partial threading of looped polypeptide segments. Nat Struct Mol Biol 2008;15:641-650.
-
(2008)
Nat Struct Mol Biol
, vol.15
, pp. 641-650
-
-
Haslberger, T.1
Zdanowicz, A.2
Brand, I.3
Kirstein, J.4
Turgay, K.5
Mogk, A.6
Bukau, B.7
-
62
-
-
0037080611
-
Cooperative kinetics of both Hsp104 ATPase domains and interdomain communication revealed by AAA sensor-1 mutants
-
Hattendorf DA, Lindquist SL. Cooperative kinetics of both Hsp104 ATPase domains and interdomain communication revealed by AAA sensor-1 mutants. EMBO J 2002;21:12-21.
-
(2002)
EMBO J
, vol.21
, pp. 12-21
-
-
Hattendorf, D.A.1
Lindquist, S.L.2
-
63
-
-
55949105643
-
Specificity of the J-protein Sis1 in the propagation of 3 yeast prions
-
Higurashi T, Hines JK, Sahi C, Aron R, Craig EA. Specificity of the J-protein Sis1 in the propagation of 3 yeast prions. Proc Natl Acad Sci USA 2008;105:16596-16601.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 16596-16601
-
-
Higurashi, T.1
Hines, J.K.2
Sahi, C.3
Aron, R.4
Craig, E.A.5
-
64
-
-
76049127471
-
Coupling ATP utilization to protein remodeling by ClpB, a hexameric AAA+ protein
-
Hoskins JR, Doyle SM, Wickner S. Coupling ATP utilization to protein remodeling by ClpB, a hexameric AAA+ protein. Proc Natl Acad Sci USA 2009;106: 22233-22238.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 22233-22238
-
-
Hoskins, J.R.1
Doyle, S.M.2
Wickner, S.3
-
65
-
-
33745419772
-
N-terminal domain of yeast Hsp104 chaperone is dispensable for thermotolerance and prion propagation but necessary for curing prions by Hsp104 overexpression
-
Hung GC, Masison DC. N-terminal domain of yeast Hsp104 chaperone is dispensable for thermotolerance and prion propagation but necessary for curing prions by Hsp104 overexpression. Genetics 2006;173:611-620.
-
(2006)
Genetics
, vol.173
, pp. 611-620
-
-
Hung, G.C.1
Masison, D.C.2
-
66
-
-
0028169925
-
Visualization of A beta 42(43) and A beta 40 in senile plaques with end-specific A beta monoclonals: evidence that an initially deposited species is A beta 42(43)
-
Iwatsubo T, Odaka A, Suzuki N, Mizusawa H, Nukina N, Ihara Y. Visualization of A beta 42(43) and A beta 40 in senile plaques with end-specific A beta monoclonals: evidence that an initially deposited species is A beta 42(43). Neuron 1994; 13:45-53.
-
(1994)
Neuron
, vol.13
, pp. 45-53
-
-
Iwatsubo, T.1
Odaka, A.2
Suzuki, N.3
Mizusawa, H.4
Nukina, N.5
Ihara, Y.6
-
67
-
-
0033048453
-
The mechanism of islet amyloid polypeptide toxicity is membrane disruption by intermediate-sized toxic amyloid particles
-
Janson J, Ashley RH, Harrison D, McIntyre S, Butler PC. The mechanism of islet amyloid polypeptide toxicity is membrane disruption by intermediate-sized toxic amyloid particles. Diabetes 1999;48:491-498.
-
(1999)
Diabetes
, vol.48
, pp. 491-498
-
-
Janson, J.1
Ashley, R.H.2
Harrison, D.3
McIntyre, S.4
Butler, P.C.5
-
68
-
-
67749133873
-
TDP-43 is intrinsically aggregation-prone and ALS-linked mutations accelerate aggregation and increase toxicity
-
Johnson BS, Snead D, Lee JJ, McCaffery JM, Shorter J, Gitler AD. TDP-43 is intrinsically aggregation-prone and ALS-linked mutations accelerate aggregation and increase toxicity. J Biol Chem 2009;284:20329-20339.
-
(2009)
J Biol Chem
, vol.284
, pp. 20329-20339
-
-
Johnson, B.S.1
Snead, D.2
Lee, J.J.3
McCaffery, J.M.4
Shorter, J.5
Gitler, A.D.6
-
69
-
-
0032969276
-
Islet amyloid: a long-recognized but underappreciated pathological feature of type 2 diabetes
-
Kahn SE, Andrikopoulos S, Verchere CB. Islet amyloid: a long-recognized but underappreciated pathological feature of type 2 diabetes. Diabetes 1999;48:241-253.
-
(1999)
Diabetes
, vol.48
, pp. 241-253
-
-
Kahn, S.E.1
Andrikopoulos, S.2
Verchere, C.B.3
-
70
-
-
34250683023
-
Safety and tolerability of gene therapy with an adenoassociated virus (AAV) borne GAD gene for Parkinson's disease: an open label, phase I trial
-
Kaplitt MG, Feigin A, Tang C, Fitzsimons HL, Mattis P, Lawlor PA, Bland RJ, Young D, Strybing K, Eidelberg D, et al. Safety and tolerability of gene therapy with an adenoassociated virus (AAV) borne GAD gene for Parkinson's disease: an open label, phase I trial. Lancet 2007;369:2097-2105.
-
(2007)
Lancet
, vol.369
, pp. 2097-2105
-
-
Kaplitt, M.G.1
Feigin, A.2
Tang, C.3
Fitzsimons, H.L.4
Mattis, P.5
Lawlor, P.A.6
Bland, R.J.7
Young, D.8
Strybing, K.9
Eidelberg, D.10
-
71
-
-
0242668337
-
Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis
-
Kayed R, Head E, Thompson JL, McIntire TM, Milton SC, Cotman CW, Glabe CG. Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis. Science 2003;300:486-489.
-
(2003)
Science
, vol.300
, pp. 486-489
-
-
Kayed, R.1
Head, E.2
Thompson, J.L.3
McIntire, T.M.4
Milton, S.C.5
Cotman, C.W.6
Glabe, C.G.7
-
72
-
-
77953028624
-
Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form
-
Kerman A, Liu HN, Croul S, Bilbao J, Rogaeva E, Zinman L, Robertson J, Chakrabartty A. Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathol 2010;119:335-344.
-
(2010)
Acta Neuropathol
, vol.119
, pp. 335-344
-
-
Kerman, A.1
Liu, H.N.2
Croul, S.3
Bilbao, J.4
Rogaeva, E.5
Zinman, L.6
Robertson, J.7
Chakrabartty, A.8
-
73
-
-
13244254047
-
Amyloid in the cardiovascular system: a review
-
Kholova I, Niessen HW. Amyloid in the cardiovascular system: a review. J Clin Pathol 2005;58:125-133.
-
(2005)
J Clin Pathol
, vol.58
, pp. 125-133
-
-
Kholova, I.1
Niessen, H.W.2
-
74
-
-
35349007441
-
The evolution of bet-hedging adaptations to rare scenarios
-
King OD, Masel J. The evolution of bet-hedging adaptations to rare scenarios. Theor Popul Biol 2007;72:560-575.
-
(2007)
Theor Popul Biol
, vol.72
, pp. 560-575
-
-
King, O.D.1
Masel, J.2
-
75
-
-
37549063068
-
Role of intermolecular forces in defining material properties of protein nanofibrils
-
Knowles TP, Fitzpatrick AW, Meehan S, Mott HR, Vendruscolo M, Dobson CM, Welland ME. Role of intermolecular forces in defining material properties of protein nanofibrils. Science 2007;318:1900-1903.
-
(2007)
Science
, vol.318
, pp. 1900-1903
-
-
Knowles, T.P.1
Fitzpatrick, A.W.2
Meehan, S.3
Mott, H.R.4
Vendruscolo, M.5
Dobson, C.M.6
Welland, M.E.7
-
76
-
-
1842644947
-
Inherited prion diseases
-
Prusiner SB, editor. New York: Cold Spring Harbor Laboratory Press
-
Kong Q, Surewicz WK, Peterson RB, Zou W, Chen SG, Gambetti P. Inherited prion diseases. In: Prusiner SB, editor. Prion biology and diseases. New York: Cold Spring Harbor Laboratory Press; 2004. p 673-776.
-
(2004)
Prion biology and diseases
, pp. 673-776
-
-
Kong, Q.1
Surewicz, W.K.2
Peterson, R.B.3
Zou, W.4
Chen, S.G.5
Gambetti, P.6
-
77
-
-
66849109240
-
The ribosome as a platform for co-translational processing, folding and targeting of newly synthesized proteins
-
Kramer G, Boehringer D, Ban N, Bukau B. The ribosome as a platform for co-translational processing, folding and targeting of newly synthesized proteins. Nat Struct Mol Biol 2009;16:589-597.
-
(2009)
Nat Struct Mol Biol
, vol.16
, pp. 589-597
-
-
Kramer, G.1
Boehringer, D.2
Ban, N.3
Bukau, B.4
-
78
-
-
20444474976
-
Structural insights into a yeast prion illuminate nucleation and strain diversity
-
Krishnan R, Lindquist SL. Structural insights into a yeast prion illuminate nucleation and strain diversity. Nature 2005;435:765-772.
-
(2005)
Nature
, vol.435
, pp. 765-772
-
-
Krishnan, R.1
Lindquist, S.L.2
-
80
-
-
61349156118
-
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis
-
Kwiatkowski TJ Jr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T, et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 2009;323:1205-1208.
-
(2009)
Science
, vol.323
, pp. 1205-1208
-
-
Kwiatkowski Jr., T.J.1
Bosco, D.A.2
Leclerc, A.L.3
Tamrazian, E.4
Vanderburg, C.R.5
Russ, C.6
Davis, A.7
Gilchrist, J.8
Kasarskis, E.J.9
Munsat, T.10
-
81
-
-
37349039461
-
TDP-43 proteinopathies: neurodegenerative protein misfolding diseases without amyloidosis
-
Kwong LK, Uryu K, Trojanowski JQ, Lee VM. TDP-43 proteinopathies: neurodegenerative protein misfolding diseases without amyloidosis. Neurosignals 2008;16:41-51.
-
(2008)
Neurosignals
, vol.16
, pp. 41-51
-
-
Kwong, L.K.1
Uryu, K.2
Trojanowski, J.Q.3
Lee, V.M.4
-
82
-
-
34250819839
-
Intracellular amyloid-beta in Alzheimer's disease
-
LaFerla FM, Green KN, Oddo S. Intracellular amyloid-beta in Alzheimer's disease. Nat Rev Neurosci 2007;8:499-509.
-
(2007)
Nat Rev Neurosci
, vol.8
, pp. 499-509
-
-
LaFerla, F.M.1
Green, K.N.2
Oddo, S.3
-
83
-
-
0029110883
-
The chemistry of scrapie infection: implications of the 'ice 9' metaphor
-
Lansbury PT Jr, Caughey B. The chemistry of scrapie infection: implications of the 'ice 9' metaphor. Chem Biol 1995;2:1-5.
-
(1995)
Chem Biol
, vol.2
, pp. 1-5
-
-
Lansbury Jr., P.T.1
Caughey, B.2
-
84
-
-
33751113009
-
Alpha-synuclein overexpression in PC12 and chromaffin cells impairs catecholamine release by interfering with a late step in exocytosis
-
Larsen KE, Schmitz Y, Troyer MD, Mosharov E, Dietrich P, Quazi AZ, Savalle M, Nemani V, Chaudhry FA, Edwards RH, et al. Alpha-synuclein overexpression in PC12 and chromaffin cells impairs catecholamine release by interfering with a late step in exocytosis. J Neurosci 2006;26:11915-11922.
-
(2006)
J Neurosci
, vol.26
, pp. 11915-11922
-
-
Larsen, K.E.1
Schmitz, Y.2
Troyer, M.D.3
Mosharov, E.4
Dietrich, P.5
Quazi, A.Z.6
Savalle, M.7
Nemani, V.8
Chaudhry, F.A.9
Edwards, R.H.10
-
85
-
-
0037130174
-
Neurodegenerative disease: amyloid pores from pathogenic mutations
-
Lashuel HA, Hartley D, Petre BM, Walz T, Lansbury PT Jr. Neurodegenerative disease: amyloid pores from pathogenic mutations. Nature 2002;418:291.
-
(2002)
Nature
, vol.418
, pp. 291
-
-
Lashuel, H.A.1
Hartley, D.2
Petre, B.M.3
Walz, T.4
Lansbury Jr., P.T.5
-
86
-
-
33748432548
-
Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration
-
Lee JW, Beebe K, Nangle LA, Jang J, Longo-Guess CM, Cook SA, Davisson MT, Sundberg JP, Schimmel P, Ackerman SL. Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration. Nature 2006;443:50-55.
-
(2006)
Nature
, vol.443
, pp. 50-55
-
-
Lee, J.W.1
Beebe, K.2
Nangle, L.A.3
Jang, J.4
Longo-Guess, C.M.5
Cook, S.A.6
Davisson, M.T.7
Sundberg, J.P.8
Schimmel, P.9
Ackerman, S.L.10
-
87
-
-
33846188909
-
Visualizing the ATPase cycle in a protein disaggregating machine: structural basis for substrate binding by ClpB
-
Lee S, Choi JM, Tsai FT. Visualizing the ATPase cycle in a protein disaggregating machine: structural basis for substrate binding by ClpB. Mol Cell 2007;25:261-271.
-
(2007)
Mol Cell
, vol.25
, pp. 261-271
-
-
Lee, S.1
Choi, J.M.2
Tsai, F.T.3
-
88
-
-
0142227208
-
The structure of ClpB: a molecular chaperone that rescues proteins from an aggregated state
-
Lee S, Sowa ME, Watanabe YH, Sigler PB, Chiu W, Yoshida M, Tsai FT. The structure of ClpB: a molecular chaperone that rescues proteins from an aggregated state. Cell 2003;115:229-240.
-
(2003)
Cell
, vol.115
, pp. 229-240
-
-
Lee, S.1
Sowa, M.E.2
Watanabe, Y.H.3
Sigler, P.B.4
Chiu, W.5
Yoshida, M.6
Tsai, F.T.7
-
89
-
-
34447639732
-
Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes
-
Legname G, Nguyen HO, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB. Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci USA 2006;103:19105-19110.
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 19105-19110
-
-
Legname, G.1
Nguyen, H.O.2
Peretz, D.3
Cohen, F.E.4
DeArmond, S.J.5
Prusiner, S.B.6
-
90
-
-
33645038471
-
A specific amyloid-beta protein assembly in the brain impairs memory
-
Lesne S, Koh MT, Kotilinek L, Kayed R, Glabe CG, Yang A, Gallagher M, Ashe KH. A specific amyloid-beta protein assembly in the brain impairs memory. Nature 2006;440:352-357.
-
(2006)
Nature
, vol.440
, pp. 352-357
-
-
Lesne, S.1
Koh, M.T.2
Kotilinek, L.3
Kayed, R.4
Glabe, C.G.5
Yang, A.6
Gallagher, M.7
Ashe, K.H.8
-
91
-
-
0036679197
-
Alpha-Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease
-
Lo Bianco C, Ridet JL, Schneider BL, Deglon N, Aebischer P. alpha-Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease. Proc Natl Acad Sci USA 2002;99:10813-10818.
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 10813-10818
-
-
Lo Bianco, C.1
Ridet, J.L.2
Schneider, B.L.3
Deglon, N.4
Aebischer, P.5
-
92
-
-
10644281090
-
Lentiviral vector delivery of parkin prevents dopaminergic degeneration in an alpha-synuclein rat model of Parkinson's disease
-
Lo Bianco C, Schneider BL, Bauer M, Sajadi A, Brice A, Iwatsubo T, Aebischer P. Lentiviral vector delivery of parkin prevents dopaminergic degeneration in an alpha-synuclein rat model of Parkinson's disease. Proc Natl Acad Sci USA 2004;101:17510-17515.
-
(2004)
Proc Natl Acad Sci USA
, vol.101
, pp. 17510-17515
-
-
Lo Bianco, C.1
Schneider, B.L.2
Bauer, M.3
Sajadi, A.4
Brice, A.5
Iwatsubo, T.6
Aebischer, P.7
-
93
-
-
51349150684
-
Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease
-
Lo Bianco C, Shorter J, Regulier E, Lashuel H, Iwatsubo T, Lindquist S, Aebischer P. Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease. J Clin Invest 2008;118:3087-3097.
-
(2008)
J Clin Invest
, vol.118
, pp. 3087-3097
-
-
Lo Bianco, C.1
Shorter, J.2
Regulier, E.3
Lashuel, H.4
Iwatsubo, T.5
Lindquist, S.6
Aebischer, P.7
-
94
-
-
0028303844
-
Pancreatic islet cell toxicity of amylin associated with type-2 diabetes mellitus
-
Lorenzo A, Razzaboni B, Weir GC, Yankner BA. Pancreatic islet cell toxicity of amylin associated with type-2 diabetes mellitus. Nature 1994;368:756-760.
-
(1994)
Nature
, vol.368
, pp. 756-760
-
-
Lorenzo, A.1
Razzaboni, B.2
Weir, G.C.3
Yankner, B.A.4
-
96
-
-
3142657524
-
Evidence for an unfolding/threading mechanism for protein disaggregation by Saccharomyces cerevisiae Hsp104
-
Lum R, Tkach JM, Vierling E, Glover JR. Evidence for an unfolding/threading mechanism for protein disaggregation by Saccharomyces cerevisiae Hsp104. J Biol Chem 2004;279:29139-29146.
-
(2004)
J Biol Chem
, vol.279
, pp. 29139-29146
-
-
Lum, R.1
Tkach, J.M.2
Vierling, E.3
Glover, J.R.4
-
97
-
-
39649122369
-
The C-terminal extension of Saccharomyces cerevisiae Hsp104 plays a role in oligomer assembly
-
Mackay RG, Helsen CW, Tkach JM, Glover JR. The C-terminal extension of Saccharomyces cerevisiae Hsp104 plays a role in oligomer assembly. Biochemistry 2008;47:1918-1927.
-
(2008)
Biochemistry
, vol.47
, pp. 1918-1927
-
-
Mackay, R.G.1
Helsen, C.W.2
Tkach, J.M.3
Glover, J.R.4
-
98
-
-
44249085878
-
Safety and efficacy of gene transfer for Leber's congenital amaurosis
-
Maguire AM, Simonelli F, Pierce EA, Pugh EN Jr, Mingozzi F, Bennicelli J, Banfi S, Marshall KA, Testa F, Surace EM, et al. Safety and efficacy of gene transfer for Leber's congenital amaurosis. N Engl J Med 2008;358:2240-2248.
-
(2008)
N Engl J Med
, vol.358
, pp. 2240-2248
-
-
Maguire, A.M.1
Simonelli, F.2
Pierce, E.A.3
Pugh Jr., E.N.4
Mingozzi, F.5
Bennicelli, J.6
Banfi, S.7
Marshall, K.A.8
Testa, F.9
Surace, E.M.10
-
99
-
-
0019805122
-
The relation of islet amyloid to the clinical type of diabetes
-
Maloy AL, Longnecker DS, Greenberg ER. The relation of islet amyloid to the clinical type of diabetes. Hum Pathol 1981;12:917-922.
-
(1981)
Hum Pathol
, vol.12
, pp. 917-922
-
-
Maloy, A.L.1
Longnecker, D.S.2
Greenberg, E.R.3
-
100
-
-
38049056730
-
Lipids revert inert Abeta amyloid fibrils to neurotoxic protofibrils that affect learning in mice
-
Martins IC, Kuperstein I, Wilkinson H, Maes E, Vanbrabant M, Jonckheere W, Van Gelder P, Hartmann D, D'Hooge R, De Strooper B, et al. Lipids revert inert Abeta amyloid fibrils to neurotoxic protofibrils that affect learning in mice. EMBO J 2008;27:224-233.
-
(2008)
EMBO J
, vol.27
, pp. 224-233
-
-
Martins, I.C.1
Kuperstein, I.2
Wilkinson, H.3
Maes, E.4
Vanbrabant, M.5
Jonckheere, W.6
Van Gelder, P.7
Hartmann, D.8
D'Hooge, R.9
De Strooper, B.10
-
101
-
-
0012510759
-
Amyloid plaque core protein in Alzheimer disease and Down syndrome
-
Masters CL, Simms G,Weinman NA, Multhaup G, McDonald BL, Beyreuther K. Amyloid plaque core protein in Alzheimer disease and Down syndrome. Proc Natl Acad Sci USA 1985;82:4245-4249.
-
(1985)
Proc Natl Acad Sci USA
, vol.82
, pp. 4245-4249
-
-
Masters, C.L.1
Simms, G.2
Weinman, N.A.3
Multhaup, G.4
McDonald, B.L.5
Beyreuther, K.6
-
103
-
-
1242317785
-
The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapies
-
Merlini G, Westermark P. The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapies. J IntMed 2004;255:159-178.
-
(2004)
J IntMed
, vol.255
, pp. 159-178
-
-
Merlini, G.1
Westermark, P.2
-
104
-
-
38949123792
-
Rapid appearance and local toxicity of amyloid-beta plaques in a mouse model of Alzheimer's disease
-
Meyer-Luehmann M, Spires-Jones TL, Prada C, Garcia-Alloza M, de Calignon A, Rozkalne A, Koenigsknecht-Talboo J, Holtzman DM, Bacskai BJ, Hyman BT. Rapid appearance and local toxicity of amyloid-beta plaques in a mouse model of Alzheimer's disease. Nature 2008;451:720-724.
-
(2008)
Nature
, vol.451
, pp. 720-724
-
-
Meyer-Luehmann, M.1
Spires-Jones, T.L.2
Prada, C.3
Garcia-Alloza, M.4
de Calignon, A.5
Rozkalne, A.6
Koenigsknecht-Talboo, J.7
Holtzman, D.M.8
Bacskai, B.J.9
Hyman, B.T.10
-
105
-
-
0033573135
-
Identification of thermolabile Escherichia coli proteins: prevention and reversion of aggregation by DnaK and ClpB
-
Mogk A, Tomoyasu T, Goloubinoff P, Rudiger S, Roder D, Langen H, Bukau B. Identification of thermolabile Escherichia coli proteins: prevention and reversion of aggregation by DnaK and ClpB. EMBO J 1999;18:6934-6949.
-
(1999)
EMBO J
, vol.18
, pp. 6934-6949
-
-
Mogk, A.1
Tomoyasu, T.2
Goloubinoff, P.3
Rudiger, S.4
Roder, D.5
Langen, H.6
Bukau, B.7
-
107
-
-
33751237087
-
Stress, aging, and neurodegenerative disease
-
Morimoto RI. Stress, aging, and neurodegenerative disease. N Engl J Med 2006; 355:2254-2255.
-
(2006)
N Engl J Med
, vol.355
, pp. 2254-2255
-
-
Morimoto, R.I.1
-
108
-
-
3042561822
-
Saccharomyces cerevisiae Hsp104 enhances the chaperone capacity of human cells and inhibits heat stress-induced proapoptotic signaling
-
Mosser DD, Ho S, Glover JR. Saccharomyces cerevisiae Hsp104 enhances the chaperone capacity of human cells and inhibits heat stress-induced proapoptotic signaling. Biochemistry 2004;43:8107-8115.
-
(2004)
Biochemistry
, vol.43
, pp. 8107-8115
-
-
Mosser, D.D.1
Ho, S.2
Glover, J.R.3
-
109
-
-
36849093472
-
Semen-derived amyloid fibrils drastically enhance HIV infection
-
Munch J, Rucker E, Standker L, Adermann K, Goffinet C, Schindler M, Wildum S, Chinnadurai R, Rajan D, Specht A, et al. Semen-derived amyloid fibrils drastically enhance HIV infection. Cell 2007;131:1059-1071.
-
(2007)
Cell
, vol.131
, pp. 1059-1071
-
-
Munch, J.1
Rucker, E.2
Standker, L.3
Adermann, K.4
Goffinet, C.5
Schindler, M.6
Wildum, S.7
Chinnadurai, R.8
Rajan, D.9
Specht, A.10
-
110
-
-
77950239058
-
Discovery and characterization of a mammalian amyloid disaggregation activity
-
Murray AN, Solomon JP, Wang YJ, Balch WE, Kelly JW. Discovery and characterization of a mammalian amyloid disaggregation activity. Protein Sci 2010;19:836-846.
-
(2010)
Protein Sci
, vol.19
, pp. 836-846
-
-
Murray, A.N.1
Solomon, J.P.2
Wang, Y.J.3
Balch, W.E.4
Kelly, J.W.5
-
111
-
-
33646794418
-
Yeast prion-protein, sup35, fibril formation proceeds by addition and substraction of oligomers
-
Narayanan S, Walter S, Reif B. Yeast prion-protein, sup35, fibril formation proceeds by addition and substraction of oligomers. Chembiochem 2006;7:757-765.
-
(2006)
Chembiochem
, vol.7
, pp. 757-765
-
-
Narayanan, S.1
Walter, S.2
Reif, B.3
-
112
-
-
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, Tanaka M. Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity. Proc Natl Acad Sci USA 2009;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
-
113
-
-
33845652277
-
Structural models of amyloid-like fibrils
-
Nelson R, Eisenberg D. Structural models of amyloid-like fibrils. Adv Protein Chem 2006;73:235-282.
-
(2006)
Adv Protein Chem
, vol.73
, pp. 235-282
-
-
Nelson, R.1
Eisenberg, D.2
-
114
-
-
33749632259
-
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
-
Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006;314: 130-133.
-
(2006)
Science
, vol.314
, pp. 130-133
-
-
Neumann, M.1
Sampathu, D.M.2
Kwong, L.K.3
Truax, A.C.4
Micsenyi, M.C.5
Chou, T.T.6
Bruce, J.7
Schuck, T.8
Grossman, M.9
Clark, C.M.10
et, al..11
-
115
-
-
0032969563
-
AAA+: a class of chaperone-like ATPases associated with the assembly, operation, and disassembly of protein complexes
-
Neuwald AF, Aravind L, Spouge JL, Koonin EV. AAA+: a class of chaperone-like ATPases associated with the assembly, operation, and disassembly of protein complexes. Genome Res 1999;9:27-43.
-
(1999)
Genome Res
, vol.9
, pp. 27-43
-
-
Neuwald, A.F.1
Aravind, L.2
Spouge, J.L.3
Koonin, E.V.4
-
116
-
-
0027981247
-
Saccharomyces cerevisiae Hsp104 protein Purification and characterization of ATP-induced structural changes
-
Parsell DA, Kowal AS, Lindquist S. Saccharomyces cerevisiae Hsp104 protein. Purification and characterization of ATP-induced structural changes. J Biol Chem 1994a;269:4480-4487.
-
(1994)
J Biol Chem
, vol.269
, pp. 4480-4487
-
-
Parsell, D.A.1
Kowal, A.S.2
Lindquist, S.3
-
117
-
-
0027996115
-
Protein disaggregation mediated by heat-shock protein Hsp104
-
Parsell DA, Kowal AS, Singer MA, Lindquist S. Protein disaggregation mediated by heat-shock protein Hsp104. Nature 1994b;372:475-478.
-
(1994)
Nature
, vol.372
, pp. 475-478
-
-
Parsell, D.A.1
Kowal, A.S.2
Singer, M.A.3
Lindquist, S.4
-
118
-
-
0027135501
-
The function of heat-shock proteins in stress tolerance: degradation and reactivation of damaged proteins
-
Parsell DA, Lindquist S. The function of heat-shock proteins in stress tolerance: degradation and reactivation of damaged proteins. Annu Rev Genet 1993;27:437-496.
-
(1993)
Annu Rev Genet
, vol.27
, pp. 437-496
-
-
Parsell, D.A.1
Lindquist, S.2
-
119
-
-
0025777272
-
Hsp104 is a highly conserved protein with two essential nucleotide-binding sites
-
Parsell DA, Sanchez Y, Stitzel JD, Lindquist S. Hsp104 is a highly conserved protein with two essential nucleotide-binding sites. Nature 1991;353:270-273.
-
(1991)
Nature
, vol.353
, pp. 270-273
-
-
Parsell, D.A.1
Sanchez, Y.2
Stitzel, J.D.3
Lindquist, S.4
-
121
-
-
0029780647
-
Support for the prion hypothesis for inheritance of a phenotypic trait in yeast
-
Patino MM, Liu JJ, Glover JR, Lindquist S. Support for the prion hypothesis for inheritance of a phenotypic trait in yeast. Science 1996;273:622-626.
-
(1996)
Science
, vol.273
, pp. 622-626
-
-
Patino, M.M.1
Liu, J.J.2
Glover, J.R.3
Lindquist, S.4
-
122
-
-
0029888121
-
Propagation of the yeast prion-like [PSI +] determinant is mediated by oligomerization of the SUP35-encoded polypeptide chain release factor
-
Paushkin SV, Kushnirov VV, Smirnov VN, Ter-Avanesyan MD. Propagation of the yeast prion-like [PSI +] determinant is mediated by oligomerization of the SUP35-encoded polypeptide chain release factor. EMBO J 1996;15:3127-3134.
-
(1996)
EMBO J
, vol.15
, pp. 3127-3134
-
-
Paushkin, S.V.1
Kushnirov, V.V.2
Smirnov, V.N.3
Ter-Avanesyan, M.D.4
-
123
-
-
34247245632
-
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease
-
Perrin V, Regulier E, Abbas-Terki T, Hassig R, Brouillet E, Aebischer P, Luthi-Carter R, Deglon N. Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Mol Ther 2007;15:903-911.
-
(2007)
Mol Ther
, vol.15
, pp. 903-911
-
-
Perrin, V.1
Regulier, E.2
Abbas-Terki, T.3
Hassig, R.4
Brouillet, E.5
Aebischer, P.6
Luthi-Carter, R.7
Deglon, N.8
-
124
-
-
12244249201
-
Selfpropagating, molecular-level polymorphism in Alzheimer's beta-amyloid fibrils
-
Petkova AT, Leapman RD, Guo Z, Yau WM, Mattson MP, Tycko R. Selfpropagating, molecular-level polymorphism in Alzheimer's beta-amyloid fibrils. Science 2005;307:262-265.
-
(2005)
Science
, vol.307
, pp. 262-265
-
-
Petkova, A.T.1
Leapman, R.D.2
Guo, Z.3
Yau, W.M.4
Mattson, M.P.5
Tycko, R.6
-
125
-
-
67650410543
-
Biological and chemical approaches to diseases of proteostasis deficiency
-
Powers ET, Morimoto RI, Dillin A, Kelly JW, Balch WE. Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem 2009;78:959-991.
-
(2009)
Annu Rev Biochem
, vol.78
, pp. 959-991
-
-
Powers, E.T.1
Morimoto, R.I.2
Dillin, A.3
Kelly, J.W.4
Balch, W.E.5
-
126
-
-
77952771512
-
Alzheimer's disease international world alzheimer report
-
Prince M, Jackson J, Ferri CP, Sousa R, Albanese E, Ribeiro WS, Honyashiki M. Alzheimer's disease international world alzheimer report. In: International AsD (ed); 2009. p 1-96.
-
(2009)
International AsD
, pp. 1-96
-
-
Prince, M.1
Jackson, J.2
Ferri, C.P.3
Sousa, R.4
Albanese, E.5
Ribeiro, W.S.6
Honyashiki, M.7
-
127
-
-
0034119621
-
Heat shock protein 101 plays a crucial role in thermotolerance in Arabidopsis
-
Queitsch C, Hong SW, Vierling E, Lindquist S. Heat shock protein 101 plays a crucial role in thermotolerance in Arabidopsis. Plant Cell 2000;12:479-492.
-
(2000)
Plant Cell
, vol.12
, pp. 479-492
-
-
Queitsch, C.1
Hong, S.W.2
Vierling, E.3
Lindquist, S.4
-
128
-
-
33750731675
-
100 years and counting: prospects for defeating Alzheimer's disease
-
Roberson ED, Mucke L. 100 years and counting: prospects for defeating Alzheimer's disease. Science 2006;314:781-784.
-
(2006)
Science
, vol.314
, pp. 781-784
-
-
Roberson, E.D.1
Mucke, L.2
-
129
-
-
73549103148
-
A synergistic small-molecule combination directly eradicates diverse prion strain structures
-
Roberts BE, Duennwald ML, Wang H, Chung C, Lopreiato NP, Sweeny EA, Knight MN, Shorter J. A synergistic small-molecule combination directly eradicates diverse prion strain structures. Nat Chem Biol 2009;5:936-946.
-
(2009)
Nat Chem Biol
, vol.5
, pp. 936-946
-
-
Roberts, B.E.1
Duennwald, M.L.2
Wang, H.3
Chung, C.4
Lopreiato, N.P.5
Sweeny, E.A.6
Knight, M.N.7
Shorter, J.8
-
130
-
-
0031720905
-
Eight prion strains have PrP(Sc) molecules with different conformations
-
Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, Cohen FE, Prusiner SB. Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med 1998;4:1157-1165.
-
(1998)
Nat Med
, vol.4
, pp. 1157-1165
-
-
Safar, J.1
Wille, H.2
Itri, V.3
Groth, D.4
Serban, H.5
Torchia, M.6
Cohen, F.E.7
Prusiner, S.B.8
-
131
-
-
0025193343
-
HSP104 required for induced thermotolerance
-
Sanchez Y, Lindquist SL. HSP104 required for induced thermotolerance. Science 1990;248:1112-1115.
-
(1990)
Science
, vol.248
, pp. 1112-1115
-
-
Sanchez, Y.1
Lindquist, S.L.2
-
133
-
-
0034705224
-
Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans
-
Satyal SH, Schmidt E, Kitagawa K, Sondheimer N, Lindquist S, Kramer JM, Morimoto RI. Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proc Natl Acad Sci USA 2000;97:5750-5755.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 5750-5755
-
-
Satyal, S.H.1
Schmidt, E.2
Kitagawa, K.3
Sondheimer, N.4
Lindquist, S.5
Kramer, J.M.6
Morimoto, R.I.7
-
134
-
-
47049118460
-
Molecular chaperones and the assembly of the prion Ure2p in vitro
-
Savistchenko J, Krzewska J, Fay N, Melki R. Molecular chaperones and the assembly of the prion Ure2p in vitro. J Biol Chem 2008;283:15732-15739.
-
(2008)
J Biol Chem
, vol.283
, pp. 15732-15739
-
-
Savistchenko, J.1
Krzewska, J.2
Fay, N.3
Melki, R.4
-
135
-
-
34249944932
-
Processing of proteins by the molecular chaperone hsp104
-
Schaupp A, Marcinowski M, Grimminger V, Bosl B, Walter S. Processing of proteins by the molecular chaperone hsp104. J Mol Biol 2007;370:674-686.
-
(2007)
J Mol Biol
, vol.370
, pp. 674-686
-
-
Schaupp, A.1
Marcinowski, M.2
Grimminger, V.3
Bosl, B.4
Walter, S.5
-
136
-
-
0031441886
-
Interactions of the chaperone Hsp104 with yeast Sup35 and mammalian PrP
-
Schirmer EC, Lindquist S. Interactions of the chaperone Hsp104 with yeast Sup35 and mammalian PrP. Proc Natl Acad Sci USA 1997;94:13932-13937.
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 13932-13937
-
-
Schirmer, E.C.1
Lindquist, S.2
-
137
-
-
0032546911
-
The ATPase activity of Hsp104, effects of environmental conditions and mutations
-
Schirmer EC, Queitsch C, Kowal AS, Parsell DA, Lindquist S. The ATPase activity of Hsp104, effects of environmental conditions and mutations. J Biol Chem 1998;273:15546-15552.
-
(1998)
J Biol Chem
, vol.273
, pp. 15546-15552
-
-
Schirmer, E.C.1
Queitsch, C.2
Kowal, A.S.3
Parsell, D.A.4
Lindquist, S.5
-
138
-
-
0035970115
-
Subunit interactions influence the biochemical and biological properties of Hsp104
-
Schirmer EC, Ware DM, Queitsch C, Kowal AS, Lindquist SL. Subunit interactions influence the biochemical and biological properties of Hsp104. Proc Natl Acad Sci USA 2001;98:914-919.
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 914-919
-
-
Schirmer, E.C.1
Ware, D.M.2
Queitsch, C.3
Kowal, A.S.4
Lindquist, S.L.5
-
139
-
-
3042642040
-
Substrate recognition by the AAA+ chaperone ClpB
-
Schlieker C, Weibezahn J, Patzelt H, Tessarz P, Strub C, Zeth K, Erbse A, Schneider-Mergener J, Chin JW, Schultz PG, et al. Substrate recognition by the AAA+ chaperone ClpB. Nat Struct Mol Biol 2004;11:607-615.
-
(2004)
Nat Struct Mol Biol
, vol.11
, pp. 607-615
-
-
Schlieker, C.1
Weibezahn, J.2
Patzelt, H.3
Tessarz, P.4
Strub, C.5
Zeth, K.6
Erbse, A.7
Schneider-Mergener, J.8
Chin, J.W.9
Schultz, P.G.10
-
140
-
-
37349102454
-
Hsp104: a weapon to combat diverse neurodegenerative disorders
-
Shorter J. Hsp104: a weapon to combat diverse neurodegenerative disorders. Neurosignals 2008;16:63-74.
-
(2008)
Neurosignals
, vol.16
, pp. 63-74
-
-
Shorter, J.1
-
141
-
-
2942722444
-
Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers
-
Shorter J, Lindquist S. Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers. Science 2004;304:1793-1797.
-
(2004)
Science
, vol.304
, pp. 1793-1797
-
-
Shorter, J.1
Lindquist, S.2
-
143
-
-
19544363062
-
Prions as adaptive conduits of memory and inheritance
-
Shorter J, Lindquist S. Prions as adaptive conduits of memory and inheritance. Nat Rev Genet 2005b;6:435-450.
-
(2005)
Nat Rev Genet
, vol.6
, pp. 435-450
-
-
Shorter, J.1
Lindquist, S.2
-
144
-
-
33746405081
-
Destruction or potentiation of different prions catalyzed by similar Hsp104 remodeling activities
-
Shorter J, Lindquist S. Destruction or potentiation of different prions catalyzed by similar Hsp104 remodeling activities. Mol Cell 2006;23:425-438.
-
(2006)
Mol Cell
, vol.23
, pp. 425-438
-
-
Shorter, J.1
Lindquist, S.2
-
145
-
-
54349091742
-
Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions
-
Shorter J, Lindquist S. Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions. EMBO J 2008;27:2712-2724.
-
(2008)
EMBO J
, vol.27
, pp. 2712-2724
-
-
Shorter, J.1
Lindquist, S.2
-
146
-
-
75749134925
-
Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation
-
Si K, Choi YB, White-Grindley E, Majumdar A, Kandel ER. Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation. Cell 2010;140:421-435.
-
(2010)
Cell
, vol.140
, pp. 421-435
-
-
Si, K.1
Choi, Y.B.2
White-Grindley, E.3
Majumdar, A.4
Kandel, E.R.5
-
147
-
-
0348077417
-
A neuronal isoform of the Aplysia CPEB has prion-like properties
-
Si K, Lindquist S, Kandel ER. A neuronal isoform of the Aplysia CPEB has prion-like properties. Cell 2003;115:879-891.
-
(2003)
Cell
, vol.115
, pp. 879-891
-
-
Si, K.1
Lindquist, S.2
Kandel, E.R.3
-
148
-
-
33645728523
-
Neurodegenerative diseases: new concepts of pathogenesis and their therapeutic implications
-
Skovronsky DM, Lee VM-Y, Trojanowski JQ. Neurodegenerative diseases: new concepts of pathogenesis and their therapeutic implications. Annu Rev Pathol Mech Dis 2006;1:151-170.
-
(2006)
Annu Rev Pathol Mech Dis
, vol.1
, pp. 151-170
-
-
Skovronsky, D.M.1
Lee, V.M.-Y.2
Trojanowski, J.Q.3
-
149
-
-
33750480868
-
Characterization of the nanoscale properties of individual amyloid fibrils
-
Smith JF, Knowles TP, Dobson CM, Macphee CE, Welland ME. Characterization of the nanoscale properties of individual amyloid fibrils. Proc Natl Acad Sci USA 2006;103:15806-15811.
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 15806-15811
-
-
Smith, J.F.1
Knowles, T.P.2
Dobson, C.M.3
Macphee, C.E.4
Welland, M.E.5
-
150
-
-
0032584686
-
Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies
-
Spillantini MG, Crowther RA, Jakes R, Cairns NJ, Lantos PL, Goedert M. Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies. Neurosci Lett 1998;251:205-208.
-
(1998)
Neurosci Lett
, vol.251
, pp. 205-208
-
-
Spillantini, M.G.1
Crowther, R.A.2
Jakes, R.3
Cairns, N.J.4
Lantos, P.L.5
Goedert, M.6
-
151
-
-
0030882856
-
Alphasynuclein in Lewy bodies
-
Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M. Alphasynuclein in Lewy bodies. Nature 1997;388:839-840.
-
(1997)
Nature
, vol.388
, pp. 839-840
-
-
Spillantini, M.G.1
Schmidt, M.L.2
Lee, V.M.3
Trojanowski, J.Q.4
Jakes, R.5
Goedert, M.6
-
153
-
-
34250617472
-
Gene therapy for Parkinson's disease: early data
-
Stoessl AJ. Gene therapy for Parkinson's disease: early data. Lancet 2007;369: 2056-2058.
-
(2007)
Lancet
, vol.369
, pp. 2056-2058
-
-
Stoessl, A.J.1
-
154
-
-
0030801746
-
The structure of amyloid fibrils by electron microscopy and X-ray diffraction
-
Sunde M, Blake C. The structure of amyloid fibrils by electron microscopy and X-ray diffraction. Adv Protein Chem 1997;50:123-159.
-
(1997)
Adv Protein Chem
, vol.50
, pp. 123-159
-
-
Sunde, M.1
Blake, C.2
-
155
-
-
0031592945
-
Common core structure of amyloid fibrils by synchrotron X-ray diffraction
-
Sunde M, Serpell LC, Bartlam M, Fraser PE, Pepys MB, Blake CC. Common core structure of amyloid fibrils by synchrotron X-ray diffraction. J Mol Biol 1997;273: 729-739.
-
(1997)
J Mol Biol
, vol.273
, pp. 729-739
-
-
Sunde, M.1
Serpell, L.C.2
Bartlam, M.3
Fraser, P.E.4
Pepys, M.B.5
Blake, C.C.6
-
156
-
-
63049114323
-
Prion proteostasis: Hsp104 meets its supporting cast
-
Sweeny EA, Shorter J. Prion proteostasis: Hsp104 meets its supporting cast. Prion 2008;2:135-140.
-
(2008)
Prion
, vol.2
, pp. 135-140
-
-
Sweeny, E.A.1
Shorter, J.2
-
157
-
-
33746698975
-
The physical basis of how prion conformations determine strain phenotypes
-
Tanaka M, Collins SR, Toyama BH, Weissman JS. The physical basis of how prion conformations determine strain phenotypes. Nature 2006;442:585-589.
-
(2006)
Nature
, vol.442
, pp. 585-589
-
-
Tanaka, M.1
Collins, S.R.2
Toyama, B.H.3
Weissman, J.S.4
-
158
-
-
34250866206
-
A non-Q/N-rich prion domain of a foreign prion, [Het-s], can propagate as a prion in yeast
-
Taneja V, Maddelein ML, Talarek N, Saupe SJ, Liebman SW. A non-Q/N-rich prion domain of a foreign prion, [Het-s], can propagate as a prion in yeast. Mol Cell 2007;27:67-77.
-
(2007)
Mol Cell
, vol.27
, pp. 67-77
-
-
Taneja, V.1
Maddelein, M.L.2
Talarek, N.3
Saupe, S.J.4
Liebman, S.W.5
-
159
-
-
0037077040
-
Toxic proteins in neurodegenerative disease
-
Taylor JP, Hardy J, Fischbeck KH. Toxic proteins in neurodegenerative disease. Science 2002;296:1991-1995.
-
(2002)
Science
, vol.296
, pp. 1991-1995
-
-
Taylor, J.P.1
Hardy, J.2
Fischbeck, K.H.3
-
160
-
-
40649098449
-
Substrate threading through the central pore of the Hsp104 chaperone as a common mechanism for protein disaggregation and prion propagation
-
Tessarz P, Mogk A, Bukau B. Substrate threading through the central pore of the Hsp104 chaperone as a common mechanism for protein disaggregation and prion propagation. Mol Microbiol 2008;68:87-97.
-
(2008)
Mol Microbiol
, vol.68
, pp. 87-97
-
-
Tessarz, P.1
Mogk, A.2
Bukau, B.3
-
161
-
-
34249903623
-
Prion recognition elements govern nucleation, strain specificity and species barriers
-
Tessier PM, Lindquist S. Prion recognition elements govern nucleation, strain specificity and species barriers. Nature 2007;447:556-561.
-
(2007)
Nature
, vol.447
, pp. 556-561
-
-
Tessier, P.M.1
Lindquist, S.2
-
162
-
-
55949109442
-
In Vivo monitoring of the prion replication cycle reveals a critical role for Sis1 in delivering substrates to Hsp104
-
Tipton KA, Verges KJ, Weissman JS. In Vivo monitoring of the prion replication cycle reveals a critical role for Sis1 in delivering substrates to Hsp104. Mol Cell 2008;32:584-591.
-
(2008)
Mol Cell
, vol.32
, pp. 584-591
-
-
Tipton, K.A.1
Verges, K.J.2
Weissman, J.S.3
-
164
-
-
66749115386
-
Amyloid deposits: protection against toxic protein species?
-
Treusch S, Cyr DM, Lindquist S. Amyloid deposits: protection against toxic protein species? Cell Cycle 2009;8:1668-1674.
-
(2009)
Cell Cycle
, vol.8
, pp. 1668-1674
-
-
Treusch, S.1
Cyr, D.M.2
Lindquist, S.3
-
165
-
-
0034727077
-
A yeast prion provides a mechanism for genetic variation and phenotypic diversity
-
True HL, Lindquist SL. A yeast prion provides a mechanism for genetic variation and phenotypic diversity. Nature 2000;407:477-483.
-
(2000)
Nature
, vol.407
, pp. 477-483
-
-
True, H.L.1
Lindquist, S.L.2
-
167
-
-
27944499891
-
Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease
-
Vacher C, Garcia-Oroz L, Rubinsztein DC. Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease. Hum Mol Genet 2005;14:3425-3433.
-
(2005)
Hum Mol Genet
, vol.14
, pp. 3425-3433
-
-
Vacher, C.1
Garcia-Oroz, L.2
Rubinsztein, D.C.3
-
168
-
-
61349162349
-
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6
-
Vance C, Rogelj B, Hortobagyi T, De Vos KJ, Nishimura AL, Sreedharan J, Hu X, Smith B, Ruddy D, Wright P, et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 2009;323:1208-1211.
-
(2009)
Science
, vol.323
, pp. 1208-1211
-
-
Vance, C.1
Rogelj, B.2
Hortobagyi, T.3
De Vos, K.J.4
Nishimura, A.L.5
Sreedharan, J.6
Hu, X.7
Smith, B.8
Ruddy, D.9
Wright, P.10
-
169
-
-
20444404618
-
Regulated protein degradation
-
Varshavsky A. Regulated protein degradation. Trends Biochem Sci 2005;30:283-286.
-
(2005)
Trends Biochem Sci
, vol.30
, pp. 283-286
-
-
Varshavsky, A.1
-
170
-
-
56749176947
-
One step at a time: endoplasmic reticulum-associated degradation
-
Vembar SS, Brodsky JL. One step at a time: endoplasmic reticulum-associated degradation. Nat Rev Mol Cell Biol 2008;9:944-957.
-
(2008)
Nat Rev Mol Cell Biol
, vol.9
, pp. 944-957
-
-
Vembar, S.S.1
Brodsky, J.L.2
-
171
-
-
44449168261
-
Direct and selective elimination of specific prions and amyloids by 4,5-dianilinophthalimide and analogs
-
Wang H, Duennwald ML, Roberts BE, Rozeboom LM, Zhang YL, Steele AD, Krishnan R, Su LJ, Griffin D, Mukhopadhyay S, et al. Direct and selective elimination of specific prions and amyloids by 4,5-dianilinophthalimide and analogs. Proc Natl Acad Sci USA 2008;105:7159-7164.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 7159-7164
-
-
Wang, H.1
Duennwald, M.L.2
Roberts, B.E.3
Rozeboom, L.M.4
Zhang, Y.L.5
Steele, A.D.6
Krishnan, R.7
Su, L.J.8
Griffin, D.9
Mukhopadhyay, S.10
-
172
-
-
72149111129
-
N-terminal domains elicit formation of functional Pmel17 amyloid fibrils
-
Watt B, van Niel G, Fowler DM, Hurbain I, Luk KC, Stayrook SE, Lemmon MA, Raposo G, Shorter J, Kelly JW, et al. N-terminal domains elicit formation of functional Pmel17 amyloid fibrils. J Biol Chem 2009;284:35543-35555.
-
(2009)
J Biol Chem
, vol.284
, pp. 35543-35555
-
-
Watt, B.1
van Niel, G.2
Fowler, D.M.3
Hurbain, I.4
Luk, K.C.5
Stayrook, S.E.6
Lemmon, M.A.7
Raposo, G.8
Shorter, J.9
Kelly, J.W.10
-
173
-
-
0042858475
-
Characterization of a trap mutant of the AAA+ chaperone ClpB
-
Weibezahn J, Schlieker C, Bukau B, Mogk A. Characterization of a trap mutant of the AAA+ chaperone ClpB. J Biol Chem 2003;278:32608-32617.
-
(2003)
J Biol Chem
, vol.278
, pp. 32608-32617
-
-
Weibezahn, J.1
Schlieker, C.2
Bukau, B.3
Mogk, A.4
-
174
-
-
26844539619
-
Novel insights into the mechanism of chaperone-assisted protein disaggregation
-
Weibezahn J, Schlieker C, Tessarz P, Mogk A, Bukau B. Novel insights into the mechanism of chaperone-assisted protein disaggregation. Biol Chem 2005;386: 739-744.
-
(2005)
Biol Chem
, vol.386
, pp. 739-744
-
-
Weibezahn, J.1
Schlieker, C.2
Tessarz, P.3
Mogk, A.4
Bukau, B.5
-
175
-
-
8844251486
-
Thermotolerance requires refolding of aggregated proteins by substrate translocation through the central pore of ClpB
-
Weibezahn J, Tessarz P, Schlieker C, Zahn R, Maglica Z, Lee S, Zentgraf H, Weber-Ban EU, Dougan DA, Tsai FT, et al. Thermotolerance requires refolding of aggregated proteins by substrate translocation through the central pore of ClpB. Cell 2004;119:653-665.
-
(2004)
Cell
, vol.119
, pp. 653-665
-
-
Weibezahn, J.1
Tessarz, P.2
Schlieker, C.3
Zahn, R.4
Maglica, Z.5
Lee, S.6
Zentgraf, H.7
Weber-Ban, E.U.8
Dougan, D.A.9
Tsai, F.T.10
-
176
-
-
75349111482
-
Cryo-electron microscopy structures of Hsp100 proteins-crowbars in or out
-
Wendler P, Saibil HR. Cryo-electron microscopy structures of Hsp100 proteins-crowbars in or out. Biochem Cell Biol 2010;88:89-96.
-
(2010)
Biochem Cell Biol
, vol.88
, pp. 89-96
-
-
Wendler, P.1
Saibil, H.R.2
-
177
-
-
37449008520
-
Atypical AAA+ subunit packing creates an expanded cavity for disaggregation by the proteinremodeling factor Hsp104
-
Wendler P, Shorter J, Plisson C, Cashikar AG, Lindquist S, Saibil HR. Atypical AAA+ subunit packing creates an expanded cavity for disaggregation by the proteinremodeling factor Hsp104. Cell 2007;131:1366-1377.
-
(2007)
Cell
, vol.131
, pp. 1366-1377
-
-
Wendler, P.1
Shorter, J.2
Plisson, C.3
Cashikar, A.G.4
Lindquist, S.5
Saibil, H.R.6
-
178
-
-
63849263045
-
Motor mechanism for protein threading through Hsp104
-
Wendler P, Shorter J, Snead D, Plisson C, Clare DK, Lindquist S, Saibil HR. Motor mechanism for protein threading through Hsp104. Mol Cell 2009;34:81-92.
-
(2009)
Mol Cell
, vol.34
, pp. 81-92
-
-
Wendler, P.1
Shorter, J.2
Snead, D.3
Plisson, C.4
Clare, D.K.5
Lindquist, S.6
Saibil, H.R.7
-
179
-
-
0042508791
-
Neuropathologic changes in Alzheimer's disease
-
Wenk GL. Neuropathologic changes in Alzheimer's disease. J Clin Psychiatry 2003;64 Suppl 9:7-10.
-
(2003)
J Clin Psychiatry
, vol.64
, Issue.SUPPL 9
, pp. 7-10
-
-
Wenk, G.L.1
-
180
-
-
41149169488
-
Coupling and dynamics of subunits in the hexameric AAA+ chaperone ClpB
-
Werbeck ND, Schlee S, Reinstein J. Coupling and dynamics of subunits in the hexameric AAA+ chaperone ClpB. J Mol Biol 2008;378:178-190.
-
(2008)
J Mol Biol
, vol.378
, pp. 178-190
-
-
Werbeck, N.D.1
Schlee, S.2
Reinstein, J.3
-
181
-
-
0027361386
-
Human neurons derived from a teratocarcinoma cell line express solely the 695-amino acid amyloid precursor protein and produce intracellular beta-amyloid or A4 peptides
-
Wertkin AM, Turner RS, Pleasure SJ, Golde TE, Younkin SG, Trojanowski JQ, Lee VM. Human neurons derived from a teratocarcinoma cell line express solely the 695-amino acid amyloid precursor protein and produce intracellular beta-amyloid or A4 peptides. Proc Natl Acad Sci USA 1993;90:9513-9517.
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 9513-9517
-
-
Wertkin, A.M.1
Turner, R.S.2
Pleasure, S.J.3
Golde, T.E.4
Younkin, S.G.5
Trojanowski, J.Q.6
Lee, V.M.7
-
182
-
-
69949187643
-
Molecular mechanisms for protein-encoded inheritance
-
Wiltzius JJ, Landau M, Nelson R, Sawaya MR, Apostol MI, Goldschmidt L, Soriaga AB, Cascio D, Rajashankar K, Eisenberg D. Molecular mechanisms for protein-encoded inheritance. Nat Struct Mol Biol 2009;16:973-978.
-
(2009)
Nat Struct Mol Biol
, vol.16
, pp. 973-978
-
-
Wiltzius, J.J.1
Landau, M.2
Nelson, R.3
Sawaya, M.R.4
Apostol, M.I.5
Goldschmidt, L.6
Soriaga, A.B.7
Cascio, D.8
Rajashankar, K.9
Eisenberg, D.10
-
183
-
-
71749089460
-
Fibril fragmentation enhances amyloid cytotoxicity
-
Xue WF, Hellewell AL, Gosal WS, Homans SW, Hewitt EW, Radford SE. Fibril fragmentation enhances amyloid cytotoxicity. J Biol Chem 2009;284:34272-34282.
-
(2009)
J Biol Chem
, vol.284
, pp. 34272-34282
-
-
Xue, W.F.1
Hellewell, A.L.2
Gosal, W.S.3
Homans, S.W.4
Hewitt, E.W.5
Radford, S.E.6
-
184
-
-
77949278013
-
Fibril fragmentation in amyloid assembly and cytotoxicity: when size matters
-
Xue WF, Hellewell AL, Hewitt EW, Radford SE. Fibril fragmentation in amyloid assembly and cytotoxicity: when size matters. Prion 2010;4:20-25.
-
(2010)
Prion
, vol.4
, pp. 20-25
-
-
Xue, W.F.1
Hellewell, A.L.2
Hewitt, E.W.3
Radford, S.E.4
|