-
1
-
-
39349083915
-
Adapting proteostasis for disease intervention
-
Balch, W. E., Morimoto, R. I., Dillin, A. & Kelly, J. W. Adapting proteostasis for disease intervention. Science 319, 916-919 (2008).
-
(2008)
Science
, vol.319
, pp. 916-919
-
-
Balch, W.E.1
Morimoto, R.I.2
Dillin, A.3
Kelly, J.W.4
-
2
-
-
11444271010
-
Chaperone-assisted folding of newly synthesized proteins in the cytosol
-
Deuerling, E. & Bukau, B. Chaperone-assisted folding of newly synthesized proteins in the cytosol. Crit. Rev. Biochem. Mol. Biol. 39, 261-277 (2004).
-
(2004)
Crit. Rev. Biochem. Mol. Biol.
, vol.39
, pp. 261-277
-
-
Deuerling, E.1
Bukau, B.2
-
3
-
-
44349090822
-
Essential role of the chaperonin folding compartment in vivo
-
Tang, Y. C., Chang, H. C., Chakraborty, K., Hartl, F. U. & Hayer-Hartl, M. Essential role of the chaperonin folding compartment in vivo. EMBO J. 27, 1458-1468 (2008).
-
(2008)
EMBO J.
, vol.27
, pp. 1458-1468
-
-
Tang, Y.C.1
Chang, H.C.2
Chakraborty, K.3
Hartl, F.U.4
Hayer-Hartl, M.5
-
4
-
-
33646127577
-
Molecular chaperones and protein quality control
-
Bukau, B., Weissman, J. & Horwich, A. Molecular chaperones and protein quality control. Cell 125, 443-451 (2006).
-
(2006)
Cell.
, vol.125
, pp. 443-451
-
-
Bukau, B.1
Weissman, J.2
Horwich, A.3
-
5
-
-
64949099855
-
Protein homeostasis and aging: Taking care of proteins from the cradle to the grave
-
Morimoto, R. I. & Cuervo, A. M. Protein homeostasis and aging: taking care of proteins from the cradle to the grave. J. Gerontol. Ser. A Biol. Sci. Med. Sci. 64A, 167-170 (2009).
-
(2009)
J. Gerontol. Ser. A Biol. Sci. Med. Sci.
, vol.64
, pp. 167-170
-
-
Morimoto, R.I.1
Cuervo, A.M.2
-
6
-
-
60749101582
-
Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1
-
Westerheide, S. D., Anckar, J., Stevens, S. M., Sistonen, L. & Morimoto, R. I. Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1. Science 323, 1063-1066 (2009).
-
(2009)
Science
, vol.323
, pp. 1063-1066
-
-
Westerheide, S.D.1
Anckar, J.2
Stevens, S.M.3
Sistonen, L.4
Morimoto, R.I.5
-
7
-
-
34548658230
-
Heat shock factor 1 is a powerful multifaceted modifier of carcinogenesis
-
Dai, C., Whitesell, L., Rogers, A. B. & Lindquist, S. Heat shock factor 1 is a powerful multifaceted modifier of carcinogenesis. Cell 130, 1005-1018 (2007).
-
(2007)
Cell.
, vol.130
, pp. 1005-1018
-
-
Dai, C.1
Whitesell, L.2
Rogers, A.B.3
Lindquist, S.4
-
8
-
-
34250899722
-
Signal integration in the endoplasmic reticulum unfolded protein response
-
Ron, D. & Walter, P. Signal integration in the endoplasmic reticulum unfolded protein response. Nat. Rev. Mol. Cell Biol. 8, 519-529 (2007).
-
(2007)
Nat. Rev. Mol. Cell. Biol.
, vol.8
, pp. 519-529
-
-
Ron, D.1
Walter, P.2
-
9
-
-
22244446505
-
The mammalian unfolded protein response
-
Schroder, M. & Kaufman, R. J. The mammalian unfolded protein response. Annu. Rev. Biochem. 74, 739-789 (2005).
-
(2005)
Annu. Rev. Biochem.
, vol.74
, pp. 739-789
-
-
Schroder, M.1
Kaufman, R.J.2
-
10
-
-
33644850056
-
Progressive disruption of cellular protein folding in models of polyglutamine diseases
-
Gidalevitz, T., Ben-Zvi, A., Ho, K. H., Brignull, H. R. & Morimoto, R. I. Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science 311, 1471-1474 (2006).
-
(2006)
Science
, vol.311
, pp. 1471-1474
-
-
Gidalevitz, T.1
Ben-Zvi, A.2
Ho, K.H.3
Brignull, H.R.4
Morimoto, R.I.5
-
11
-
-
33750842131
-
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fbrosis
-
Wang, X. et al. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fbrosis. Cell 127, 803-815 (2006).
-
(2006)
Cell.
, vol.127
, pp. 803-815
-
-
Wang, X.1
-
12
-
-
40149095757
-
Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis
-
Mu, T. W., Fowler, D. M. & Kelly, J. W. Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis. PLoS Biol. 6, e26 (2008).
-
(2008)
PLoS Biol.
, vol.6
-
-
Mu, T.W.1
Fowler, D.M.2
Kelly, J.W.3
-
13
-
-
50249175120
-
Chemical and biological approaches synergize to ameliorate protein-folding diseases
-
Mu, T. W. et al. Chemical and biological approaches synergize to ameliorate protein-folding diseases. Cell 134, 769-781 (2008).
-
(2008)
Cell.
, vol.134
, pp. 769-781
-
-
Mu, T.W.1
-
14
-
-
33748792821
-
Opposing activities protect against age-onset proteotoxicity
-
Cohen, E., Bieschke, J., Perciavalle, R. M., Kelly, J. W. & Dillin, A. Opposing activities protect against age-onset proteotoxicity. Science 313, 1604-1610 (2006).
-
(2006)
Science
, vol.313
, pp. 1604-1610
-
-
Cohen, E.1
Bieschke, J.2
Perciavalle, R.M.3
Kelly, J.W.4
Dillin, A.5
-
15
-
-
0036678146
-
The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans
-
Morley, J. F., Brignull, H. R., Weyers, J. J. & Morimoto, R. I. The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans. Proc. Natl. Acad. Sci. USA 99, 10417-10422 (2002).
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 10417-10422
-
-
Morley, J.F.1
Brignull, H.R.2
Weyers, J.J.3
Morimoto, R.I.4
-
17
-
-
0037180511
-
Chemical chaperones increase the cellular activity of N370S beta-glucosidase: A therapeutic strategy for Gaucher disease
-
Sawkar, A. R. et al. Chemical chaperones increase the cellular activity of N370S beta-glucosidase: a therapeutic strategy for Gaucher disease. Proc. Natl. Acad. Sci. USA 99, 15428-15433 (2002).
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 15428-15433
-
-
Sawkar, A.R.1
-
18
-
-
33745293617
-
Therapeutic strategies to ameliorate lysosomal storage disorders-a focus on Gaucher disease
-
Sawkar, A. R., D'Haeze, W. & Kelly, J. W. Therapeutic strategies to ameliorate lysosomal storage disorders-a focus on Gaucher disease. Cell. Mol. Life Sci. 63, 1179-1192 (2006).
-
(2006)
Cell. Mol. Life Sci.
, vol.63
, pp. 1179-1192
-
-
Sawkar, A.R.1
D'Haeze, W.2
Kelly, J.W.3
-
19
-
-
23944477827
-
Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease
-
Schmitz, M., Alfalah, M., Aerts, J., Naim, H. Y. & Zimmer, K. P. Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease. Int. J. Biochem. Cell Biol. 37, 2310-2320 (2005).
-
(2005)
Int. J. Biochem. Cell. Biol.
, vol.37
, pp. 2310-2320
-
-
Schmitz, M.1
Alfalah, M.2
Aerts, J.3
Naim, H.Y.4
Zimmer, K.P.5
-
20
-
-
26444609722
-
ER retention and degradation as the molecular basis underlying Gaucher disease heterogeneity
-
Ron, I. & Horowitz, M. ER retention and degradation as the molecular basis underlying Gaucher disease heterogeneity. Hum. Mol. Genet. 14, 2387-2398 (2005).
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 2387-2398
-
-
Ron, I.1
Horowitz, M.2
-
21
-
-
4644224470
-
Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease
-
Schueler, U. H. et al. Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. J. Inherit. Metab. Dis. 27, 649-658 (2004).
-
(2004)
J. Inherit. Metab. Dis.
, vol.27
, pp. 649-658
-
-
Schueler, U.H.1
-
22
-
-
0032798194
-
Intracellular transport of acid β-glucosidase and lysosome-associated membrane proteins is affected in Gaucher's disease (G202R mutation)
-
Zimmer, K.-P. et al. Intracellular transport of acid β-glucosidase and lysosome-associated membrane proteins is affected in Gaucher's disease (G202R mutation). J. Pathol. 188, 407-414 (1999).
-
(1999)
J. Pathol.
, vol.188
, pp. 407-414
-
-
Zimmer, K.-P.1
-
23
-
-
33845240108
-
Chemical chaperones and permissive temperatures alter the cellular localization of Gaucher disease associated glucocerebrosidase variants
-
Sawkar, A. R. et al. Chemical chaperones and permissive temperatures alter the cellular localization of Gaucher disease associated glucocerebrosidase variants. ACS Chem. Biol. 1, 235-251 (2006).
-
(2006)
ACS Chem. Biol.
, vol.1
, pp. 235-251
-
-
Sawkar, A.R.1
-
24
-
-
0036895451
-
Enzyme replacement and enhancement therapies: Lessons from lysosomal disorders
-
Desnick, R. J. & Schuchman, E. H. Enzyme replacement and enhancement therapies: Lessons from lysosomal disorders. Nat. Rev. Genet. 3, 954-966 (2002).
-
(2002)
Nat. Rev. Genet.
, vol.3
, pp. 954-966
-
-
Desnick, R.J.1
Schuchman, E.H.2
-
25
-
-
0025727902
-
Characterization of human glucocerebrosidase from different mutant alleles
-
Ohashi, T. et al. Characterization of human glucocerebrosidase from different mutant alleles. J. Biol. Chem. 266, 3661-3667 (1991).
-
(1991)
J. Biol. Chem.
, vol.266
, pp. 3661-3667
-
-
Ohashi, T.1
-
26
-
-
0033618336
-
Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons
-
Korkotian, E. et al. Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons. J. Biol. Chem. 274, 21673-21678 (1999).
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 21673-21678
-
-
Korkotian, E.1
-
27
-
-
0038607434
-
Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms
-
Lloyd-Evans, E. et al. Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms. J. Biol. Chem. 278, 23594-23599 (2003).
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 23594-23599
-
-
Lloyd-Evans, E.1
-
28
-
-
11844278539
-
Enhanced calcium release in the acute neuronopathic form of Gaucher disease
-
Pelled, D. et al. Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiol. Dis. 18, 83-88 (2005).
-
(2005)
Neurobiol. Dis.
, vol.18
, pp. 83-88
-
-
Pelled, D.1
-
29
-
-
0025040546
-
Direct binding of verapamil to the ryanodine receptor channel of sarcoplasmic-reticulum
-
Valdivia, H. H., Valdivia, C., Ma, J. J. & Coronado, R. Direct binding of verapamil to the ryanodine receptor channel of sarcoplasmic- reticulum. Biophys. J. 58, 471-481 (1990).
-
(1990)
Biophys. J.
, vol.58
, pp. 471-481
-
-
Valdivia, H.H.1
Valdivia, C.2
Ma, J.J.3
Coronado, R.4
-
30
-
-
0025764306
-
Characterization of high-affinity ryanodine-binding sites of rat liver endoplasmic-reticulum-differences between liver and skeletal-muscle
-
Shoshan-Barmatz, V., Pressley, T. A., Higham, S. & Kraus-Friedmann, N. Characterization of high-affinity ryanodine-binding sites of rat liver endoplasmic-reticulum-differences between liver and skeletal-muscle. Biochem. J. 276, 41-46 (1991).
-
(1991)
Biochem. J.
, vol.276
, pp. 41-46
-
-
Shoshan-Barmatz, V.1
Pressley, T.A.2
Higham, S.3
Kraus-Friedmann, N.4
-
31
-
-
34548644786
-
Modulation of the ryanodine receptor and intracellular calcium
-
Zalk, R., Lehnart, S. E. & Marks, A. R. Modulation of the ryanodine receptor and intracellular calcium. Annu. Rev. Biochem. 76, 367-385 (2007).
-
(2007)
Annu. Rev. Biochem.
, vol.76
, pp. 367-385
-
-
Zalk, R.1
Lehnart, S.E.2
Marks, A.R.3
-
32
-
-
0022529046
-
Dantrolene-a review of its pharmacodynamic and pharmacokinetic properties and therapeutic use in malignant hyperthermia, the neuroleptic malignant syndrome and an update of its use in muscle spasticity
-
Ward, A., Chaffman, M. O. & Sorkin, E. M. Dantrolene-a review of its pharmacodynamic and pharmacokinetic properties and therapeutic use in malignant hyperthermia, the neuroleptic malignant syndrome and an update of its use in muscle spasticity. Drugs 32, 130-168 (1986).
-
(1986)
Drugs
, vol.32
, pp. 130-168
-
-
Ward, A.1
Chaffman, M.O.2
Sorkin, E.M.3
-
33
-
-
0028101231
-
Effects of bipyridylium compounds on calcium-release from triadic vesicles isolated from rabbit skeletal-muscle
-
Kang, J. J., Hsu, K. S. & Linshiau, S. Y. Effects of bipyridylium compounds on calcium-release from triadic vesicles isolated from rabbit skeletal-muscle. Br. J. Pharmacol. 112, 1216-1222 (1994).
-
(1994)
Br. J. Pharmacol.
, vol.112
, pp. 1216-1222
-
-
Kang, J.J.1
Hsu, K.S.2
Linshiau, S.Y.3
-
34
-
-
0027397544
-
Inositol trisphosphate and calcium signaling
-
Berridge, M. J. Inositol trisphosphate and calcium signaling. Nature 361, 315-325 (1993).
-
(1993)
Nature
, vol.361
, pp. 315-325
-
-
Berridge, M.J.1
-
37
-
-
0036877146
-
2+ signaling and calcium binding chaperones of the endoplasmic reticulum
-
2+ signaling and calcium binding chaperones of the endoplasmic reticulum. Cell Calcium 32, 269-278 (2002).
-
(2002)
Cell. Calcium
, vol.32
, pp. 269-278
-
-
Michalak, M.1
Parker, J.M.R.2
Opas, M.3
-
38
-
-
0034799402
-
The structure of calnexin, an ER chaperone involved in quality control of protein folding
-
Schrag, J. D. et al. The structure of calnexin, an ER chaperone involved in quality control of protein folding. Mol. Cell 8, 633-644 (2001).
-
(2001)
Mol. Cell.
, vol.8
, pp. 633-644
-
-
Schrag, J.D.1
-
39
-
-
0027156495
-
Interaction with newly synthesized and retained proteins in the endoplasmic-reticulum suggests a chaperone function for human integral membrane-protein IP90 (calnexin)
-
David, V., Hochstenbach, F., Rajagopalan, S. & Brenner, M. B. Interaction with newly synthesized and retained proteins in the endoplasmic-reticulum suggests a chaperone function for human integral membrane-protein IP90 (calnexin). J. Biol. Chem. 268, 9585-9592 (1993).
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 9585-9592
-
-
David, V.1
Hochstenbach, F.2
Rajagopalan, S.3
Brenner, M.B.4
-
40
-
-
33646844964
-
2+ binding site on GRP78 is differentially enhanced by ADP and ATP
-
2+ binding site on GRP78 is differentially enhanced by ADP and ATP. J. Biol. Chem. 281, 8796-8805 (2006).
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 8796-8805
-
-
Lamb, H.K.1
-
42
-
-
0034647539
-
Pivotal role of calnexin and mannose trimming in regulating the endoplasmic reticulum-associated degradation of major histocompatibility complex class I heavy chain
-
Wilson, C. M., Farmery, M. R. & Bulleid, N. J. Pivotal role of calnexin and mannose trimming in regulating the endoplasmic reticulum-associated degradation of major histocompatibility complex class I heavy chain. J. Biol. Chem. 275, 21224-21232 (2000).
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 21224-21232
-
-
Wilson, C.M.1
Farmery, M.R.2
Bulleid, N.J.3
-
43
-
-
0032479290
-
Inhibition of glucose trimming with castanospermine reduces calnexin association and promotes proteasome degradation of the alpha-subunit of the nicotinic acetylcholine receptor
-
Keller, S. H., Lindstrom, J. & Taylor, P. Inhibition of glucose trimming with castanospermine reduces calnexin association and promotes proteasome degradation of the alpha-subunit of the nicotinic acetylcholine receptor. J. Biol. Chem. 273, 17064-17072 (1998).
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 17064-17072
-
-
Keller, S.H.1
Lindstrom, J.2
Taylor, P.3
-
44
-
-
0034254433
-
Role of calnexin, calreticulin, and endoplasmic reticulum mannosidase I in apolipoprotein (a) intracellular targeting
-
Wang, J. & White, A. L. Role of calnexin, calreticulin, and endoplasmic reticulum mannosidase I in apolipoprotein (a) intracellular targeting. Biochemistry 39, 8993-9000 (2000).
-
(2000)
Biochemistry
, vol.39
, pp. 8993-9000
-
-
Wang, J.1
White, A.L.2
-
45
-
-
33750340637
-
Potent lectin-independent chaperone function of calnexin under conditions prevalent within the lumen of the endoplasmic reticulum
-
Brockmeier, A. & Williams, D. B. Potent lectin-independent chaperone function of calnexin under conditions prevalent within the lumen of the endoplasmic reticulum. Biochemistry 45, 12906-12916 (2006).
-
(2006)
Biochemistry
, vol.45
, pp. 12906-12916
-
-
Brockmeier, A.1
Williams, D.B.2
-
46
-
-
25844474620
-
Polypeptide substrate recognition by calnexin requires specific conformations of the calnexin protein
-
Tammavongsa, V., Mancino, L. & Raghavan, M. Polypeptide substrate recognition by calnexin requires specific conformations of the calnexin protein. J. Biol. Chem. 280, 33497-33505 (2005).
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 33497-33505
-
-
Tammavongsa, V.1
Mancino, L.2
Raghavan, M.3
-
47
-
-
0028205240
-
Human, mouse and rat calreticulin cDNA cloning-identification of potential calcium-binding motifs and gene localization to human-chromosome-5
-
Tjoelker, L. W. et al. Human, mouse and rat calreticulin cDNA cloning-identification of potential calcium-binding motifs and gene localization to human-chromosome-5. Biochemistry 33, 3229-3236 (1994).
-
(1994)
Biochemistry
, vol.33
, pp. 3229-3236
-
-
Tjoelker, L.W.1
-
48
-
-
0034282386
-
The conformation of calreticulin is influenced by the endoplasmic reticulum luminal environment
-
Corbett, E. F. et al. The conformation of calreticulin is influenced by the endoplasmic reticulum luminal environment. J. Biol. Chem. 275, 27177-27185 (2000).
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 27177-27185
-
-
Corbett, E.F.1
-
49
-
-
67650410543
-
Biological and chemical approaches to diseases of proteostasis deficiency
-
Powers, E. T., Morimoto, R. I., Dillin, A., Kelly, J. W. & Balch, W. E. Biological and chemical approaches to diseases of proteostasis deficiency. Annu. Rev. Biochem. 78, 23.1-33 (2009).
-
(2009)
Annu. Rev. Biochem.
, vol.78
, pp. 231-233
-
-
Powers, E.T.1
Morimoto, R.I.2
Dillin, A.3
Kelly, J.W.4
Balch, W.E.5
|