-
1
-
-
0033071176
-
Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold
-
Paulson H. Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold. Am J Hum Genet 1999; 64: 339-45
-
(1999)
Am J Hum Genet
, vol.64
, pp. 339-345
-
-
Paulson, H.1
-
2
-
-
0037264120
-
Unfolding the role of protein misfolding in neurodegenerative diseases
-
Soto C. Unfolding the role of protein misfolding in neurodegenerative diseases. Nat Rev Neurosci 2003; 4: 49-60
-
(2003)
Nat Rev Neurosci
, vol.4
, pp. 49-60
-
-
Soto, C.1
-
3
-
-
34547692622
-
Trinucleotide repeat disorders
-
Orr HT, Zoghbi HY. Trinucleotide repeat disorders. Annu Rev Neurosci 2007; 30: 575-621
-
(2007)
Annu Rev Neurosci
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
4
-
-
52049093169
-
Polyglutamine neurodegeneration: protein misfolding revisited
-
Williams AJ, Paulson HL. Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci 2008; 31: 512-18
-
(2008)
Trends Neurosci
, vol.31
, pp. 512-518
-
-
Williams, A.J.1
Paulson, H.L.2
-
5
-
-
11144356369
-
Autosomal dominant cerebellar ataxias: clinical features, genetics and pathogenesis
-
Schöls L, Bauer P, Schmidt T, Schulte T, Riess O. Autosomal dominant cerebellar ataxias: clinical features, genetics and pathogenesis. Neurology 2004; 3: 291-304
-
(2004)
Neurology
, vol.3
, pp. 291-304
-
-
Schöls, L.1
Bauer, P.2
Schmidt, T.3
Schulte, T.4
Riess, O.5
-
6
-
-
34447520352
-
Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence
-
Bichelmeier U, Schmidt T, Hübener J, Boy J, Rüttiger L, Häbig K, Poths S, Bonin M, Knipper M, Schmidt WJ, Wilbertz J, Wolburg H, Laccone F, Riess O. Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence. J Neurosci 2007; 27: 7418-28
-
(2007)
J Neurosci
, vol.27
, pp. 7418-7428
-
-
Bichelmeier, U.1
Schmidt, T.2
Hübener, J.3
Boy, J.4
Rüttiger, L.5
Häbig, K.6
Poths, S.7
Bonin, M.8
Knipper, M.9
Schmidt, W.J.10
Wilbertz, J.11
Wolburg, H.12
Laccone, F.13
Riess, O.14
-
7
-
-
33746791455
-
Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions
-
Rüb U, de Vos RA, Brunt ER, Sebestény T, Schöls L, Auburger G, Bohl J, Ghebremedhin E, Gierga K, Seidel K, den Dunnen W, Heinsen H, Paulson H, Deller T. Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathol 2006; 16: 218-27
-
(2006)
Brain Pathol
, vol.16
, pp. 218-227
-
-
Rüb, U.1
de Vos, R.A.2
Brunt, E.R.3
Sebestény, T.4
Schöls, L.5
Auburger, G.6
Bohl, J.7
Ghebremedhin, E.8
Gierga, K.9
Seidel, K.10
den Dunnen, W.11
Heinsen, H.12
Paulson, H.13
Deller, T.14
-
8
-
-
36949022900
-
CAG repeat disorder models and human neuropathology: similarities and differences
-
Yamada M, Sato T, Tsuji S, Takahashi H. CAG repeat disorder models and human neuropathology: similarities and differences. Acta Neuropathol 2008; 115: 71-86
-
(2008)
Acta Neuropathol
, vol.115
, pp. 71-86
-
-
Yamada, M.1
Sato, T.2
Tsuji, S.3
Takahashi, H.4
-
9
-
-
77956183748
-
Axonal inclusions in spinocerebellar ataxia type 3
-
Seidel K, den Dunnen WF, Schultz C, Paulson H, Frank S, de Vos RA, Brunt ER, Deller T, Kampinga HH, Rüb U. Axonal inclusions in spinocerebellar ataxia type 3. Acta Neuropathol 2010; 120: 449-60
-
(2010)
Acta Neuropathol
, vol.120
, pp. 449-460
-
-
Seidel, K.1
den Dunnen, W.F.2
Schultz, C.3
Paulson, H.4
Frank, S.5
de Vos, R.A.6
Brunt, E.R.7
Deller, T.8
Kampinga, H.H.9
Rüb, U.10
-
10
-
-
77954947810
-
The HSP70 chaperone machinery: J proteins as drivers of functional specifity
-
Kampinga HH, Craig EA. The HSP70 chaperone machinery: J proteins as drivers of functional specifity. Nat Rev Mol Cell Biol 2010; 11: 579-92
-
(2010)
Nat Rev Mol Cell Biol
, vol.11
, pp. 579-592
-
-
Kampinga, H.H.1
Craig, E.A.2
-
11
-
-
46849116411
-
Structural and functional diversities between members of the human HSPB, HSPH, HSPA and DNAJ Chaperone families
-
Vos MJ, Hageman J, Carra S, Kampinga HH. Structural and functional diversities between members of the human HSPB, HSPH, HSPA and DNAJ Chaperone families. Biochemistry 2008; 47: 7001-11
-
(2008)
Biochemistry
, vol.47
, pp. 7001-7011
-
-
Vos, M.J.1
Hageman, J.2
Carra, S.3
Kampinga, H.H.4
-
12
-
-
0032983061
-
In vivo chaperone activity of heat shock protein 70 and thermotolerance
-
Nollen EA, Brunsting JF, Roelofsen H, Weber LA, Kampinga HH. In vivo chaperone activity of heat shock protein 70 and thermotolerance. Mol Cell Biol 1999; 19: 2069-79
-
(1999)
Mol Cell Biol
, vol.19
, pp. 2069-2079
-
-
Nollen, E.A.1
Brunsting, J.F.2
Roelofsen, H.3
Weber, L.A.4
Kampinga, H.H.5
-
13
-
-
75949094261
-
A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation
-
Hageman J, Rujano MA, van Waarde MA, Kakkar V, Dirks RP, Govorukhina N, Oosterveld-Hut HM, Lubsen NH, Kampinga HH. A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation. Mol Cell 2010; 37: 355-69
-
(2010)
Mol Cell
, vol.37
, pp. 355-369
-
-
Hageman, J.1
Rujano, M.A.2
van Waarde, M.A.3
Kakkar, V.4
Dirks, R.P.5
Govorukhina, N.6
Oosterveld-Hut, H.M.7
Lubsen, N.H.8
Kampinga, H.H.9
-
14
-
-
0033499931
-
Analysis of heat shock protein (HSP) molecular chaperones in polyglutamine disease
-
Chai Y, Koppenhafer SL, Bonini NM, Paulson HL. Analysis of heat shock protein (HSP) molecular chaperones in polyglutamine disease. J Neurosci 1999; 19: 10338-47
-
(1999)
J Neurosci
, vol.19
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
15
-
-
77956407962
-
Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3
-
Zijlstra MP, Rujano MA, van Waarde MA, Vis E, Brunt ER, Kampinga HH. Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3. Eur J Neurosci 2010; 32: 760-70
-
(2010)
Eur J Neurosci
, vol.32
, pp. 760-770
-
-
Zijlstra, M.P.1
Rujano, M.A.2
van Waarde, M.A.3
Vis, E.4
Brunt, E.R.5
Kampinga, H.H.6
-
16
-
-
0034608868
-
HSP70 and HSP40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
-
Muchowski PJ, Schaffar G, Sittler A, Wanker EE, Hayer-Hartl MK, Hartl FU. HSP70 and HSP40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proc Natl Acad Sci U S A 2000; 97: 7841-6
-
(2000)
Proc Natl Acad Sci U S A
, vol.97
, pp. 7841-7846
-
-
Muchowski, P.J.1
Schaffar, G.2
Sittler, A.3
Wanker, E.E.4
Hayer-Hartl, M.K.5
Hartl, F.U.6
-
17
-
-
0036501074
-
Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in cellular model of spinal and bulbar muscular atrophy
-
Bailey CK, Andriola IF, Kampinga HH, Merry DE. Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in cellular model of spinal and bulbar muscular atrophy. Hum Mol Genet 2002; 11: 515-23
-
(2002)
Hum Mol Genet
, vol.11
, pp. 515-523
-
-
Bailey, C.K.1
Andriola, I.F.2
Kampinga, H.H.3
Merry, D.E.4
-
18
-
-
0032535483
-
The ubiquitin-proteasome pathway: on protein death and cell life
-
Ciechanover A. The ubiquitin-proteasome pathway: on protein death and cell life. EMBO J 1998; 17: 7151-60
-
(1998)
EMBO J
, vol.17
, pp. 7151-7160
-
-
Ciechanover, A.1
-
19
-
-
0242432394
-
Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease. Psych
-
Hayashi M, Kobayashi K, Furuta H. Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease. Psych. Clin Neurosci 2003; 57: 205-13
-
(2003)
Clin Neurosci
, vol.57
, pp. 205-213
-
-
Hayashi, M.1
Kobayashi, K.2
Furuta, H.3
-
20
-
-
38449083555
-
The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias
-
Suppl.
-
Davies JE, Sarkar S, Rubinsztein DC. The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias. BMC Biochem 2007; 8 (Suppl. 1): S2
-
(2007)
BMC Biochem
, vol.8
, Issue.1
-
-
Davies, J.E.1
Sarkar, S.2
Rubinsztein, D.C.3
-
21
-
-
27944504351
-
p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death
-
Bjorkoy G, Lamark T, Brech A, Outzen H, Perander M, Overvatn A, Stenmark H, Johansen T. p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death. J Cell Biol 2005; 171: 602-14
-
(2005)
J Cell Biol
, vol.171
, pp. 602-614
-
-
Bjorkoy, G.1
Lamark, T.2
Brech, A.3
Outzen, H.4
Perander, M.5
Overvatn, A.6
Stenmark, H.7
Johansen, T.8
-
22
-
-
33746805387
-
Signaling, polyubiquitination, trafficking, and inclusions: sequestosome 1/p62's role in neurodegenerative disease
-
Wooten MW, Hu X, Babu JR, Seibenhener ML, Geetha T, Paine MG, Wooten MC. Signaling, polyubiquitination, trafficking, and inclusions: sequestosome 1/p62's role in neurodegenerative disease. J Biomed Biotechnol 2006; 2006: 62079
-
(2006)
J Biomed Biotechnol
, vol.2006
, pp. 62079
-
-
Wooten, M.W.1
Hu, X.2
Babu, J.R.3
Seibenhener, M.L.4
Geetha, T.5
Paine, M.G.6
Wooten, M.C.7
-
23
-
-
34548259958
-
p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy
-
Pankiv A, Clausen TH, Lamark T, Brech A, Bruun JA, Outzen H, Overvatn A, Bjorkoy G, Johansen T. p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem 2007; 282: 24131-45
-
(2007)
J Biol Chem
, vol.282
, pp. 24131-24145
-
-
Pankiv, A.1
Clausen, T.H.2
Lamark, T.3
Brech, A.4
Bruun, J.A.5
Outzen, H.6
Overvatn, A.7
Bjorkoy, G.8
Johansen, T.9
-
24
-
-
33645926989
-
p62/SQSTM1: a missing link between protein aggregates and the autophagy machinery
-
Bjørkøy G, Lamark T, Johansen T. p62/SQSTM1: a missing link between protein aggregates and the autophagy machinery. Autophagy 2006; 2: 138-9
-
(2006)
Autophagy
, vol.2
, pp. 138-139
-
-
Bjørkøy, G.1
Lamark, T.2
Johansen, T.3
-
25
-
-
6844258835
-
Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients
-
Van Leeuwen FW, de Kleijn DP, van den Hurk HH, Neubauer A, Sonnemans MA, Sluijs JA, Köycü S, Ramdijelal RD, Salehi A, Martens GJ, Grosveld FG, Peter J, Burbach H, Hol EM. Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients. Science 1998; 279: 242-7
-
(1998)
Science
, vol.279
, pp. 242-247
-
-
Van Leeuwen, F.W.1
de Kleijn, D.P.2
van den Hurk, H.H.3
Neubauer, A.4
Sonnemans, M.A.5
Sluijs, J.A.6
Köycü, S.7
Ramdijelal, R.D.8
Salehi, A.9
Martens, G.J.10
Grosveld, F.G.11
Peter, J.12
Burbach, H.13
Hol, E.M.14
-
26
-
-
77955353515
-
Low levels of mutant ubiquitin are degraded by the proteasome in vivo
-
Van Tijn P, Verhage MC, Hobo B, van Leeuwen FW, Fischer DF. Low levels of mutant ubiquitin are degraded by the proteasome in vivo. J Neurosci Res 2010; 88: 2325-37
-
(2010)
J Neurosci Res
, vol.88
, pp. 2325-2337
-
-
Van Tijn, P.1
Verhage, M.C.2
Hobo, B.3
van Leeuwen, F.W.4
Fischer, D.F.5
-
27
-
-
0028972448
-
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias
-
Trottier Y, Lutz Y, Stevanin G, Imbert G, Devys S, Cancel G, Saudou F, Weber C, David G, Tora L, Agid Y, Brice A, Mandel JL. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 1995; 378: 403-6
-
(1995)
Nature
, vol.378
, pp. 403-406
-
-
Trottier, Y.1
Lutz, Y.2
Stevanin, G.3
Imbert, G.4
Devys, S.5
Cancel, G.6
Saudou, F.7
Weber, C.8
David, G.9
Tora, L.10
Agid, Y.11
Brice, A.12
Mandel, J.L.13
-
28
-
-
0242611535
-
Disease-specific accumulation of mutant ubiquitin as a marker for proteasomal dysfunction in the brain
-
Fischer DF, de Vos RA, van Dijk R, De Vrij FM, Proper EA, Sonnemans MA, Verhage MC, Sluijs JA, Hobo B, Zouambia M, Steur EN, Kamphorst W, Hol EM, Van Leeuwen FW. Disease-specific accumulation of mutant ubiquitin as a marker for proteasomal dysfunction in the brain. FASEB J 2003; 17: 2014-24
-
(2003)
FASEB J
, vol.17
, pp. 2014-2024
-
-
Fischer, D.F.1
de Vos, R.A.2
van Dijk, R.3
De Vrij, F.M.4
Proper, E.A.5
Sonnemans, M.A.6
Verhage, M.C.7
Sluijs, J.A.8
Hobo, B.9
Zouambia, M.10
Steur, E.N.11
Kamphorst, W.12
Hol, E.M.13
Van Leeuwen, F.W.14
-
29
-
-
75849128321
-
Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in a Huntington disease mouse model
-
de Pril R, Hobo B, van Tijn P, Roos RA, van Leeuwen FW, Fischer DF. Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in a Huntington disease mouse model. Mol Cell Neurosci 2010; 43: 281-6
-
(2010)
Mol Cell Neurosci
, vol.43
, pp. 281-286
-
-
de Pril, R.1
Hobo, B.2
van Tijn, P.3
Roos, R.A.4
van Leeuwen, F.W.5
Fischer, D.F.6
-
30
-
-
77956412376
-
The HSP70 chaperone machine as guardian of the proteome: implications for protein misfolding diseases
-
Eds J Radons, G Multhoff. Kerala: Research Signpost
-
Rujano MA, Kampinga HH. The HSP70 chaperone machine as guardian of the proteome: implications for protein misfolding diseases. In Heat Shock Proteins and Medicine. Eds J Radons, G Multhoff Kerala: Research Signpost, 2006; 59-85
-
(2006)
Heat Shock Proteins and Medicine
, pp. 59-85
-
-
Rujano, M.A.1
Kampinga, H.H.2
-
31
-
-
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: 805-10
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
32
-
-
77955647865
-
Identification of MOAG-4/SERF as a regulator of age-related proteotoxicity
-
van Ham TJ, Holmberg MA, van der Groot AT, Teuling E, Garcia-Arencibia M, Kim HE, Du D, Thijssen KL, Wiersma M, Burggraf R, van Bergeijk P, van Rheenen J, Jerre van Veluw G, Hofstra RM, Rubinsztein DC, Nollen EA. Identification of MOAG-4/SERF as a regulator of age-related proteotoxicity. Cell 2010; 142: 601-12
-
(2010)
Cell
, vol.142
, pp. 601-612
-
-
van Ham, T.J.1
Holmberg, M.A.2
van der Groot, A.T.3
Teuling, E.4
Garcia-Arencibia, M.5
Kim, H.E.6
Du, D.7
Thijssen, K.L.8
Wiersma, M.9
Burggraf, R.10
van Bergeijk, P.11
van Rheenen, J.12
Jerre van Veluw, G.13
Hofstra, R.M.14
Rubinsztein, D.C.15
Nollen, E.A.16
-
33
-
-
0032576605
-
Aggresomes: a cellular response to misfolded proteins
-
Johnston JA, Ward CL, Kopito RR. Aggresomes: a cellular response to misfolded proteins. J Cell Biol 1998; 143: 1883-98
-
(1998)
J Cell Biol
, vol.143
, pp. 1883-1898
-
-
Johnston, J.A.1
Ward, C.L.2
Kopito, R.R.3
-
34
-
-
77950903972
-
The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein Alfy
-
Filimonenko M, Isakson P, Finley KD, Anderson M, Jeong H, Melia TJ, Bartlett BJ, Myers KM, Birkeland HC, Lamark T, Krainc D, Brech A, Stenmark H, Simonsen A, Yamamoto A. The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein Alfy. Mol Cell 2010; 23: 265-79
-
(2010)
Mol Cell
, vol.23
, pp. 265-279
-
-
Filimonenko, M.1
Isakson, P.2
Finley, K.D.3
Anderson, M.4
Jeong, H.5
Melia, T.J.6
Bartlett, B.J.7
Myers, K.M.8
Birkeland, H.C.9
Lamark, T.10
Krainc, D.11
Brech, A.12
Stenmark, H.13
Simonsen, A.14
Yamamoto, A.15
-
35
-
-
63049132756
-
Acetylation targets mutant huntingtin to autophagosomes for degradation
-
Jeong H, Then F, Melia TH, Mazzulli JR, Cui L, Savas JN, Voisine C, Paganetti P, Tanese N, Hart AC, Yamamoto A, Krainc D. Acetylation targets mutant huntingtin to autophagosomes for degradation. Cell 2009; 137: 60-72
-
(2009)
Cell
, vol.137
, pp. 60-72
-
-
Jeong, H.1
Then, F.2
Melia, T.H.3
Mazzulli, J.R.4
Cui, L.5
Savas, J.N.6
Voisine, C.7
Paganetti, P.8
Tanese, N.9
Hart, A.C.10
Yamamoto, A.11
Krainc, D.12
-
36
-
-
0028829166
-
Thermostability of a nuclear-targeted luciferase expressed in mammalian cells. Destabilizing influence of the intranuclear microenvironment
-
Michels AA, Nguyen VT, Konings AW, Kampinga HH, Bensaude O. Thermostability of a nuclear-targeted luciferase expressed in mammalian cells. Destabilizing influence of the intranuclear microenvironment. Eur J Biochem 1995; 234: 382-9
-
(1995)
Eur J Biochem
, vol.234
, pp. 382-389
-
-
Michels, A.A.1
Nguyen, V.T.2
Konings, A.W.3
Kampinga, H.H.4
Bensaude, O.5
-
37
-
-
34547807613
-
Global changes to the ubiquitin system in Huntington's disease
-
Bennett EJ, Shaler TA, Woodman B, Ryu KY, Zaitseva TS, Becker CH, Bates GP, Schulman H, Kopito RR. Global changes to the ubiquitin system in Huntington's disease. Nature 2007; 448: 704-8
-
(2007)
Nature
, vol.448
, pp. 704-708
-
-
Bennett, E.J.1
Shaler, T.A.2
Woodman, B.3
Ryu, K.Y.4
Zaitseva, T.S.5
Becker, C.H.6
Bates, G.P.7
Schulman, H.8
Kopito, R.R.9
-
38
-
-
77955480316
-
Cellular and molecular pathways triggering neurodegeneration in the spinocerebellar ataxias
-
Matilla-Duenas A, Sánchez I, Corral-Juan M, Dávalos A, Alvarez R, Latorre P. Cellular and molecular pathways triggering neurodegeneration in the spinocerebellar ataxias. Cerebellum 2010; 9: 148-66
-
(2010)
Cerebellum
, vol.9
, pp. 148-166
-
-
Matilla-Duenas, A.1
Sánchez, I.2
Corral-Juan, M.3
Dávalos, A.4
Alvarez, R.5
Latorre, P.6
|