-
1
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
Arrasate, M., Mitra, S., Schweitzer, E.S., Segal, M.R. Finkbeiner, S. (2004) Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature, 431, 805 810.
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
2
-
-
24744447434
-
Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3
-
Berke, S.J., Chai, Y., Marrs, G.L., Wen, H. Paulson, H.L. (2005) Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3. J. Biol. Chem., 280, 32026 32034.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 32026-32034
-
-
Berke, S.J.1
Chai, Y.2
Marrs, G.L.3
Wen, H.4
Paulson, H.L.5
-
3
-
-
0037059040
-
Chaperoning brain degeneration
-
Bonini, N.M. (2002) Chaperoning brain degeneration. Proc. Natl. Acad. Sci. USA, 99 (Suppl 4 16407 16411.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, Issue.SUPPL. 4
, pp. 16407-16411
-
-
Bonini, N.M.1
-
4
-
-
21244489544
-
HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells
-
Carra, S., Sivilotti, M., Chavez Zobel, A.T., Lambert, H. Landry, J. (2005) HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells. Hum. Mol. Genet., 14, 1659 1669.
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 1659-1669
-
-
Carra, S.1
Sivilotti, M.2
Chavez Zobel, A.T.3
Lambert, H.4
Landry, J.5
-
5
-
-
0033499931
-
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
-
Chai, Y., Koppenhafer, S.L., Bonini, N.M. Paulson, H.L. (1999) Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J. Neurosci., 19, 10338 10347.
-
(1999)
J. Neurosci.
, vol.19
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
6
-
-
0942287194
-
Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways
-
Chai, Y., Berke, S.S., Cohen, R.E. Paulson, H.L. (2004) Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. J. Biol. Chem., 279, 3605 3611.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 3605-3611
-
-
Chai, Y.1
Berke, S.S.2
Cohen, R.E.3
Paulson, H.L.4
-
7
-
-
0034703863
-
Mechanisms of chaperone suppression of polyglutamine disease: Selectivity, synergy and modulation of protein solubility in Drosophila
-
Chan, H.Y., Warrick, J.M., Gray-Board, G.L., Paulson, H.L. Bonini, N.M. (2000) Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Hum. Mol. Genet., 9, 2811 2820.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2811-2820
-
-
Chan, H.Y.1
Warrick, J.M.2
Gray-Board, G.L.3
Paulson, H.L.4
Bonini, N.M.5
-
8
-
-
24344496948
-
Dynamic expression of Hsp27 in the presence of mutant ataxin-3
-
Chang, W.H., Cemal, C.K., Hsu, Y.H., Kuo, C.L., Nukina, N., Chang, M.H., Hu, H.T., Li, C. Hsieh, M. (2005) Dynamic expression of Hsp27 in the presence of mutant ataxin-3. Biochem. Biophys. Res. Commun., 336, 258 267.
-
(2005)
Biochem. Biophys. Res. Commun.
, vol.336
, pp. 258-267
-
-
Chang, W.H.1
Cemal, C.K.2
Hsu, Y.H.3
Kuo, C.L.4
Nukina, N.5
Chang, M.H.6
Hu, H.T.7
Li, C.8
Hsieh, M.9
-
9
-
-
0036549767
-
Discordant protein and mRNA expression in lung adenocarcinomas
-
Chen, G., Gharib, T.G., Huang, C.C., Taylor, J.M., Misek, D.E., Kardia, S.L., Giordano, T.J., Iannettoni, M.D., Orringer, M.B., Hanash, S.M. Beer, D.G. (2002) Discordant protein and mRNA expression in lung adenocarcinomas. Mol. Cell Proteomics., 1, 304 313.
-
(2002)
Mol. Cell Proteomics.
, vol.1
, pp. 304-313
-
-
Chen, G.1
Gharib, T.G.2
Huang, C.C.3
Taylor, J.M.4
Misek, D.E.5
Kardia, S.L.6
Giordano, T.J.7
Iannettoni, M.D.8
Orringer, M.B.9
Hanash, S.M.10
Beer, D.G.11
-
11
-
-
0035394668
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
-
Cummings, C.J., Sun, Y., Opal, P., Antalffy, B., Mestril, R., Orr, H.T., Dillmann, W.H. Zoghbi, H.Y. (2001) Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet., 10, 1511 1518.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1511-1518
-
-
Cummings, C.J.1
Sun, Y.2
Opal, P.3
Antalffy, B.4
Mestril, R.5
Orr, H.T.6
Dillmann, W.H.7
Zoghbi, H.Y.8
-
12
-
-
0029391198
-
Gender equality in Machado-Joseph disease
-
DeStefano, A.L., Farrer, L.A., Maciel, P., Gaspar, C., Rouleau, G.A., Coutinho, P. Sequeiros, J. (1995) Gender equality in Machado-Joseph disease. Nat. Genet., 11, 118 119.
-
(1995)
Nat. Genet.
, vol.11
, pp. 118-119
-
-
Destefano, A.L.1
Farrer, L.A.2
MacIel, P.3
Gaspar, C.4
Rouleau, G.A.5
Coutinho, P.6
Sequeiros, J.7
-
13
-
-
0029391198
-
Gender equality in Machado-Joseph disease
-
Durr, A., Stevanin, G., Cancel, G., Abbas, N., Chneiweiss, H., Agid, Y., Feingold, J. Brice, A. (1995) Gender equality in Machado-Joseph disease. Nat. Genet., 11, 118 119.
-
(1995)
Nat. Genet.
, vol.11
, pp. 118-119
-
-
Durr, A.1
Stevanin, G.2
Cancel, G.3
Abbas, N.4
Chneiweiss, H.5
Agid, Y.6
Feingold, J.7
Brice, A.8
-
14
-
-
0141891166
-
Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3
-
Evert, B.O., Vogt, I.R., Vieira-Saecker, A.M., Ozimek, L., de Vos, R.A., Brunt, E.R., Klockgether, T. Wullner, U. (2003) Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3. J. Neuropathol. Exp. Neurol., 62, 1006 1018.
-
(2003)
J. Neuropathol. Exp. Neurol.
, vol.62
, pp. 1006-1018
-
-
Evert, B.O.1
Vogt, I.R.2
Vieira-Saecker, A.M.3
Ozimek, L.4
De Vos, R.A.5
Brunt, E.R.6
Klockgether, T.7
Wullner, U.8
-
15
-
-
0034626735
-
Oxidants, oxidative stress and the biology of ageing
-
Finkel, T. Holbrook, N.J. (2000) Oxidants, oxidative stress and the biology of ageing. Nature, 408, 239 247.
-
(2000)
Nature
, vol.408
, pp. 239-247
-
-
Finkel, T.1
Holbrook, N.J.2
-
16
-
-
5744248243
-
Comparison of pathways controlling toxicity in the eye and brain in Drosophila models of human neurodegenerative diseases
-
Ghosh, S. Feany, M.B. (2004) Comparison of pathways controlling toxicity in the eye and brain in Drosophila models of human neurodegenerative diseases. Hum. Mol. Genet., 13, 2011 2018.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 2011-2018
-
-
Ghosh, S.1
Feany, M.B.2
-
17
-
-
0141750470
-
Disruption of axonal transport by loss of huntingtin or expression of pathogenic poly-Q proteins in Drosophila
-
Gunawardena, S., Her, L.S., Brusch, R.G., Laymon, R.A., Niesman, I.R., Gordesky-Gold, B., Sintasath, L., Bonini, N.M. Goldstein, L.S. (2003) Disruption of axonal transport by loss of huntingtin or expression of pathogenic poly-Q proteins in Drosophila. Neuron, 40, 25 40.
-
(2003)
Neuron
, vol.40
, 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
-
18
-
-
0034329159
-
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
-
Gusella, J.F. MacDonald, M.E. (2000) Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat. Rev. Neurosci., 1, 109 115.
-
(2000)
Nat. Rev. Neurosci.
, vol.1
, pp. 109-115
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
19
-
-
0033016717
-
Correlation between protein and mRNA abundance in yeast
-
Gygi, S.P., Rochon, Y., Franza, B.R. Aebersold, R. (1999) Correlation between protein and mRNA abundance in yeast. Mol. Cell Biol., 19, 1720 1730.
-
(1999)
Mol. Cell Biol.
, vol.19
, pp. 1720-1730
-
-
Gygi, S.P.1
Rochon, Y.2
Franza, B.R.3
Aebersold, R.4
-
20
-
-
67649219195
-
Computational analysis of the human HSPH/HSPA/DNAJ family and cloning of a human HSPH/HSPA/DNAJ expression library
-
Hageman, J. Kampinga, H.H. (2009) Computational analysis of the human HSPH/HSPA/DNAJ family and cloning of a human HSPH/HSPA/DNAJ expression library. Cell Stress. Chaperones., 14, 1 21.
-
(2009)
Cell Stress. Chaperones.
, vol.14
, pp. 1-21
-
-
Hageman, J.1
Kampinga, H.H.2
-
21
-
-
75949094261
-
A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation
-
Hageman, J., Rujano, M.A., van Waarde, M.A., Kakkar, V., Dirks, R.P., Govorukhina, N., Oosterveld-Hut, H.M., Lubsen, N.H. Kampinga, H.H. (2010) A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation. Mol. Cell, 37, 355 369.
-
(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
-
22
-
-
0347928859
-
Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression
-
Hansson, O., Nylandsted, J., Castilho, R.F., Leist, M., Jaattela, M. Brundin, P. (2003) Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression. Brain Res., 970, 47 57.
-
(2003)
Brain Res.
, vol.970
, pp. 47-57
-
-
Hansson, O.1
Nylandsted, J.2
Castilho, R.F.3
Leist, M.4
Jaattela, M.5
Brundin, P.6
-
23
-
-
0029992278
-
Molecular chaperones in cellular protein folding
-
Hartl, F.U. (1996) Molecular chaperones in cellular protein folding. Nature, 381, 571 579.
-
(1996)
Nature
, vol.381
, pp. 571-579
-
-
Hartl, F.U.1
-
24
-
-
0034641589
-
Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
-
Jana, N.R., Tanaka, M., Wang, G. Nukina, N. (2000) Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity. Hum. Mol. Genet., 9, 2009 2018.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2009-2018
-
-
Jana, N.R.1
Tanaka, M.2
Wang, G.3
Nukina, N.4
-
25
-
-
64549097439
-
Guidelines for the nomenclature of the human heat shock proteins
-
Kampinga, H.H., Hageman, J., Vos, M.J., Kubota, H., Tanguay, R.M., Bruford, E.A., Cheetham, M.E., Chen, B. Hightower, L.E. (2009) Guidelines for the nomenclature of the human heat shock proteins. Cell Stress. Chaperones., 14, 105 111.
-
(2009)
Cell Stress. Chaperones.
, vol.14
, pp. 105-111
-
-
Kampinga, H.H.1
Hageman, J.2
Vos, M.J.3
Kubota, H.4
Tanguay, R.M.5
Bruford, E.A.6
Cheetham, M.E.7
Chen, B.8
Hightower, L.E.9
-
26
-
-
0029286517
-
Unique features of the CAG repeats in Machado-Joseph disease
-
Kawakami, H., Maruyama, H., Nakamura, S., Kawaguchi, Y., Kakizuka, A., Doyu, M. Sobue, G. (1995) Unique features of the CAG repeats in Machado-Joseph disease. Nat. Genet., 9, 344 345.
-
(1995)
Nat. Genet.
, vol.9
, pp. 344-345
-
-
Kawakami, H.1
Maruyama, H.2
Nakamura, S.3
Kawaguchi, Y.4
Kakizuka, A.5
Doyu, M.6
Sobue, G.7
-
27
-
-
0028024318
-
Homozygous inheritance of the Machado-Joseph disease gene
-
Lang, A.E., Rogaeva, E.A., Tsuda, T., Hutterer, J. St George-Hyslop, P. (1994) Homozygous inheritance of the Machado-Joseph disease gene. Ann. Neurol., 36, 443 447.
-
(1994)
Ann. Neurol.
, vol.36
, pp. 443-447
-
-
Lang, A.E.1
Rogaeva, E.A.2
Tsuda, T.3
Hutterer, J.4
St George-Hyslop, P.5
-
28
-
-
33847652900
-
Autophagy and neurodegeneration: When the cleaning crew goes on strike
-
Martinez-Vicente, M. Cuervo, A.M. (2007) Autophagy and neurodegeneration: when the cleaning crew goes on strike. Lancet Neurol., 6, 352 361.
-
(2007)
Lancet Neurol.
, vol.6
, pp. 352-361
-
-
Martinez-Vicente, M.1
Cuervo, A.M.2
-
29
-
-
0029947377
-
Reevaluation of the exact CAG repeat length in hereditary cerebellar ataxias using highly denaturing conditions and long PCR
-
Maruyama, H., Kawakami, H. Nakamura, S. (1996) Reevaluation of the exact CAG repeat length in hereditary cerebellar ataxias using highly denaturing conditions and long PCR. Hum. Genet., 97, 591 595.
-
(1996)
Hum. Genet.
, vol.97
, pp. 591-595
-
-
Maruyama, H.1
Kawakami, H.2
Nakamura, S.3
-
30
-
-
0032535245
-
Regulation of the heat shock transcriptional response: Cross talk between a family of heat shock factors, molecular chaperones, and negative regulators
-
Morimoto, R.I. (1998) Regulation of the heat shock transcriptional response: cross talk between a family of heat shock factors, molecular chaperones, and negative regulators. Genes Dev., 12, 3788 3796.
-
(1998)
Genes Dev.
, vol.12
, pp. 3788-3796
-
-
Morimoto, R.I.1
-
31
-
-
11144243412
-
Modulation of neurodegeneration by molecular chaperones
-
Muchowski, P.J. Wacker, J.L. (2005) Modulation of neurodegeneration by molecular chaperones. Nat. Rev. Neurosci., 6, 11 22.
-
(2005)
Nat. Rev. Neurosci.
, vol.6
, pp. 11-22
-
-
Muchowski, P.J.1
Wacker, J.L.2
-
32
-
-
0036247596
-
The role of chaperones in polyglutamine disease
-
Opal, P. Zoghbi, H.Y. (2002) The role of chaperones in polyglutamine disease. Trends Mol. Med., 8, 232 236.
-
(2002)
Trends Mol. Med.
, vol.8
, pp. 232-236
-
-
Opal, P.1
Zoghbi, H.Y.2
-
33
-
-
34547692622
-
Trinucleotide repeat disorders
-
Orr, H.T. Zoghbi, H.Y. (2007) Trinucleotide repeat disorders. Annu. Rev. Neurosci., 30, 575 621.
-
(2007)
Annu. Rev. Neurosci.
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
34
-
-
2342455798
-
Desmin aggregate formation by R120G alphaB-crystallin is caused by altered filament interactions and is dependent upon network status in cells
-
Perng, M.D., Wen, S.F., van den ijssel, P., Prescott, A.R. Quinlan, R.A. (2004) Desmin aggregate formation by R120G alphaB-crystallin is caused by altered filament interactions and is dependent upon network status in cells. Mol. Biol. Cell, 15, 2335 2346.
-
(2004)
Mol. Biol. Cell
, vol.15
, pp. 2335-2346
-
-
Perng, M.D.1
Wen, S.F.2
Van Den Ijssel, P.3
Prescott, A.R.4
Quinlan, R.A.5
-
35
-
-
34247245632
-
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease
-
Perrin, V., Regulier, E., bbas-Terki, T., Hassig, R., Brouillet, E., Aebischer, P., Luthi-Carter, R. Deglon, N. (2007) Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Mol. Ther., 15, 903 911.
-
(2007)
Mol. Ther.
, vol.15
, pp. 903-911
-
-
Perrin, V.1
Regulier, E.2
Bbas-Terki, T.3
Hassig, R.4
Brouillet, E.5
Aebischer, P.6
Luthi-Carter, R.7
Deglon, N.8
-
36
-
-
0036366525
-
Cytoskeletal competence requires protein chaperones
-
Quinlan, R. (2002) Cytoskeletal competence requires protein chaperones. Prog. Mol. Subcell. Biol., 28, 219 233.
-
(2002)
Prog. Mol. Subcell. Biol.
, vol.28
, pp. 219-233
-
-
Quinlan, R.1
-
37
-
-
0029134871
-
Spinocerebellar ataxia type 1 and Machado-Joseph disease: Incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia
-
Ranum, L.P., Lundgren, J.K., Schut, L.J., Ahrens, M.J., Perlman, S., Aita, J., Bird, T.D., Gomez, C. Orr, H.T. (1995) Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia. Am. J. Hum. Genet., 57, 603 608.
-
(1995)
Am. J. Hum. Genet.
, vol.57
, pp. 603-608
-
-
Ranum, L.P.1
Lundgren, J.K.2
Schut, L.J.3
Ahrens, M.J.4
Perlman, S.5
Aita, J.6
Bird, T.D.7
Gomez, C.8
Orr, H.T.9
-
38
-
-
0026541340
-
Alpha B-crystallin is present in reactive glia in Creutzfeldt-Jakob disease
-
Renkawek, K., de Jong, W.W., Merck, K.B., Frenken, C.W., van Workum, F.P. Bosman, G.J. (1992) alpha B-crystallin is present in reactive glia in Creutzfeldt-Jakob disease. Acta Neuropathol., 83, 324 327.
-
(1992)
Acta Neuropathol.
, vol.83
, pp. 324-327
-
-
Renkawek, K.1
De Jong, W.W.2
Merck, K.B.3
Frenken, C.W.4
Van Workum, F.P.5
Bosman, G.J.6
-
39
-
-
0006607248
-
Dementia, gliosis and expression of the small heat shock proteins hsp27 and alpha B-crystallin in Parkinson's disease
-
Renkawek, K., Stege, G.J. Bosman, G.J. (1999) Dementia, gliosis and expression of the small heat shock proteins hsp27 and alpha B-crystallin in Parkinson's disease. Neuroreport, 10, 2273 2276.
-
(1999)
Neuroreport
, vol.10
, pp. 2273-2276
-
-
Renkawek, K.1
Stege, G.J.2
Bosman, G.J.3
-
40
-
-
0031015378
-
Transmission distortion of the mutant alleles in spinocerebellar ataxia
-
Riess, O., Epplen, J.T., Amoiridis, G., Przuntek, H. Schols, L. (1997) Transmission distortion of the mutant alleles in spinocerebellar ataxia. Hum. Genet., 99, 282 284.
-
(1997)
Hum. Genet.
, vol.99
, pp. 282-284
-
-
Riess, O.1
Epplen, J.T.2
Amoiridis, G.3
Przuntek, H.4
Schols, L.5
-
41
-
-
65849514220
-
SCA3: Neurological features, pathogenesis and animal models
-
Riess, O., Rub, U., Pastore, A., Bauer, P. Schols, L. (2008) SCA3: Neurological features, pathogenesis and animal models. Cerebellum., 7, 125 137.
-
(2008)
Cerebellum.
, vol.7
, pp. 125-137
-
-
Riess, O.1
Rub, U.2
Pastore, A.3
Bauer, P.4
Schols, L.5
-
42
-
-
33746791455
-
Spinocerebellar ataxia type 3 (SCA3): Thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions
-
Rub, U., de Vos, R.A., Brunt, E.R., Sebesteny, T., Schols, L., Auburger, G., Bohl, J., Ghebremedhin, E., Gierga, K., Seidel, K., den, D.W., Heinsen, H., Paulson, H. Deller, T. (2006) Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathol., 16, 218 227.
-
(2006)
Brain Pathol.
, vol.16
, pp. 218-227
-
-
Rub, U.1
De Vos, R.A.2
Brunt, E.R.3
Sebesteny, T.4
Schols, L.5
Auburger, G.6
Bohl, J.7
Ghebremedhin, E.8
Gierga, K.9
Seidel, K.10
Den, D.W.11
Heinsen, H.12
Paulson, H.13
Deller, T.14
-
43
-
-
26444515364
-
Autophagy and its possible roles in nervous system diseases, damage and repair
-
Rubinsztein, D.C., DiFiglia, M., Heintz, N., Nixon, R.A., Qin, Z.H., Ravikumar, B., Stefanis, L. Tolkovsky, A. (2005) Autophagy and its possible roles in nervous system diseases, damage and repair. Autophagy., 1, 11 22.
-
(2005)
Autophagy.
, vol.1
, pp. 11-22
-
-
Rubinsztein, D.C.1
Difiglia, M.2
Heintz, N.3
Nixon, R.A.4
Qin, Z.H.5
Ravikumar, B.6
Stefanis, L.7
Tolkovsky, A.8
-
44
-
-
77956412376
-
The HSP70 chaperone machine as guardian of the proteome: Implications for protein folding diseases
-
In. Radons, J. Multhoff, G. Eds. Research Signpost. Kerala. pp
-
Rujano, M.A. Kampinga, H.H. 2008. The HSP70 chaperone machine as guardian of the proteome: Implications for protein folding diseases. In Radons, J. Multhoff, G. (Eds Heat Shock Proteins in Biology and Medicine. Research Signpost, Kerala, pp. 59 86.
-
(2008)
Heat Shock Proteins in Biology and Medicine.
, pp. 59-86
-
-
Rujano, M.A.1
Kampinga, H.H.2
-
45
-
-
34548608465
-
Modulation of polyglutamine inclusion formation by the Hsp70 chaperone machine
-
Rujano, M.A., Kampinga, H.H. Salomons, F.A. (2007) Modulation of polyglutamine inclusion formation by the Hsp70 chaperone machine. Exp. Cell Res., 313, 3568 3578.
-
(2007)
Exp. Cell Res.
, vol.313
, pp. 3568-3578
-
-
Rujano, M.A.1
Kampinga, H.H.2
Salomons, F.A.3
-
46
-
-
0028988941
-
Trinucleotide expansion within the MJD1 gene presents clinically as spinocerebellar ataxia and occurs most frequently in German SCA patients
-
Schols, L., Vieira-Saecker, A.M., Schols, S., Przuntek, H., Epplen, J.T. Riess, O. (1995) Trinucleotide expansion within the MJD1 gene presents clinically as spinocerebellar ataxia and occurs most frequently in German SCA patients. Hum. Mol. Genet., 4, 1001 1005.
-
(1995)
Hum. Mol. Genet.
, vol.4
, pp. 1001-1005
-
-
Schols, L.1
Vieira-Saecker, A.M.2
Schols, S.3
Przuntek, H.4
Epplen, J.T.5
Riess, O.6
-
47
-
-
11144356369
-
Autosomal dominant cerebellar ataxias: Clinical features, genetics, and pathogenesis
-
Schols, L., Bauer, P., Schmidt, T., Schulte, T. Riess, O. (2004) Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol., 3, 291 304.
-
(2004)
Lancet Neurol.
, vol.3
, pp. 291-304
-
-
Schols, L.1
Bauer, P.2
Schmidt, T.3
Schulte, T.4
Riess, O.5
-
48
-
-
40849140683
-
Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression
-
Sharp, P.S., Akbar, M.T., Bouri, S., Senda, A., Joshi, K., Chen, H.J., Latchman, D.S., Wells, D.J. de Belleroche, J. (2008) Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression. Neurobiol. Dis., 30, 42 55.
-
(2008)
Neurobiol. Dis.
, vol.30
, pp. 42-55
-
-
Sharp, P.S.1
Akbar, M.T.2
Bouri, S.3
Senda, A.4
Joshi, K.5
Chen, H.J.6
Latchman, D.S.7
Wells, D.J.8
De Belleroche, J.9
-
49
-
-
0027330972
-
Alpha B crystallin and HSP28 are enhanced in the cerebral cortex of patients with Alzheimer's disease
-
Shinohara, H., Inaguma, Y., Goto, S., Inagaki, T. Kato, K. (1993) Alpha B crystallin and HSP28 are enhanced in the cerebral cortex of patients with Alzheimer's disease. J. Neurol. Sci., 119, 203 208.
-
(1993)
J. Neurol. Sci.
, vol.119
, pp. 203-208
-
-
Shinohara, H.1
Inaguma, Y.2
Goto, S.3
Inagaki, T.4
Kato, K.5
-
50
-
-
0029009456
-
Evidence for inter-generational instability in the CAG repeat in the MJD1 gene and for conserved haplotypes at flanking markers amongst Japanese and Caucasian subjects with Machado-Joseph disease
-
Takiyama, Y., Igarashi, S., Rogaeva, E.A., Endo, K., Rogaev, E.I., Tanaka, H., Sherrington, R., Sanpei, K., Liang, Y., Saito, M., T.Tsuda, T., Takano, H., lkeda, M., Lin, C., Chi, H., Kennedy, J.L., Lang, A.E., Wherrett, J.R., Segawa, M., Nomura, Y., Yuasa, T., Weissenbach, J., Yoshida, M., Nishizawa, M., Kidd, K.K., Tsuji, S. St George-Hyslop, P.H. (1995) Evidence for inter-generational instability in the CAG repeat in the MJD1 gene and for conserved haplotypes at flanking markers amongst Japanese and Caucasian subjects with Machado-Joseph disease. Hum. Mol. Genet., 4, 1137 1146.
-
(1995)
Hum. Mol. Genet.
, vol.4
, pp. 1137-1146
-
-
Takiyama, Y.1
Igarashi, S.2
Rogaeva, E.A.3
Endo, K.4
Rogaev, E.I.5
Tanaka, H.6
Sherrington, R.7
Sanpei, K.8
Liang, Y.9
Saito, M.10
Tsuda, T.11
Takano, H.12
Lkeda, M.13
Lin, C.14
Chi, H.15
Kennedy, J.L.16
Lang, A.E.17
Wherrett, J.R.18
Segawa, M.19
Nomura, Y.20
Yuasa, T.21
Weissenbach, J.22
Yoshida, M.23
Nishizawa, M.24
Kidd, K.K.25
Tsuji, S.26
St George-Hyslop, P.H.27
more..
-
51
-
-
0036841876
-
Upregulation of HSP27 in a transgenic model of ALS
-
Vleminckx, V., Van, D.P., Goffin, K., Delye, H., Van Den, B.L. Robberecht, W. (2002) Upregulation of HSP27 in a transgenic model of ALS. J. Neuropathol. Exp. Neurol., 61, 968 974.
-
(2002)
J. Neuropathol. Exp. Neurol.
, vol.61
, pp. 968-974
-
-
Vleminckx, V.1
Van, D.P.2
Goffin, K.3
Delye, H.4
Van Den, B.L.5
Robberecht, W.6
-
52
-
-
67651092101
-
HSPB7 is a SC35 speckle resident small heat shock protein
-
Vos, M.J., Kanon, B. Kampinga, H.H. (2009) HSPB7 is a SC35 speckle resident small heat shock protein. Biochim.Biophys.Acta, 1793, 1343 1353.
-
(2009)
Biochim.Biophys.Acta
, vol.1793
, pp. 1343-1353
-
-
Vos, M.J.1
Kanon, B.2
Kampinga, H.H.3
-
53
-
-
0037066111
-
Spinocerebellar ataxias in the Netherlands: Prevalence and age at onset variance analysis
-
van de Warrenburg, B.P., Sinke, R.J., Verschuuren-Bemelmans, C.C., Scheffer, H., Brunt, E.R., Ippel, P.F., Maat-Kievit, J.A., Dooijes, D., Notermans, N.C., Lindhout, D., Knoers, N.V. Kremer, H.P. (2002) Spinocerebellar ataxias in the Netherlands: prevalence and age at onset variance analysis. Neurology, 58, 702 708.
-
(2002)
Neurology
, vol.58
, pp. 702-708
-
-
Van De Warrenburg, B.P.1
Sinke, R.J.2
Verschuuren-Bemelmans, C.C.3
Scheffer, H.4
Brunt, E.R.5
Ippel, P.F.6
Maat-Kievit, J.A.7
Dooijes, D.8
Notermans, N.C.9
Lindhout, D.10
Knoers, N.V.11
Kremer, H.P.12
-
54
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
-
Warrick, J.M., Chan, H.Y., Gray-Board, G.L., Chai, Y., Paulson, H.L. Bonini, N.M. (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet., 23, 425 428.
-
(1999)
Nat. Genet.
, vol.23
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
55
-
-
15944419824
-
Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism
-
Warrick, J.M., Morabito, L.M., Bilen, J., Gordesky-Gold, B., Faust, L.Z., Paulson, H.L. Bonini, N.M. (2005) Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol. Cell, 18, 37 48.
-
(2005)
Mol. Cell
, vol.18
, pp. 37-48
-
-
Warrick, J.M.1
Morabito, L.M.2
Bilen, J.3
Gordesky-Gold, B.4
Faust, L.Z.5
Paulson, H.L.6
Bonini, N.M.7
-
56
-
-
55549086868
-
The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains
-
Winborn, B.J., Travis, S.M., Todi, S.V., Scaglione, K.M., Xu, P., Williams, A.J., Cohen, R.E., Peng, J. Paulson, H.L. (2008) The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J. Biol. Chem., 283, 26436 26443.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 26436-26443
-
-
Winborn, B.J.1
Travis, S.M.2
Todi, S.V.3
Scaglione, K.M.4
Xu, P.5
Williams, A.J.6
Cohen, R.E.7
Peng, J.8
Paulson, H.L.9
-
57
-
-
0036566675
-
Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin
-
Wyttenbach, A., Sauvageot, O., Carmichael, J., az-Latoud, C., Arrigo, A.P. Rubinsztein, D.C. (2002) Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin. Hum. Mol. Genet., 11, 1137 1151.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 1137-1151
-
-
Wyttenbach, A.1
Sauvageot, O.2
Carmichael, J.3
Az-Latoud, C.4
Arrigo, A.P.5
Rubinsztein, D.C.6
-
58
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi, H.Y. Orr, H.T. (2000) Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci., 23, 217 247.
-
(2000)
Annu. Rev. Neurosci.
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
-
59
-
-
34447331291
-
Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: Chronic neurodegeneration does not induce Hsp27 activation
-
Zourlidou, A., Gidalevitz, T., Kristiansen, M., Landles, C., Woodman, B., Wells, D.J., Latchman, D.S., de, B.J., Tabrizi, S.J., Morimoto, R.I. Bates, G.P. (2007) Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation. Hum. Mol. Genet., 16, 1078 1090.
-
(2007)
Hum. Mol. Genet.
, vol.16
, pp. 1078-1090
-
-
Zourlidou, A.1
Gidalevitz, T.2
Kristiansen, M.3
Landles, C.4
Woodman, B.5
Wells, D.J.6
Latchman, D.S.7
De, B.J.8
Tabrizi, S.J.9
Morimoto, R.I.10
Bates, G.P.11
|