-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989; 245: 1066-73.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
-
2
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng SH, Gregory RJ, Marshall J, et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 1990; 63: 827-34.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
-
3
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
Jensen TJ, Loo MA, Pind S, Williams DB, Goldberg AL, Riordan JR. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 1995; 83: 129-35.
-
(1995)
Cell
, vol.83
, pp. 129-135
-
-
Jensen, T.J.1
Loo, M.A.2
Pind, S.3
Williams, D.B.4
Goldberg, A.L.5
Riordan, J.R.6
-
4
-
-
0028840915
-
Degradation of CFTR by the ubiquitin-proteasome pathway
-
Ward CL, Omura S, Kopito RR. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 1995; 83: 121-7.
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
-
5
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 1992; 358: 761-4.
-
(1992)
Nature
, vol.358
, pp. 761-764
-
-
Denning, G.M.1
Anderson, M.P.2
Amara, J.F.3
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
6
-
-
0026337305
-
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes
-
Drumm ML, Wilkinson DJ, Smit LS, et al. Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. Science 1991; 254: 1797-9.
-
(1991)
Science
, vol.254
, pp. 1797-1799
-
-
Drumm, M.L.1
Wilkinson, D.J.2
Smit, L.S.3
-
7
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
Sato S, Ward CL, Krouse ME, Wine JJ, Kopito RR. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol Chem 1996; 271: 635-8.
-
(1996)
J Biol Chem
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
8
-
-
11444266284
-
The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests posttranslational folding of CFTR
-
Du K, Sharma M, Lukacs GL. The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests posttranslational folding of CFTR. Nat Struct Mol Biol 2005; 12: 17-25.
-
(2005)
Nat Struct Mol Biol
, vol.12
, pp. 17-25
-
-
Du, K.1
Sharma, M.2
Lukacs, G.L.3
-
9
-
-
0842334751
-
Turning up the heat in the lungs. A key mechanism to preserve their function
-
Sartori C, Scherrer U. Turning up the heat in the lungs. A key mechanism to preserve their function. Adv Exp Med Biol 2003; 543: 263-75.
-
(2003)
Adv Exp Med Biol
, vol.543
, pp. 263-275
-
-
Sartori, C.1
Scherrer, U.2
-
10
-
-
84893708885
-
Stress and the inflammatory process: A major cause of pancreatic cell death in type 2 diabetes
-
Montane J, Cadavez L, Novials A. Stress and the inflammatory process: a major cause of pancreatic cell death in type 2 diabetes. Diabetes Metab Syndr Obes 2014; 7: 25-34.
-
(2014)
Diabetes Metab Syndr Obes
, vol.7
, pp. 25-34
-
-
Montane, J.1
Cadavez, L.2
Novials, A.3
-
11
-
-
0027488993
-
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
-
Yang Y, Janich S, Cohn JA, Wilson JM. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc Natl Acad Sci USA 1993; 90: 9480-4.
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 9480-9484
-
-
Yang, Y.1
Janich, S.2
Cohn, J.A.3
Wilson, J.M.4
-
12
-
-
0030798979
-
The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotidebinding domain of the cystic fibrosis transmembrane conductance regulator
-
Strickland E, Qu BH, Millen L, Thomas PJ. The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotidebinding domain of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1997; 272: 25421-4.
-
(1997)
J Biol Chem
, vol.272
, pp. 25421-25424
-
-
Strickland, E.1
Qu, B.H.2
Millen, L.3
Thomas, P.J.4
-
13
-
-
0035146685
-
The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins
-
Connell P, Ballinger CA, Jiang J, et al. The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins. Nat Cell Biol 2001; 3: 93-6.
-
(2001)
Nat Cell Biol
, vol.3
, pp. 93-96
-
-
Connell, P.1
Ballinger, C.A.2
Jiang, J.3
-
14
-
-
0035142877
-
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
-
Meacham GC, Patterson C, Zhang W, Younger JM, Cyr DM. The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation. Nat Cell Biol 2001; 3: 100-5.
-
(2001)
Nat Cell Biol
, vol.3
, pp. 100-105
-
-
Meacham, G.C.1
Patterson, C.2
Zhang, W.3
Younger, J.M.4
Cyr, D.M.5
-
15
-
-
4344578534
-
The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator
-
Alberti S, Bohse K, Arndt V, Schmitz A, Hohfeld J. The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator. Mol Biol Cell 2004; 15: 4003-10.
-
(2004)
Mol Biol Cell
, vol.15
, pp. 4003-4010
-
-
Alberti, S.1
Bohse, K.2
Arndt, V.3
Schmitz, A.4
Hohfeld, J.5
-
16
-
-
28644442088
-
BAG-2 Acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP
-
Arndt V, Daniel C, Nastainczyk W, Alberti S, Hohfeld J. BAG-2 Acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP. Mol Biol Cell 2005; 16: 5891-900.
-
(2005)
Mol Biol Cell
, vol.16
, pp. 5891-5900
-
-
Arndt, V.1
Daniel, C.2
Nastainczyk, W.3
Alberti, S.4
Hohfeld, J.5
-
17
-
-
0037106481
-
The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 cochaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70
-
Farinha CM, Nogueira P, Mendes F, Penque D, Amaral MD. The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 cochaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. Biochem J 2002; 366: 797-806.
-
(2002)
Biochem J
, vol.366
, pp. 797-806
-
-
Farinha, C.M.1
Nogueira, P.2
Mendes, F.3
Penque, D.4
Amaral, M.D.5
-
18
-
-
79551678082
-
The endoplasmic reticulumassociated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508
-
Grove DE, Fan CY, Ren HY, Cyr DM. The endoplasmic reticulumassociated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508. Mol Biol Cell 2011; 22: 301-14.
-
(2011)
Mol Biol Cell
, vol.22
, pp. 301-314
-
-
Grove, D.E.1
Fan, C.Y.2
Ren, H.Y.3
Cyr, D.M.4
-
19
-
-
0033559258
-
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
-
Meacham GC, Lu Z, King S, Sorscher E, Tousson A, Cyr DM. The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J 1999; 18: 1492-505.
-
(1999)
EMBO J
, vol.18
, pp. 1492-1505
-
-
Meacham, G.C.1
Lu, Z.2
King, S.3
Sorscher, E.4
Tousson, A.5
Cyr, D.M.6
-
20
-
-
84869203984
-
BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-likedomain-dependent manner
-
Mendes F, Farinha CM, Felicio V, Alves PC, Vieira I, Amaral MD. BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-likedomain-dependent manner. Cell Physiol Biochem 2012; 30: 1120-33.
-
(2012)
Cell Physiol Biochem
, vol.30
, pp. 1120-1133
-
-
Mendes, F.1
Farinha, C.M.2
Felicio, V.3
Alves, P.C.4
Vieira, I.5
Amaral, M.D.6
-
21
-
-
84861749562
-
Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels
-
Saxena A, Banasavadi-Siddegowda YK, Fan Y, et al. Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels. J Biol Chem 2012; 287: 19158-70.
-
(2012)
J Biol Chem
, vol.287
, pp. 19158-19170
-
-
Saxena, A.1
Banasavadi-Siddegowda, Y.K.2
Fan, Y.3
-
22
-
-
63249095885
-
Cysteine string protein promotes proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) by increasing its interaction with the C terminus of Hsp70-interacting protein and promoting CFTR ubiquitylation
-
Schmidt BZ, Watts RJ, Aridor M, Frizzell RA. Cysteine string protein promotes proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) by increasing its interaction with the C terminus of Hsp70-interacting protein and promoting CFTR ubiquitylation. J Biol Chem 2009; 284: 4168-78.
-
(2009)
J Biol Chem
, vol.284
, pp. 4168-4178
-
-
Schmidt, B.Z.1
Watts, R.J.2
Aridor, M.3
Frizzell, R.A.4
-
23
-
-
84885942427
-
A novel mammalian ER-located J-protein, DNAJB14, can accelerate ERAD of misfolded membrane proteins
-
Sopha P, Kadokura H, Yamamoto YH, et al. A novel mammalian ER-located J-protein, DNAJB14, can accelerate ERAD of misfolded membrane proteins. Cell Struct Funct 2012; 37: 177-87.
-
(2012)
Cell Struct Funct
, vol.37
, pp. 177-187
-
-
Sopha, P.1
Kadokura, H.2
Yamamoto, Y.H.3
-
24
-
-
79952524195
-
A novel ER Jprotein DNAJB12 accelerates ER-associated degradation of membrane proteins including CFTR
-
Yamamoto YH, Kimura T, Momohara S, et al. A novel ER Jprotein DNAJB12 accelerates ER-associated degradation of membrane proteins including CFTR. Cell Struct Funct 2010; 35: 107-16.
-
(2010)
Cell Struct Funct
, vol.35
, pp. 107-116
-
-
Yamamoto, Y.H.1
Kimura, T.2
Momohara, S.3
-
25
-
-
0039172708
-
The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome
-
Luders J, Demand J, Hohfeld J. The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome. J Biol Chem 2000; 275: 4613-7.
-
(2000)
J Biol Chem
, vol.275
, pp. 4613-4617
-
-
Luders, J.1
Demand, J.2
Hohfeld, J.3
-
26
-
-
0037195907
-
Ubiquitylation of BAG-1 suggests a novel regulatory mechanism during the sorting of chaperone substrates to the proteasome
-
Alberti S, Demand J, Esser C, Emmerich N, Schild H, Hohfeld J. Ubiquitylation of BAG-1 suggests a novel regulatory mechanism during the sorting of chaperone substrates to the proteasome. J Biol Chem 2002; 277: 45920-7.
-
(2002)
J Biol Chem
, vol.277
, pp. 45920-45927
-
-
Alberti, S.1
Demand, J.2
Esser, C.3
Emmerich, N.4
Schild, H.5
Hohfeld, J.6
-
27
-
-
0035899897
-
Cooperation of a ubiquitin domain protein and an E3 ubiquitin ligase during chaperone/ proteasome coupling
-
Demand J, Alberti S, Patterson C, Hohfeld J. Cooperation of a ubiquitin domain protein and an E3 ubiquitin ligase during chaperone/ proteasome coupling. Curr Biol 2001; 11: 1569-77.
-
(2001)
Curr Biol
, vol.11
, pp. 1569-1577
-
-
Demand, J.1
Alberti, S.2
Patterson, C.3
Hohfeld, J.4
-
28
-
-
0030809817
-
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR
-
Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest 1997; 100: 2457-65.
-
(1997)
J Clin Invest
, vol.100
, pp. 2457-2465
-
-
Rubenstein, R.C.1
Egan, M.E.2
Zeitlin, P.L.3
-
30
-
-
0034805392
-
Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation
-
Rubenstein RC, Lyons BM. Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation. Am J Physiol Lung Cell Mol Physiol 2001; 281: L43-51.
-
(2001)
Am J Physiol Lung Cell Mol Physiol
, vol.281
, pp. L43-L51
-
-
Rubenstein, R.C.1
Lyons, B.M.2
-
31
-
-
84455173094
-
4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells
-
Suaud L, Miller K, Panichelli AE, Randell RL, Marando CM, Rubenstein RC. 4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells. J Biol Chem 2011; 286: 45083-92.
-
(2011)
J Biol Chem
, vol.286
, pp. 45083-45092
-
-
Suaud, L.1
Miller, K.2
Panichelli, A.E.3
Randell, R.L.4
Marando, C.M.5
Rubenstein, R.C.6
-
32
-
-
77951211566
-
Heat stress cognate 70 host protein as a potential drug target against drug resistance in hepatitis B virus
-
Wang YP, Liu F, He HW, et al. Heat stress cognate 70 host protein as a potential drug target against drug resistance in hepatitis B virus. Antimicrob Agents Chemother 2010; 54: 2070-7.
-
(2010)
Antimicrob Agents Chemother
, vol.54
, pp. 2070-2077
-
-
Wang, Y.P.1
Liu, F.2
He, H.W.3
-
33
-
-
84862074188
-
Matrine modulates HSC70 levels and rescues DeltaF508-CFTR
-
Basile A, Pascale M, Franceschelli S, et al. Matrine modulates HSC70 levels and rescues DeltaF508-CFTR. J Cell Physiol 2012; 227: 3317-23.
-
(2012)
J Cell Physiol
, vol.227
, pp. 3317-3323
-
-
Basile, A.1
Pascale, M.2
Franceschelli, S.3
-
34
-
-
0032401771
-
Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome
-
Loo MA, Jensen TJ, Cui L, Hou Y, Chang XB, Riordan JR. Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome. EMBO J 1998; 17: 6879-87.
-
(1998)
EMBO J
, vol.17
, pp. 6879-6887
-
-
Loo, M.A.1
Jensen, T.J.2
Cui, L.3
Hou, Y.4
Chang, X.B.5
Riordan, J.R.6
-
35
-
-
6344275303
-
Distinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast
-
Youker RT, Walsh P, Beilharz T, Lithgow T, Brodsky JL. Distinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast. Mol Biol Cell 2004; 15: 4787-97.
-
(2004)
Mol Biol Cell
, vol.15
, pp. 4787-4797
-
-
Youker, R.T.1
Walsh, P.2
Beilharz, T.3
Lithgow, T.4
Brodsky, J.L.5
-
36
-
-
0037315208
-
Regulation of signaling protein function and trafficking by the hsp90/hsp70-based chaperone machinery
-
Pratt WB, Toft DO. Regulation of signaling protein function and trafficking by the hsp90/hsp70-based chaperone machinery. Exp Biol Med (Maywood) 2003; 228: 111-33.
-
(2003)
Exp Biol Med (Maywood)
, vol.228
, pp. 111-133
-
-
Pratt, W.B.1
Toft, D.O.2
-
37
-
-
33750842131
-
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis
-
Wang X, Venable J, Lapointe P, et al. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006; 127: 803-15.
-
(2006)
Cell
, vol.127
, pp. 803-815
-
-
Wang, X.1
Venable, J.2
Lapointe, P.3
-
38
-
-
85039835790
-
Cochaperones Hop and Hsp105 functionally link Hsp70 and Hsp90 during DeltaF508 CFTR maturation at low temperature
-
Saxena A, Bhattacharya S, Fan Y, et al. Cochaperones Hop and Hsp105 functionally link Hsp70 and Hsp90 during DeltaF508 CFTR maturation at low temperature. Pediatr Pulmonol 2011; 46: S215.
-
(2011)
Pediatr Pulmonol
, vol.46
-
-
Saxena, A.1
Bhattacharya, S.2
Fan, Y.3
-
39
-
-
0036931438
-
Activation of the ATPase activity of hsp90 by the stress-regulated cochaperone aha1
-
Panaretou B, Siligardi G, Meyer P, et al. Activation of the ATPase activity of hsp90 by the stress-regulated cochaperone aha1. Mol Cell 2002; 10: 1307-18.
-
(2002)
Mol Cell
, vol.10
, pp. 1307-1318
-
-
Panaretou, B.1
Siligardi, G.2
Meyer, P.3
-
40
-
-
77949438155
-
Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis
-
Koulov AV, Lapointe P, Lu B, et al. Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis. Mol Biol Cell 2010; 21: 871-84.
-
(2010)
Mol Biol Cell
, vol.21
, pp. 871-884
-
-
Koulov, A.V.1
Lapointe, P.2
Lu, B.3
-
41
-
-
81755163563
-
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
-
Van Goor F, Hadida S, Grootenhuis PD, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A 2011; 108: 18843-8.
-
(2011)
Proc Natl Acad Sci U S A
, vol.108
, pp. 18843-18848
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
42
-
-
0027380236
-
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
-
Lukacs GL, Chang XB, Bear C, et al. The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J Biol Chem 1993; 268: 21592-8.
-
(1993)
J Biol Chem
, vol.268
, pp. 21592-21598
-
-
Lukacs, G.L.1
Chang, X.B.2
Bear, C.3
-
43
-
-
76649127413
-
Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue
-
Roy G, Chalfin EM, Saxena A, Wang X. Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue. Mol Biol Cell 2010; 21: 597-609.
-
(2010)
Mol Biol Cell
, vol.21
, pp. 597-609
-
-
Roy, G.1
Chalfin, E.M.2
Saxena, A.3
Wang, X.4
-
44
-
-
77955607650
-
Peripheral protein quality control removes unfolded CFTR from the plasma membrane
-
Okiyoneda T, Barriere H, Bagdany M, et al. Peripheral protein quality control removes unfolded CFTR from the plasma membrane. Science 2010; 329: 805-10.
-
(2010)
Science
, vol.329
, pp. 805-810
-
-
Okiyoneda, T.1
Barriere, H.2
Bagdany, M.3
-
45
-
-
0034804969
-
S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation
-
Zaman K, Mcpherson M, Vaughan J, et al. S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation. Biochem Biophys Res Commun 2001; 284: 65-70.
-
(2001)
Biochem Biophys Res Commun
, vol.284
, pp. 65-70
-
-
Zaman, K.1
Mcpherson, M.2
Vaughan, J.3
-
46
-
-
33748944330
-
S-nitrosylating agents: A novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells
-
Zaman K, Carraro S, Doherty J, et al. S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells. Mol Pharmacol 2006; 70: 1435-42.
-
(2006)
Mol Pharmacol
, vol.70
, pp. 1435-1442
-
-
Zaman, K.1
Carraro, S.2
Doherty, J.3
-
47
-
-
77954939833
-
Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy
-
Marozkina NV, Yemen S, Borowitz M, et al. Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy. Proc Natl Acad Sci USA 2010; 107: 11393-8.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 11393-11398
-
-
Marozkina, N.V.1
Yemen, S.2
Borowitz, M.3
-
48
-
-
53849149321
-
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking
-
Wang X, Koulov AV, Kellner WA, Riordan JR, Balch WE. Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic 2008; 9: 1878-93.
-
(2008)
Traffic
, vol.9
, pp. 1878-1893
-
-
Wang, X.1
Koulov, A.V.2
Kellner, W.A.3
Riordan, J.R.4
Balch, W.E.5
-
49
-
-
0028230782
-
Quantitation of the interaction of the immunosuppressant deoxyspergualin and analogs with Hsc70 and Hsp90
-
Nadeau K, Nadler SG, Saulnier M, Tepper MA, Walsh CT. Quantitation of the interaction of the immunosuppressant deoxyspergualin and analogs with Hsc70 and Hsp90. Biochemistry 1994; 33: 2561-7.
-
(1994)
Biochemistry
, vol.33
, pp. 2561-2567
-
-
Nadeau, K.1
Nadler, S.G.2
Saulnier, M.3
Tepper, M.A.4
Walsh, C.T.5
-
50
-
-
0026526303
-
Interaction of the immunosuppressant deoxyspergualin with a member of the Hsp70 family of heat shock proteins
-
Nadler SG, Tepper MA, Schacter B, Mazzucco CE. Interaction of the immunosuppressant deoxyspergualin with a member of the Hsp70 family of heat shock proteins. Science 1992; 258: 484-6.
-
(1992)
Science
, vol.258
, pp. 484-486
-
-
Nadler, S.G.1
Tepper, M.A.2
Schacter, B.3
Mazzucco, C.E.4
-
51
-
-
0031853136
-
Partial restoration of cAMPstimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin
-
Jiang C, Fang SL, Xiao YF, et al. Partial restoration of cAMPstimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin. Am J Physiol 1998; 275: C171-8.
-
(1998)
Am J Physiol
, vol.275
, pp. C171-C178
-
-
Jiang, C.1
Fang, S.L.2
Xiao, Y.F.3
-
52
-
-
52149103361
-
Chemical conjugation of DeltaF508-CFTR corrector deoxyspergualin to transporter human serum albumin enhances its ability to rescue Cl-channel functions
-
Norez C, Pasetto M, Dechecchi MC, et al. Chemical conjugation of DeltaF508-CFTR corrector deoxyspergualin to transporter human serum albumin enhances its ability to rescue Cl-channel functions. Am J Physiol Lung Cell Mol Physiol 2008; 295: L336-47.
-
(2008)
Am J Physiol Lung Cell Mol Physiol
, vol.295
, pp. L336-L347
-
-
Norez, C.1
Pasetto, M.2
Dechecchi, M.C.3
-
53
-
-
34548370676
-
Altered biogenesis of deltaF508-CFTR following treatment with doxorubicin
-
Maitra R, Hamilton JW. Altered biogenesis of deltaF508-CFTR following treatment with doxorubicin. Cell Physiol Biochem 2007; 20: 465-72.
-
(2007)
Cell Physiol Biochem
, vol.20
, pp. 465-472
-
-
Maitra, R.1
Hamilton, J.W.2
-
54
-
-
0028232167
-
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
-
Pind S, Riordan JR, Williams DB. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1994; 269: 12784-8.
-
(1994)
J Biol Chem
, vol.269
, pp. 12784-12788
-
-
Pind, S.1
Riordan, J.R.2
Williams, D.B.3
-
55
-
-
33645211759
-
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alphaglucosidase inhibitor miglustat
-
Norez C, Noel S, Wilke M, et al. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alphaglucosidase inhibitor miglustat. FEBS Lett 2006; 580: 2081-6.
-
(2006)
FEBS Lett
, vol.580
, pp. 2081-2086
-
-
Norez, C.1
Noel, S.2
Wilke, M.3
-
56
-
-
51349113842
-
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia
-
Sun F, Mi Z, Condliffe SB, et al. Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia. FASEB J 2008; 22: 3255-63.
-
(2008)
FASEB J
, vol.22
, pp. 3255-3263
-
-
Sun, F.1
Mi, Z.2
Condliffe, S.B.3
-
57
-
-
29144527460
-
Small heat shock proteins: Molecular structure and chaperone function
-
Sun Y, Macrae TH. Small heat shock proteins: molecular structure and chaperone function. Cell Mol Life Sci 2005; 62: 2460-76.
-
(2005)
Cell Mol Life Sci
, vol.62
, pp. 2460-2476
-
-
Sun, Y.1
Macrae, T.H.2
-
58
-
-
33947134399
-
Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation
-
Ahner A, Nakatsukasa K, Zhang H, Frizzell RA, Brodsky JL. Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation. Mol Biol Cell 2007; 18: 806-14.
-
(2007)
Mol Biol Cell
, vol.18
, pp. 806-814
-
-
Ahner, A.1
Nakatsukasa, K.2
Zhang, H.3
Frizzell, R.A.4
Brodsky, J.L.5
-
59
-
-
84872286562
-
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway
-
Ahner A, Gong X, Schmidt BZ, et al. Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway. Mol Biol Cell 2013; 24: 74-84.
-
(2013)
Mol Biol Cell
, vol.24
, pp. 74-84
-
-
Ahner, A.1
Gong, X.2
Schmidt, B.Z.3
-
60
-
-
44849094781
-
Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging
-
Morimoto RI. Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging. Genes Dev 2008; 22: 1427-38.
-
(2008)
Genes Dev
, vol.22
, pp. 1427-1438
-
-
Morimoto, R.I.1
-
61
-
-
19944426695
-
Celastrols as inducers of the heat shock response and cytoprotection
-
Westerheide SD, Bosman JD, Mbadugha BN, et al. Celastrols as inducers of the heat shock response and cytoprotection. J Biol Chem 2004; 279: 56053-60.
-
(2004)
J Biol Chem
, vol.279
, pp. 56053-56060
-
-
Westerheide, S.D.1
Bosman, J.D.2
Mbadugha, B.N.3
-
62
-
-
0033111685
-
Histone deacetylases: Complex transducers of nuclear signals
-
Johnson CA, Turner BM. Histone deacetylases: complex transducers of nuclear signals. Semin Cell Dev Biol 1999; 10: 179-88.
-
(1999)
Semin Cell Dev Biol
, vol.10
, pp. 179-188
-
-
Johnson, C.A.1
Turner, B.M.2
-
63
-
-
77950428804
-
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis
-
Hutt DM, Herman D, Rodrigues AP, et al. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol 2010; 6: 25-33.
-
(2010)
Nat Chem Biol
, vol.6
, pp. 25-33
-
-
Hutt, D.M.1
Herman, D.2
Rodrigues, A.P.3
-
64
-
-
70149091116
-
SUMOylation of tissue transglutaminase as link between oxidative stress and inflammation
-
Luciani A, Villella VR, Vasaturo A, et al. SUMOylation of tissue transglutaminase as link between oxidative stress and inflammation. J Immunol 2009; 183: 2775-84.
-
(2009)
J Immunol
, vol.183
, pp. 2775-2784
-
-
Luciani, A.1
Villella, V.R.2
Vasaturo, A.3
-
65
-
-
47249102033
-
Tissue transglutaminase activation modulates inflammation in cystic fibrosis via PPARgamma down-regulation
-
Maiuri L, Luciani A, Giardino I, et al. Tissue transglutaminase activation modulates inflammation in cystic fibrosis via PPARgamma down-regulation. J Immunol 2008; 180: 7697-705.
-
(2008)
J Immunol
, vol.180
, pp. 7697-7705
-
-
Maiuri, L.1
Luciani, A.2
Giardino, I.3
-
66
-
-
0034537290
-
Autophagy as a regulated pathway of cellular degradation
-
Klionsky DJ, Emr SD. Autophagy as a regulated pathway of cellular degradation. Science 2000; 290: 1717-21.
-
(2000)
Science
, vol.290
, pp. 1717-1721
-
-
Klionsky, D.J.1
Emr, S.D.2
-
67
-
-
77956472436
-
The role of autophagy during development in higher eukaryotes
-
Di Bartolomeo S, Nazio F, Cecconi F. The role of autophagy during development in higher eukaryotes. Traffic 2010; 11: 1280-9.
-
(2010)
Traffic
, vol.11
, pp. 1280-1289
-
-
Di Bartolomeo, S.1
Nazio, F.2
Cecconi, F.3
-
68
-
-
10744225487
-
A unified nomenclature for yeast autophagy-related genes
-
Klionsky DJ, Cregg JM, Dunn WA Jr, et al. A unified nomenclature for yeast autophagy-related genes. Dev Cell 2003; 5: 539-45.
-
(2003)
Dev Cell
, vol.5
, pp. 539-545
-
-
Klionsky, D.J.1
Cregg, J.M.2
Dunn, W.A.3
-
69
-
-
84910155187
-
Autophagy in macrophages: Impacting inflammation and bacterial infection
-
Vural A, Kehrl JH. Autophagy in macrophages: impacting inflammation and bacterial infection. Scientifica (Cairo) 2014; 2014: 825463.
-
(2014)
Scientifica (Cairo)
, vol.2014
-
-
Vural, A.1
Kehrl, J.H.2
-
70
-
-
77956396747
-
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition
-
Luciani A, Villella VR, Esposito S, et al. Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition. Nat Cell Biol 2010; 12: 863-75.
-
(2010)
Nat Cell Biol
, vol.12
, pp. 863-875
-
-
Luciani, A.1
Villella, V.R.2
Esposito, S.3
-
71
-
-
84869389296
-
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on DeltaF508 cystic fibrosis transmembrane conductance regulator
-
Luciani A, Villella VR, Esposito S, et al. Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on DeltaF508 cystic fibrosis transmembrane conductance regulator. Autophagy 2012; 8: 1657-72.
-
(2012)
Autophagy
, vol.8
, pp. 1657-1672
-
-
Luciani, A.1
Villella, V.R.2
Esposito, S.3
-
72
-
-
84919764939
-
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
-
De Stefano D, Villella VR, Esposito S, et al. Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Autophagy 2014; 10: 2053-74.
-
(2014)
Autophagy
, vol.10
, pp. 2053-2074
-
-
De Stefano, D.1
Villella, V.R.2
Esposito, S.3
-
73
-
-
4344673498
-
Mechanisms of chaperone-mediated autophagy
-
Majeski AE, Dice JF. Mechanisms of chaperone-mediated autophagy. Int J Biochem Cell Biol 2004; 36: 2435-44.
-
(2004)
Int J Biochem Cell Biol
, vol.36
, pp. 2435-2444
-
-
Majeski, A.E.1
Dice, J.F.2
-
74
-
-
84891741302
-
Chaperone-mediated autophagy: Roles in disease and aging
-
Cuervo AM, Wong E. Chaperone-mediated autophagy: roles in disease and aging. Cell Res 2014; 24: 92-104.
-
(2014)
Cell Res
, vol.24
, pp. 92-104
-
-
Cuervo, A.M.1
Wong, E.2
-
75
-
-
84874529463
-
It's a cell-eat-cell world: Autophagy and phagocytosis
-
Oczypok EA, Oury TD, Chu CT. It's a cell-eat-cell world: autophagy and phagocytosis. Am J Pathol 2013; 182: 612-22.
-
(2013)
Am J Pathol
, vol.182
, pp. 612-622
-
-
Oczypok, E.A.1
Oury, T.D.2
Chu, C.T.3
-
76
-
-
80655134731
-
Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis
-
Abdulrahman BA, Khweek AA, Akhter A, et al. Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis. Autophagy 2011; 7: 1359-70.
-
(2011)
Autophagy
, vol.7
, pp. 1359-1370
-
-
Abdulrahman, B.A.1
Khweek, A.A.2
Akhter, A.3
-
77
-
-
80052378958
-
Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection
-
Renna M, Schaffner C, Brown K, et al. Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection. J Clin Invest 2011; 121: 3554-63.
-
(2011)
J Clin Invest
, vol.121
, pp. 3554-3563
-
-
Renna, M.1
Schaffner, C.2
Brown, K.3
-
78
-
-
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, et al. p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death. J Cell Biol 2005; 171: 603-14.
-
(2005)
J Cell Biol
, vol.171
, pp. 603-614
-
-
Bjorkoy, G.1
Lamark, T.2
Brech, A.3
-
79
-
-
84872742749
-
Depletion of the ubiquitin-binding adaptor molecule SQSTM1/p62 from macrophages harboring cftr DeltaF508 mutation improves the delivery of Burkholderia cenocepacia to the autophagic machinery
-
Abdulrahman BA, Khweek AA, Akhter A, et al. Depletion of the ubiquitin-binding adaptor molecule SQSTM1/p62 from macrophages harboring cftr DeltaF508 mutation improves the delivery of Burkholderia cenocepacia to the autophagic machinery. J Biol Chem 2013; 288: 2049-58.
-
(2013)
J Biol Chem
, vol.288
, pp. 2049-2058
-
-
Abdulrahman, B.A.1
Khweek, A.A.2
Akhter, A.3
-
80
-
-
84877804452
-
Ivacaftor: The first therapy acting on the primary cause of cystic fibrosis
-
Mcphail GL, Clancy JP. Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis. Drugs Today (Barc) 2013; 49: 253-60.
-
(2013)
Drugs Today (Barc)
, vol.49
, pp. 253-260
-
-
Mcphail, G.L.1
Clancy, J.P.2
-
81
-
-
84862776940
-
Ivacaftor potentiation of multiple CFTR channels with gating mutations
-
Yu H, Burton B, Huang CJ, et al. Ivacaftor potentiation of multiple CFTR channels with gating mutations. J Cyst Fibros 2012; 11: 237-45.
-
(2012)
J Cyst Fibros
, vol.11
, pp. 237-245
-
-
Yu, H.1
Burton, B.2
Huang, C.J.3
-
82
-
-
84855202429
-
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
-
Clancy JP, Rowe SM, Accurso FJ, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 2012; 67: 12-8.
-
(2012)
Thorax
, vol.67
, pp. 12-18
-
-
Clancy, J.P.1
Rowe, S.M.2
Accurso, F.J.3
-
83
-
-
84930680956
-
Enhancing the potency of F508del correction: A multi-layer combinational approach to drug discovery for cystic fibrosis
-
Kirby EF, Heard AS, Wang XR. Enhancing the potency of F508del correction: a multi-layer combinational approach to drug discovery for cystic fibrosis. J Pharmacol Clin Toxicol 2013; 1: 1007.
-
(2013)
J Pharmacol Clin Toxicol
, vol.1
, pp. 1007
-
-
Kirby, E.F.1
Heard, A.S.2
Wang, X.R.3
-
84
-
-
0026782073
-
Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current
-
Mcdonald TV, Nghiem PT, Gardner P, Martens CL. Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current. J Biol Chem 1992; 267: 3242-8.
-
(1992)
J Biol Chem
, vol.267
, pp. 3242-3248
-
-
Mcdonald, T.V.1
Nghiem, P.T.2
Gardner, P.3
Martens, C.L.4
-
85
-
-
33748654201
-
CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis
-
Painter RG, Valentine VG, Lanson NA, Jr., et al. CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis. Biochemistry 2006; 45: 10260-9.
-
(2006)
Biochemistry
, vol.45
, pp. 10260-10269
-
-
Painter, R.G.1
Valentine, V.G.2
Lanson, N.A.3
|