-
1
-
-
79958083917
-
New clinical diagnostic procedures for cystic fibrosis in Europe
-
De Boeck K, Derichs N, Fajac I, et al. New clinical diagnostic procedures for cystic fibrosis in Europe. J Cyst Fibros 2011; 10 Suppl 2: S53-66.
-
(2011)
J Cyst Fibros
, vol.10
, Issue.SUPPL. 2
, pp. 53-66
-
-
De Boeck, K.1
Derichs, N.2
Fajac, I.3
-
2
-
-
0029866870
-
The molecular basis for disease variability in cystic fibrosis
-
Kerem B, Kerem E. The molecular basis for disease variability in cystic fibrosis. Eur J Hum Genet 1996; 4: 65-73.
-
(1996)
Eur J Hum Genet
, vol.4
, pp. 65-73
-
-
Kerem, B.1
Kerem, E.2
-
3
-
-
80052307732
-
Introduction to Section I: The relevance of CF diagnostic tools for measuring restoration of CFTR function after therapeutic interventions in human clinical trials
-
De Boeck K, Ashlock M. Introduction to Section I: the relevance of CF diagnostic tools for measuring restoration of CFTR function after therapeutic interventions in human clinical trials. Methods Mol Biol 2011; 741: 3-11.
-
(2011)
Methods Mol Biol
, vol.741
, pp. 3-11
-
-
De Boeck, K.1
Ashlock, M.2
-
4
-
-
79551596804
-
Therapeutics development for cystic fibrosis: A successful model for a multisystem genetic disease
-
Ashlock MA, Olson ER. Therapeutics development for cystic fibrosis: a successful model for a multisystem genetic disease. Ann Rev Med 2011; 62: 107-25.
-
(2011)
Ann Rev Med
, vol.62
, pp. 107-125
-
-
Ashlock, M.A.1
Olson, E.R.2
-
5
-
-
85047691737
-
CFTR, a channel with the structure of a transporter
-
Riordan JR, Chang XB. CFTR, a channel with the structure of a transporter. Biochim Biophys Acta 1992; 1101: 221-2.
-
(1992)
Biochim Biophys Acta
, vol.1101
, pp. 221-222
-
-
Riordan, J.R.1
Chang, X.B.2
-
6
-
-
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
-
7
-
-
50649123290
-
CFTR function and prospects for therapy
-
Riordan JR. CFTR function and prospects for therapy. Annu Rev Biochem 2008; 77: 701-26.
-
(2008)
Annu Rev Biochem
, vol.77
, pp. 701-726
-
-
Riordan, J.R.1
-
8
-
-
15544371839
-
Assembly of functional CFTR chloride channels
-
Riordan JR. Assembly of functional CFTR chloride channels. Ann Rev Physiol 2005; 67: 701-18.
-
(2005)
Ann Rev Physiol
, vol.67
, pp. 701-718
-
-
Riordan, J.R.1
-
10
-
-
34347333381
-
Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis
-
Amaral MD, Kunzelmann K. Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends Pharm Sci 2007; 28: 334-41.
-
(2007)
Trends Pharm Sci
, vol.28
, pp. 334-341
-
-
Amaral, M.D.1
Kunzelmann, K.2
-
11
-
-
79953300971
-
Targeting CFTR: How to treat cystic fibrosis by CFTR-repairing therapies
-
Amaral MD. Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies. Curr Drug Targets 2011; 12: 683-93.
-
(2011)
Curr Drug Targets
, vol.12
, pp. 683-693
-
-
Amaral, M.D.1
-
12
-
-
0029616734
-
Cystic fibrosis: Genotypic and phenotypic variations
-
Zielenski J, Tsui LC. Cystic fibrosis: genotypic and phenotypic variations. Ann Rev Genet 1995; 29: 777-807.
-
(1995)
Ann Rev Genet
, vol.29
, pp. 777-807
-
-
Zielenski, J.1
Tsui, L.C.2
-
13
-
-
79953700578
-
New horizons in the treatment of cystic fibrosis
-
Cuthbert AW. New horizons in the treatment of cystic fibrosis. Br J Pharm 2011; 163: 173-83.
-
(2011)
Br J Pharm
, vol.163
, pp. 173-183
-
-
Cuthbert, A.W.1
-
14
-
-
0026523829
-
Cystic fibrosis: Molecular biology and therapeutic implications
-
Collins FS. Cystic fibrosis: molecular biology and therapeutic implications. Science 1992; 256: 774-9.
-
(1992)
Science
, vol.256
, pp. 774-779
-
-
Collins, F.S.1
-
15
-
-
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
-
16
-
-
0037023764
-
A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator
-
Gelman MS, Kannegaard ES, Kopito RR. A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator. J Biol Chem 2002; 277: 11709-14.
-
(2002)
J Biol Chem
, vol.277
, pp. 11709-11714
-
-
Gelman, M.S.1
Kannegaard, E.S.2
Kopito, R.R.3
-
17
-
-
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
-
18
-
-
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
-
19
-
-
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
-
20
-
-
20344378216
-
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin
-
Farinha CM, Amaral MD. Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol 2005; 25: 5242-52.
-
(2005)
Mol Cell Biol
, vol.25
, pp. 5242-5252
-
-
Farinha, C.M.1
Amaral, M.D.2
-
21
-
-
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
-
22
-
-
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
-
23
-
-
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
-
24
-
-
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
-
25
-
-
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
-
26
-
-
0037047293
-
Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator
-
Zhang H, Peters KW, Sun F, et al. Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 2002; 277: 28948-58.
-
(2002)
J Biol Chem
, vol.277
, pp. 28948-28958
-
-
Zhang, H.1
Peters, K.W.2
Sun, F.3
-
27
-
-
33845197320
-
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotidebinding domain of CFTR by different mechanisms
-
Roxo-Rosa M, Xu Z, Schmidt A, et al. Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotidebinding domain of CFTR by different mechanisms. Proc Natl Acad Sci USA 2006; 103: 17891-6.
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 17891-17896
-
-
Roxo-Rosa, M.1
Xu, Z.2
Schmidt, A.3
-
28
-
-
0033166350
-
Removal of multiple arginineframed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis
-
Chang X, Cui L, Hou Y, et al. Removal of multiple arginineframed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis. Mol Cell 1999; 4: 137-42.
-
(1999)
Mol Cell
, vol.4
, pp. 137-142
-
-
Chang, X.1
Cui, L.2
Hou, Y.3
-
29
-
-
5444220240
-
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code
-
Wang X, Matteson J, An Y, et al. COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. J Cell Biol 2004; 167: 65-74.
-
(2004)
J Cell Biol
, vol.167
, pp. 65-74
-
-
Wang, X.1
Matteson, J.2
An, Y.3
-
30
-
-
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
-
31
-
-
78149270037
-
The cystic fibrosiscausing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis
-
Thibodeau PH, Richardson JM, Wang W, et al. The cystic fibrosiscausing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis. J Biol Chem 2010; 285: 35825-35.
-
(2010)
J Biol Chem
, vol.285
, pp. 35825-35835
-
-
Thibodeau, P.H.1
Richardson, J.M.2
Wang, W.3
-
32
-
-
33746675669
-
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator
-
Younger JM, Chen L, Ren HY, et al. Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator. Cell 2006; 126: 571-82.
-
(2006)
Cell
, vol.126
, pp. 571-582
-
-
Younger, J.M.1
Chen, L.2
Ren, H.Y.3
-
33
-
-
0029997424
-
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
-
Qu BH, Thomas PJ. Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway. J Biol Chem 1996; 271: 7261-4.
-
(1996)
J Biol Chem
, vol.271
, pp. 7261-7264
-
-
Qu, B.H.1
Thomas, P.J.2
-
34
-
-
38349050413
-
Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation
-
Pissarra LS, Farinha CM, Xu Z, et al. Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chem Biol 2008; 15: 62-9.
-
(2008)
Chem Biol
, vol.15
, pp. 62-69
-
-
Pissarra, L.S.1
Farinha, C.M.2
Xu, Z.3
-
35
-
-
70449701828
-
Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone
-
Scott-Ward TS, Amaral MD. Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone. FEBS J 2009; 276: 7097-109.
-
(2009)
FEBS J
, vol.276
, pp. 7097-7109
-
-
Scott-Ward, T.S.1
Amaral, M.D.2
-
36
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation
-
Dalemans W, Barbry P, Champigny G, et al. Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation. Nature 1991; 354: 526-8.
-
(1991)
Nature
, vol.354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
-
37
-
-
42149120706
-
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
-
Serohijos AW, Hegedus T, Aleksandrov AA, et al. Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function. Proc Natl Acad Sci USA 2008; 105: 3256-61.
-
(2008)
Proc Natl Acad Sci USA
, vol.105
, pp. 3256-3261
-
-
Serohijos, A.W.1
Hegedus, T.2
Aleksandrov, A.A.3
-
38
-
-
77955025743
-
The V510D Suppressor Mutation Stabilizes DeltaF508-CFTR at the Cell Surface
-
Loo TW, Bartlett MC, Clarke DM. The V510D Suppressor Mutation Stabilizes DeltaF508-CFTR at the Cell Surface. Biochemistry 2010; 49: 6352.
-
(2010)
Biochemistry
, vol.49
, pp. 6352
-
-
Loo, T.W.1
Bartlett, M.C.2
Clarke, D.M.3
-
39
-
-
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
-
40
-
-
0035937847
-
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments
-
Sharma M, Benharouga M, Hu W, Lukacs GL. Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments. J Biol Chem 2001; 276: 8942-50.
-
(2001)
J Biol Chem
, vol.276
, pp. 8942-8950
-
-
Sharma, M.1
Benharouga, M.2
Hu, W.3
Lukacs, G.L.4
-
41
-
-
0036714504
-
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications
-
Gentzsch M, Aleksandrov A, Aleksandrov L, Riordan JR. Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications. Biochem J 2002; 366: 541-8.
-
(2002)
Biochem J
, vol.366
, pp. 541-548
-
-
Gentzsch, M.1
Aleksandrov, A.2
Aleksandrov, L.3
Riordan, J.R.4
-
42
-
-
27744482654
-
The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells
-
Swiatecka-Urban A, Brown A, Moreau-Marquis S, et al. The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells. J Biol Chem 2005; 280: 36762-72.
-
(2005)
J Biol Chem
, vol.280
, pp. 36762-36772
-
-
Swiatecka-Urban, A.1
Brown, A.2
Moreau-Marquis, S.3
-
43
-
-
12144287602
-
Misfolding diverts CFTR from recycling to degradation: Quality control at early endosomes
-
Sharma M, Pampinella F, Nemes C, et al. Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes. J Cell Biol 2004; 164: 923-33.
-
(2004)
J Cell Biol
, vol.164
, pp. 923-933
-
-
Sharma, M.1
Pampinella, F.2
Nemes, C.3
-
44
-
-
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
-
45
-
-
0029785795
-
A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo
-
French PJ, van Doorninck JH, Peters RH, et al. A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo. J Clin Invest. 1996; 98: 1304-12.
-
(1996)
J Clin Invest.
, vol.98
, pp. 1304-1312
-
-
French, P.J.1
van Doorninck, J.H.2
Peters, R.H.3
-
46
-
-
0025076114
-
Empty MHC class I molecules come out in the cold
-
Ljunggren HG, Stam NJ, Ohlen C, et al. Empty MHC class I molecules come out in the cold. Nature 1990; 346: 476-80.
-
(1990)
Nature
, vol.346
, pp. 476-480
-
-
Ljunggren, H.G.1
Stam, N.J.2
Ohlen, C.3
-
47
-
-
0034697167
-
A common temperature-sensitive allelic form of human tyrosinase is retained in the endoplasmic reticulum at the nonpermissive temperature
-
Berson JF, Frank DW, Calvo PA, Bieler BM, Marks MS. A common temperature-sensitive allelic form of human tyrosinase is retained in the endoplasmic reticulum at the nonpermissive temperature. J Biol Chem 2000; 275: 12281-9.
-
(2000)
J Biol Chem
, vol.275
, pp. 12281-12289
-
-
Berson, J.F.1
Frank, D.W.2
Calvo, P.A.3
Bieler, B.M.4
Marks, M.S.5
-
48
-
-
22544453614
-
Low temperature completely rescues the function of two misfolded K ATP channel disease-mutants
-
Yang K, Fang K, Fromondi L, Chan KW. Low temperature completely rescues the function of two misfolded K ATP channel disease-mutants. FEBS Lett 2005; 579: 4113-8.
-
(2005)
FEBS Lett
, vol.579
, pp. 4113-4118
-
-
Yang, K.1
Fang, K.2
Fromondi, L.3
Chan, K.W.4
-
49
-
-
0035000631
-
The role of chaperone-assisted folding and quality control in inborn errors of metabolism: Protein folding disorders
-
Gregersen N, Bross P, Andrese BS, Pedersen CB, Corydon TJ, Bolund L. The role of chaperone-assisted folding and quality control in inborn errors of metabolism: protein folding disorders. Journal of inherited metabolic disease. J Inherit Metab Dis 2001; 24: 189-212.
-
(2001)
Journal of inherited metabolic disease. J Inherit Metab Dis
, vol.24
, pp. 189-212
-
-
Gregersen, N.1
Bross, P.2
Andrese, B.S.3
Pedersen, C.B.4
Corydon, T.J.5
Bolund, L.6
-
50
-
-
0030046574
-
In vitro folding of inclusion body proteins
-
Rudolph R, Lilie H. In vitro folding of inclusion body proteins. FASEB J 1996; 10: 49-56.
-
(1996)
FASEB J
, vol.10
, pp. 49-56
-
-
Rudolph, R.1
Lilie, H.2
-
51
-
-
33846020543
-
Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs
-
Arakawa T, Ejima D, Kita Y, Tsumoto K. Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs. Biochim Biophys Acta 2006; 1764: 1677-87.
-
(2006)
Biochim Biophys Acta
, vol.1764
, pp. 1677-1687
-
-
Arakawa, T.1
Ejima, D.2
Kita, Y.3
Tsumoto, K.4
-
52
-
-
2542480784
-
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator
-
Gentzsch M, Chang XB, Cui L, et al. Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator. Mol Biol Cell 2004; 15: 2684-96.
-
(2004)
Mol Biol Cell
, vol.15
, pp. 2684-2696
-
-
Gentzsch, M.1
Chang, X.B.2
Cui, L.3
-
53
-
-
41149113942
-
Enhanced cellsurface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
-
Varga K, Goldstein RF, Jurkuvenaite A, et al. Enhanced cellsurface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochem J 2008; 410: 555-64.
-
(2008)
Biochem J
, vol.410
, pp. 555-564
-
-
Varga, K.1
Goldstein, R.F.2
Jurkuvenaite, A.3
-
55
-
-
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
-
56
-
-
80053198436
-
Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling
-
Sondo E, Tomati V, Caci E, et al. Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling. Am J Physiol Cell Physiol 2011; 301: C872-85.
-
(2011)
Am J Physiol Cell Physiol
, vol.301
, pp. 872-885
-
-
Sondo, E.1
Tomati, V.2
Caci, E.3
-
57
-
-
70449521295
-
Low temperature restoring effect on F508del-CFTR misprocessing: A proteomic approach
-
Gomes-Alves P, Neves S, Coelho AV, Penque D. Low temperature restoring effect on F508del-CFTR misprocessing: A proteomic approach. J Proteomics 2009; 73: 218-30.
-
(2009)
J Proteomics
, vol.73
, pp. 218-230
-
-
Gomes-Alves, P.1
Neves, S.2
Coelho, A.V.3
Penque, D.4
-
58
-
-
37549035927
-
Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer
-
Berdiev BK, Cormet-Boyaka E, Tousson A, et al. Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer. J Biol Chem 2007; 282: 36481-8.
-
(2007)
J Biol Chem
, vol.282
, pp. 36481-36488
-
-
Berdiev, B.K.1
Cormet-Boyaka, E.2
Tousson, A.3
-
59
-
-
84860869149
-
Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC)
-
Qadri YJ, Cormet-Boyaka E, Rooj AK, et al. Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC). J Biol Chem 2012; 287: 16781-90.
-
(2012)
J Biol Chem
, vol.287
, pp. 16781-16790
-
-
Qadri, Y.J.1
Cormet-Boyaka, E.2
Rooj, A.K.3
-
60
-
-
0027153083
-
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast
-
Teem JL, Berger HA, Ostedgaard LS, Rich DP, Tsui LC, Welsh MJ. Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast. Cell 1993; 73: 335-46.
-
(1993)
Cell
, vol.73
, pp. 335-346
-
-
Teem, J.L.1
Berger, H.A.2
Ostedgaard, L.S.3
Rich, D.P.4
Tsui, L.C.5
Welsh, M.J.6
-
61
-
-
0029864612
-
Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing
-
Teem JL, Carson MR, Welsh MJ. Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing. Recept Channels 1996; 4: 63-72.
-
(1996)
Recept Channels
, vol.4
, pp. 63-72
-
-
Teem, J.L.1
Carson, M.R.2
Welsh, M.J.3
-
62
-
-
0031006695
-
Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding
-
Qu BH, Strickland EH, Thomas PJ. Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding. J Biol Chem 1997; 272: 15739-44.
-
(1997)
J Biol Chem
, vol.272
, pp. 15739-15744
-
-
Qu, B.H.1
Strickland, E.H.2
Thomas, P.J.3
-
63
-
-
0037184104
-
Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508
-
DeCarvalho AC, Gansheroff LJ, Teem JL. Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508. J Biol Chem 2002; 277: 35896-905.
-
(2002)
J Biol Chem
, vol.277
, pp. 35896-35905
-
-
DeCarvalho, A.C.1
Gansheroff, L.J.2
Teem, J.L.3
-
64
-
-
0033103174
-
A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels
-
Zerangue N, Schwappach B, Jan YN, Jan LY. A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels. Neuron 1999; 22: 537-48.
-
(1999)
Neuron
, vol.22
, pp. 537-548
-
-
Zerangue, N.1
Schwappach, B.2
Jan, Y.N.3
Jan, L.Y.4
-
65
-
-
33745240417
-
F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive
-
Hegedus T, Aleksandrov A, Cui L, Gentzsch M, Chang XB, Riordan JR. F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive. Biochim Biophys Acta 2006; 1758: 565-72.
-
(2006)
Biochim Biophys Acta
, vol.1758
, pp. 565-572
-
-
Hegedus, T.1
Aleksandrov, A.2
Cui, L.3
Gentzsch, M.4
Chang, X.B.5
Riordan, J.R.6
-
66
-
-
65749107939
-
Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs
-
Kim Chiaw P, Huan LJ, Gagnon S, et al. Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs. Chem Biol 2009; 16: 520-30.
-
(2009)
Chem Biol
, vol.16
, pp. 520-530
-
-
Kim Chiaw, P.1
Huan, L.J.2
Gagnon, S.3
-
67
-
-
10744230777
-
Structure of nucleotidebinding domain 1 of the cystic fibrosis transmembrane conductance regulator
-
Lewis HA, Buchanan SG, Burley SK, et al. Structure of nucleotidebinding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J 2004; 23: 282-93.
-
(2004)
EMBO J
, vol.23
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
-
68
-
-
19944432524
-
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure
-
Lewis HA, Zhao X, Wang C, et al. Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J Biol Chem 2005; 280: 1346-53.
-
(2005)
J Biol Chem
, vol.280
, pp. 1346-1353
-
-
Lewis, H.A.1
Zhao, X.2
Wang, C.3
-
70
-
-
0030154323
-
Influence of molecular and chemical chaperones on protein folding
-
Welch WJ, Brown CR. Influence of molecular and chemical chaperones on protein folding. Cell Stress Chaperones 1996; 1: 109-15.
-
(1996)
Cell Stress Chaperones
, vol.1
, pp. 109-115
-
-
Welch, W.J.1
Brown, C.R.2
-
71
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein
-
Brown CR, Hong-Brown LQ, Biwersi J, Verkman AS, Welch WJ. Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1996; 1: 117-25.
-
(1996)
Cell Stress Chaperones
, vol.1
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
Verkman, A.S.4
Welch, W.J.5
-
72
-
-
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
-
74
-
-
0034815866
-
Partial restoration of defective chloride conductance in DeltaF508 CF mice by trimethylamine oxide
-
Fischer H, Fukuda N, Barbry P, Illek B, Sartori C, Matthay MA. Partial restoration of defective chloride conductance in DeltaF508 CF mice by trimethylamine oxide. Am J Physiol Lung Cell Mol Physiol 2001; 281: L52-7.
-
(2001)
Am J Physiol Lung Cell Mol Physiol
, vol.281
, pp. 52-57
-
-
Fischer, H.1
Fukuda, N.2
Barbry, P.3
Illek, B.4
Sartori, C.5
Matthay, M.A.6
-
75
-
-
77955637643
-
Cell Biology. The proteome in balance
-
Hutt D, Balch WE. Cell Biology. The proteome in balance. Science 2010; 329: 766-7.
-
(2010)
Science
, vol.329
, pp. 766-767
-
-
Hutt, D.1
Balch, W.E.2
-
76
-
-
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
-
77
-
-
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
-
78
-
-
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
-
79
-
-
24644464284
-
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening
-
Pedemonte N, Lukacs GL, Du K, et al. Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 2005; 115: 2564-71.
-
(2005)
J Clin Invest
, vol.115
, pp. 2564-2571
-
-
Pedemonte, N.1
Lukacs, G.L.2
Du, K.3
-
80
-
-
33744831154
-
Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F, Straley KS, Cao D, et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006; 290: L1117-30.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
, pp. 1117-1130
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
-
81
-
-
0036846329
-
5% of normal CFTR mRNA ameliorates the severity of pulmonary disease in Cystic Fibrosis
-
Ramalho AS, Beck S, Meyer M, Penque D, Cutting G, Amaral MD. 5% of normal CFTR mRNA ameliorates the severity of pulmonary disease in Cystic Fibrosis. Am J Respir Cell Mol Biol 2002; 27: 619.
-
(2002)
Am J Respir Cell Mol Biol
, vol.27
, pp. 619
-
-
Ramalho, A.S.1
Beck, S.2
Meyer, M.3
Penque, D.4
Cutting, G.5
Amaral, M.D.6
-
82
-
-
27144481548
-
Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound
-
Loo TW, Bartlett MC, Clarke DM. Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound. Mol Pharm 2005; 2: 407-13.
-
(2005)
Mol Pharm
, vol.2
, pp. 407-413
-
-
Loo, T.W.1
Bartlett, M.C.2
Clarke, D.M.3
-
83
-
-
33645530653
-
The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants
-
Loo TW, Bartlett MC, Wang Y, Clarke DM. The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants. Biochem J 2006; 395: 537-42.
-
(2006)
Biochem J
, vol.395
, pp. 537-542
-
-
Loo, T.W.1
Bartlett, M.C.2
Wang, Y.3
Clarke, D.M.4
-
84
-
-
77956237958
-
A chemical corrector modifies the channel function of F508del-CFTR
-
Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE. A chemical corrector modifies the channel function of F508del-CFTR. Mol Pharmacol 2010; 78: 411-8.
-
(2010)
Mol Pharmacol
, vol.78
, pp. 411-418
-
-
Kim Chiaw, P.1
Wellhauser, L.2
Huan, L.J.3
Ramjeesingh, M.4
Bear, C.E.5
-
85
-
-
33745282127
-
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones
-
Wang Y, Bartlett MC, Loo TW, Clarke DM. Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones. Mol Pharmacol 2006; 70: 297-302.
-
(2006)
Mol Pharmacol
, vol.70
, pp. 297-302
-
-
Wang, Y.1
Bartlett, M.C.2
Loo, T.W.3
Clarke, D.M.4
-
86
-
-
33745282127
-
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones
-
Wang Y, Bartlett MC, Loo TW, Clarke DM. Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones. Mol Pharmacol 2006; 70: 297-302.
-
(2006)
Mol Pharmacol
, vol.70
, pp. 297-302
-
-
Wang, Y.1
Bartlett, M.C.2
Loo, T.W.3
Clarke, D.M.4
-
87
-
-
66849129301
-
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability
-
Wellhauser L, Kim Chiaw P, Pasyk S, Li C, Ramjeesingh M, Bear CE. A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Mol Pharmacol 2009; 75: 1430-8.
-
(2009)
Mol Pharmacol
, vol.75
, pp. 1430-1438
-
-
Wellhauser, L.1
Kim Chiaw, P.2
Pasyk, S.3
Li, C.4
Ramjeesingh, M.5
Bear, C.E.6
-
88
-
-
34548154971
-
Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants
-
Wang Y, Loo TW, Bartlett MC, Clarke DM. Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants. Biochem J 2007; 406: 257-63.
-
(2007)
Biochem J
, vol.406
, pp. 257-263
-
-
Wang, Y.1
Loo, T.W.2
Bartlett, M.C.3
Clarke, D.M.4
-
89
-
-
38549125726
-
Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect
-
Robert R, Carlile GW, Pavel C, et al. Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect. Mol Pharmacol 2008; 73: 478-89.
-
(2008)
Mol Pharmacol
, vol.73
, pp. 478-489
-
-
Robert, R.1
Carlile, G.W.2
Pavel, C.3
-
90
-
-
11944265976
-
Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis
-
Dormer RL, Harris CM, Clark Z, et al. Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis. Thorax 2005; 60: 55-9.
-
(2005)
Thorax
, vol.60
, pp. 55-59
-
-
Dormer, R.L.1
Harris, C.M.2
Clark, Z.3
-
91
-
-
84455208061
-
Corrector-mediated rescue of misprocessed CFTR mutants can be reduced by the Pglycoprotein drug pump
-
Loo TW, Bartlett MC, Shi L, Clarke DM. Corrector-mediated rescue of misprocessed CFTR mutants can be reduced by the Pglycoprotein drug pump. Biochem Pharmacol 2012; 83: 345-54.
-
(2012)
Biochem Pharmacol
, vol.83
, pp. 345-354
-
-
Loo, T.W.1
Bartlett, M.C.2
Shi, L.3
Clarke, D.M.4
-
92
-
-
84861354967
-
Sildenafil acts as potentiator and corrector of CFTR but might be not suitable for the treatment of CF lung disease
-
Leier G, Bangel-Ruland N, Sobczak K, Knieper Y, Weber WM. Sildenafil acts as potentiator and corrector of CFTR but might be not suitable for the treatment of CF lung disease. Cell Physiol Biochem 2012; 29: 775-90.
-
(2012)
Cell Physiol Biochem
, vol.29
, pp. 775-790
-
-
Leier, G.1
Bangel-Ruland, N.2
Sobczak, K.3
Knieper, Y.4
Weber, W.M.5
-
93
-
-
41849133213
-
4'-Methyl-4,5'-bithiazole-based correctors of defective delta F508-CFTR cellular processing
-
Yoo CL, Yu GJ, Yang B, Robins LI, Verkman AS, Kurth MJ. 4'-Methyl-4,5'-bithiazole-based correctors of defective delta F508-CFTR cellular processing. Bioorg Med Chem Lett 2008; 18: 2610-4.
-
(2008)
Bioorg Med Chem Lett
, vol.18
, pp. 2610-2614
-
-
Yoo, C.L.1
Yu, G.J.2
Yang, B.3
Robins, L.I.4
Verkman, A.S.5
Kurth, M.J.6
-
94
-
-
53549097399
-
Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy
-
Yu GJ, Yoo CL, Yang B, et al. Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy. J Med Chem 2008; 51: 6044-54.
-
(2008)
J Med Chem
, vol.51
, pp. 6044-6054
-
-
Yu, G.J.1
Yoo, C.L.2
Yang, B.3
-
95
-
-
72049120101
-
Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTR
-
Mills AD, Yoo C, Butler JD, Yang B, Verkman AS, Kurth MJ. Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTR. Bioorg Med Chem Lett 2010; 20: 87-91.
-
(2010)
Bioorg Med Chem Lett
, vol.20
, pp. 87-91
-
-
Mills, A.D.1
Yoo, C.2
Butler, J.D.3
Yang, B.4
Verkman, A.S.5
Kurth, M.J.6
-
96
-
-
78651264296
-
Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening
-
Kalid O, Mense M, Fischman S, et al. Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening. J Comput Aided Mol Des 2010; 24: 971-91.
-
(2010)
J Comput Aided Mol Des
, vol.24
, pp. 971-991
-
-
Kalid, O.1
Mense, M.2
Fischman, S.3
-
97
-
-
79955368563
-
Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations
-
Pedemonte N, Tomati V, Sondo E, et al. Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations. J Biol Chem 2011; 286: 15215-26.
-
(2011)
J Biol Chem
, vol.286
, pp. 15215-15226
-
-
Pedemonte, N.1
Tomati, V.2
Sondo, E.3
-
98
-
-
77249138830
-
Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells
-
Jurkuvenaite A, Chen L, Bartoszewski R, et al. Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. Am J Resp Cell Mol Biol 2010; 42: 363-72.
-
(2010)
Am J Resp Cell Mol Biol
, vol.42
, pp. 363-372
-
-
Jurkuvenaite, A.1
Chen, L.2
Bartoszewski, R.3
-
99
-
-
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 USA 2011; 108: 18843-8.
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, pp. 18843-18848
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
100
-
-
77954983869
-
Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR
-
He L, Aleksandrov LA, Cui L, Jensen TJ, Nesbitt KL, Riordan JR. Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR. FASEB J 2010; 24: 3103-12.
-
(2010)
FASEB J
, vol.24
, pp. 3103-3112
-
-
He, L.1
Aleksandrov, L.A.2
Cui, L.3
Jensen, T.J.4
Nesbitt, K.L.5
Riordan, J.R.6
-
101
-
-
84862909346
-
Requirements for efficient correction of DeltaF508 CFTR revealed by analyses of evolved sequences
-
Mendoza JL, Schmidt A, Li Q, et al. Requirements for efficient correction of DeltaF508 CFTR revealed by analyses of evolved sequences. Cell 2012; 148: 164-74.
-
(2012)
Cell
, vol.148
, pp. 164-174
-
-
Mendoza, J.L.1
Schmidt, A.2
Li, Q.3
-
102
-
-
84862908028
-
Correction of both NBD1 energetics and domain interface is required to restore DeltaF508 CFTR folding and function
-
Rabeh WM, Bossard F, Xu H, et al. Correction of both NBD1 energetics and domain interface is required to restore DeltaF508 CFTR folding and function. Cell 2012; 148: 150-63.
-
(2012)
Cell
, vol.148
, pp. 150-163
-
-
Rabeh, W.M.1
Bossard, F.2
Xu, H.3
-
103
-
-
77953122968
-
DeltaF508 CFTR processing correction and activity in polarized airway and nonairway cell monolayers
-
Rowe SM, Pyle LC, Jurkevante A, et al. DeltaF508 CFTR processing correction and activity in polarized airway and nonairway cell monolayers. Pulm Pharmacol Ther 2010; 23: 268-78.
-
(2010)
Pulm Pharmacol Ther
, vol.23
, pp. 268-278
-
-
Rowe, S.M.1
Pyle, L.C.2
Jurkevante, A.3
-
105
-
-
80052327898
-
Use of primary cultures of human bronchial epithelial cells isolated from cystic fibrosis patients for the pre-clinical testing of CFTR modulators
-
Neuberger T, Burton B, Clark H, Van Goor F. Use of primary cultures of human bronchial epithelial cells isolated from cystic fibrosis patients for the pre-clinical testing of CFTR modulators. Methods Mol Biol 2011; 741: 39-54.
-
(2011)
Methods Mol Biol
, vol.741
, pp. 39-54
-
-
Neuberger, T.1
Burton, B.2
Clark, H.3
Van Goor, F.4
-
107
-
-
5144232620
-
CFTR Cl-channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis
-
Hirtz S, Gonska T, Seydewitz HH, et al. CFTR Cl-channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology 2004; 127: 1085-95.
-
(2004)
Gastroenterology
, vol.127
, pp. 1085-1095
-
-
Hirtz, S.1
Gonska, T.2
Seydewitz, H.H.3
-
108
-
-
84867641604
-
Measurements of CFTR-Mediated Cl(-) Secretion in Human Rectal Biopsies Constitute a Robust Biomarker for Cystic Fibrosis Diagnosis and Prognosis
-
Sousa M, Servidoni MF, Vinagre AM, et al. Measurements of CFTR-Mediated Cl(-) Secretion in Human Rectal Biopsies Constitute a Robust Biomarker for Cystic Fibrosis Diagnosis and Prognosis. PLoS One 2012; 7: e47708.
-
(2012)
PLoS One
, vol.7
-
-
Sousa, M.1
Servidoni, M.F.2
Vinagre, A.M.3
-
109
-
-
80052328287
-
The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients
-
Roth EK, Hirtz S, Duerr J, et al. The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients. PLoS One 2011; 6: e24445.
-
(2011)
PLoS One
, vol.6
-
-
Roth, E.K.1
Hirtz, S.2
Duerr, J.3
-
110
-
-
0036258208
-
Cystic fibrosis: A worldwide analysis of CFTR mutations--correlation with incidence data and application to screening
-
Bobadilla JL, Macek M, Jr., Fine JP, Farrell PM. Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening. Hum Mutat 2002; 19: 575-606.
-
(2002)
Hum Mutat
, vol.19
, pp. 575-606
-
-
Bobadilla, J.L.1
Macek Jr., M.2
Fine, J.P.3
Farrell, P.M.4
-
111
-
-
0242363119
-
Pharmacological approaches to correcting the ion transport defect in cystic fibrosis
-
Roomans GM. Pharmacological approaches to correcting the ion transport defect in cystic fibrosis. Am J Respir Med 2003; 2: 413-31.
-
(2003)
Am J Respir Med
, vol.2
, pp. 413-431
-
-
Roomans, G.M.1
-
112
-
-
0033153764
-
Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX
-
Schultz BD, Frizzell RA, Bridges RJ. Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX. J Membr Biol 1999; 170: 51-66.
-
(1999)
J Membr Biol
, vol.170
, pp. 51-66
-
-
Schultz, B.D.1
Frizzell, R.A.2
Bridges, R.J.3
-
113
-
-
33744831154
-
Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van GF, Straley KS, Cao D, et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006; 290: L1117-30.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
, pp. 1117-1130
-
-
Van, G.F.1
Straley, K.S.2
Cao, D.3
-
114
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
Van Goor F, Hadida S, Grootenhuis PD, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci USA 2009; 106: 18825-30.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 18825-18830
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
115
-
-
78549279173
-
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation
-
Accurso FJ, Rowe SM, Clancy JP, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010; 363: 1991-2003.
-
(2010)
N Engl J Med
, vol.363
, pp. 1991-2003
-
-
Accurso, F.J.1
Rowe, S.M.2
Clancy, J.P.3
-
116
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-72.
-
(2011)
N Engl J Med
, vol.365
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
-
117
-
-
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
-
118
-
-
70450231758
-
Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients
-
Ramalho AS, Lewandowska MA, Farinha CM, et al. Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients. Cell Physiol Biochem 2009; 24: 335-46.
-
(2009)
Cell Physiol Biochem
, vol.24
, pp. 335-346
-
-
Ramalho, A.S.1
Lewandowska, M.A.2
Farinha, C.M.3
-
119
-
-
84893036735
-
Mechanism of Rac1 signaling evidences how to enhance chemical correction of misfolded CFTR through HGF for treatment of Cystic Fibrosis
-
[E-pub ahead of print]
-
Moniz S, Sousa M, Moraes B, et al. Mechanism of Rac1 signaling evidences how to enhance chemical correction of misfolded CFTR through HGF for treatment of Cystic Fibrosis. ACS Chem Biol 2012; [E-pub ahead of print].
-
(2012)
ACS Chem Biol
-
-
Moniz, S.1
Sousa, M.2
Moraes, B.3
-
120
-
-
15944387059
-
Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport
-
Hirth BH, Qiao S, Cuff LM, et al. Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport. Bioorg Med Chem Lett 2005; 15: 2087-91.
-
(2005)
Bioorg Med Chem Lett
, vol.15
, pp. 2087-2091
-
-
Hirth, B.H.1
Qiao, S.2
Cuff, L.M.3
-
121
-
-
33646892646
-
Dynasore, a cell-permeable inhibitor of dynamin
-
Macia E, Ehrlich M, Massol R, Boucrot E, Brunner C, Kirchhausen T. Dynasore, a cell-permeable inhibitor of dynamin. Dev Cell 2006; 10: 839-50.
-
(2006)
Dev Cell
, vol.10
, pp. 839-850
-
-
Macia, E.1
Ehrlich, M.2
Massol, R.3
Boucrot, E.4
Brunner, C.5
Kirchhausen, T.6
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