-
1
-
-
33645130994
-
Cystic fibrosis since 1938
-
Davis PB Cystic fibrosis since 1938. Am J Respir Crit Care Med 2006, 173:475-482.
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 475-482
-
-
Davis, P.B.1
-
2
-
-
33847711906
-
Cystic fibrosis mortality and survival in the UK: 1947-2003
-
Dodge JA, Lewis PA, Stanton M, Wilsher J Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 2007, 29:522-526.
-
(2007)
Eur Respir J
, vol.29
, pp. 522-526
-
-
Dodge, J.A.1
Lewis, P.A.2
Stanton, M.3
Wilsher, J.4
-
3
-
-
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, 245:1066-1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
-
4
-
-
0032912589
-
Structure and function of the CFTR chloride channel
-
Sheppard DN, Welsh MJ Structure and function of the CFTR chloride channel. Physiol Rev 1999, 79(1 Suppl):S23-S45.
-
(1999)
Physiol Rev
, vol.79
, Issue.1 SUPPL.
-
-
Sheppard, D.N.1
Welsh, M.J.2
-
5
-
-
0032956071
-
CFTR is a conductance regulator as well as a chloride channel
-
Schwiebert EM, Benos DJ, Egan ME, Stutts MJ, Guggino WB CFTR is a conductance regulator as well as a chloride channel. Physiol Rev 1999, 79(1 Suppl):S 145-S 166.
-
(1999)
Physiol Rev
, vol.79
, Issue.1 SUPPL.
-
-
Schwiebert, E.M.1
Benos, D.J.2
Egan, M.E.3
Stutts, M.J.4
Guggino, W.B.5
-
6
-
-
0001752544
-
Cystic Fibrosis
-
McGraw-Hill Inc., New York, C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.)
-
Welsh MJ, Ramsey BW, Accurso F, Cutting GR Cystic Fibrosis. The Metabolic and Molecular Basis of Inherited Disease 2001, 5121-5188. McGraw-Hill Inc., New York. C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.).
-
(2001)
The Metabolic and Molecular Basis of Inherited Disease
, pp. 5121-5188
-
-
Welsh, M.J.1
Ramsey, B.W.2
Accurso, F.3
Cutting, G.R.4
-
8
-
-
79958107503
-
Gene and cell therapy for cystic fibrosis: From bench to bedside
-
Conese M, Ascenzioni F, Boyd AC, et al. Gene and cell therapy for cystic fibrosis: From bench to bedside. J Cyst Fibros 2011, 10(Suppl 2):114-128.
-
(2011)
J Cyst Fibros
, vol.10
, Issue.SUPPL. 2
, pp. 114-128
-
-
Conese, M.1
Ascenzioni, F.2
Boyd, A.C.3
-
9
-
-
0027162649
-
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
-
Welsh MJ, Smith AE Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 1993, 73:1251-1254.
-
(1993)
Cell
, vol.73
, pp. 1251-1254
-
-
Welsh, M.J.1
Smith, A.E.2
-
10
-
-
0029616734
-
Cystic fibrosis: genotypic and phenotypic variations
-
Zielenski J, Tsui LC Cystic fibrosis: genotypic and phenotypic variations. Annu Rev Genet 1995, 29:777-807.
-
(1995)
Annu Rev Genet
, vol.29
, pp. 777-807
-
-
Zielenski, J.1
Tsui, L.C.2
-
11
-
-
18844384936
-
Processing of CFTR: traversing the cellular maze - how much CFTR needs to go through to avoid cystic fibrosis?
-
Amaral MD Processing of CFTR: traversing the cellular maze - how much CFTR needs to go through to avoid cystic fibrosis?. Pediatr Pulmonol 2005, 39:479-491.
-
(2005)
Pediatr Pulmonol
, vol.39
, pp. 479-491
-
-
Amaral, M.D.1
-
12
-
-
65749102092
-
- channel by ATP-driven nucleotide-binding domain dimerisation
-
- channel by ATP-driven nucleotide-binding domain dimerisation. J Physiol 2009, 587:2151-2161.
-
(2009)
J Physiol
, vol.587
, pp. 2151-2161
-
-
Hwang, T.C.1
Sheppard, D.N.2
-
14
-
-
0027423190
-
Multi-ion pore behaviour in the CFTR chloride channel
-
Tabcharani JA, Rommens JM, Hou YX, et al. Multi-ion pore behaviour in the CFTR chloride channel. Nature 1993, 366:79-82.
-
(1993)
Nature
, vol.366
, pp. 79-82
-
-
Tabcharani, J.A.1
Rommens, J.M.2
Hou, Y.X.3
-
15
-
-
0028929909
-
Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency
-
Sheppard DN, Ostedgaard LS, Winter MC, Welsh MJ Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency. EMBO J 1995, 14:876-883.
-
(1995)
EMBO J
, vol.14
, pp. 876-883
-
-
Sheppard, D.N.1
Ostedgaard, L.S.2
Winter, M.C.3
Welsh, M.J.4
-
16
-
-
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, 63:827-834.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
-
17
-
-
0026547330
-
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia
-
Denning GM, Ostedgaard LS, Cheng SH, Smith AE, Welsh MJ Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia. J Clin Invest 1992, 89:339-349.
-
(1992)
J Clin Invest
, vol.89
, pp. 339-349
-
-
Denning, G.M.1
Ostedgaard, L.S.2
Cheng, S.H.3
Smith, A.E.4
Welsh, M.J.5
-
18
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
-
Dalemans W, Barbry P, Champigny G, et al. Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation. Nature 1991, 354:526-528.
-
(1991)
Nature
, vol.354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
-
19
-
-
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, 358:761-764.
-
(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
-
20
-
-
34247588271
-
PTC124 targets genetic disorders caused by nonsense mutations
-
Welch EM, Barton ER, Zhuo J, et al. PTC124 targets genetic disorders caused by nonsense mutations. Nature 2007, 447:87-91.
-
(2007)
Nature
, vol.447
, pp. 87-91
-
-
Welch, E.M.1
Barton, E.R.2
Zhuo, J.3
-
21
-
-
50149098401
-
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial
-
Kerem E, Hirawat S, Armoni S, et al. Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. Lancet 2008, 372:719-727.
-
(2008)
Lancet
, vol.372
, pp. 719-727
-
-
Kerem, E.1
Hirawat, S.2
Armoni, S.3
-
22
-
-
33645211759
-
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the a-glucosidase inhibitor miglustat
-
Norez C, Noel S, Wilke M, et al. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the a-glucosidase inhibitor miglustat. FEBS Lett 2006, 580:2081-2086.
-
(2006)
FEBS Lett
, vol.580
, pp. 2081-2086
-
-
Norez, C.1
Noel, S.2
Wilke, M.3
-
23
-
-
44249097535
-
Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells
-
Noël S, Wilke M, Bot AG, De Jonge HR, Becq F Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. J Pharmacol Exp Ther 2008, 325:1016-1023.
-
(2008)
J Pharmacol Exp Ther
, vol.325
, pp. 1016-1023
-
-
Noël, S.1
Wilke, M.2
Bot, A.G.3
De Jonge, H.R.4
Becq, F.5
-
24
-
-
24644464284
-
Small-molecule correctors of defective ΔF508-CFTR cellular processing identified by high-throughput screening
-
Pedemonte N, Lukacs GL, Du K, et al. Small-molecule correctors of defective ΔF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 2005, 115:2564-2571.
-
(2005)
J Clin Invest
, vol.115
, pp. 2564-2571
-
-
Pedemonte, N.1
Lukacs, G.L.2
Du, K.3
-
25
-
-
33744831154
-
Rescue of ΔF508-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 ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006, 290:L1117-L1130.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
-
26
-
-
78650218064
-
Rescue of the protein folding defect in cystic fibrosis in vitro by the investigational small molecule, VX-809
-
Van Goor F, Hadida S, Grootenhuis PDJ, et al. Rescue of the protein folding defect in cystic fibrosis in vitro by the investigational small molecule, VX-809. J Cyst Fibros 2010, 9(Suppl 1):S14.
-
(2010)
J Cyst Fibros
, vol.9
, Issue.SUPPL. 1
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.J.3
-
27
-
-
78649471960
-
Clinical evaluation of VX-809, a novel investigational oral F508del-CFTR corrector, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
-
for the VX-809-101 Study Group
-
Clancy JP, Spencer-Green G, for the VX-809-101 Study Group Clinical evaluation of VX-809, a novel investigational oral F508del-CFTR corrector, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. J Cyst Fibros 2010, 9(Suppl 1):S20.
-
(2010)
J Cyst Fibros
, vol.9
, Issue.SUPPL. 1
-
-
Clancy, J.P.1
Spencer-Green, G.2
-
29
-
-
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
-
30
-
-
0028577602
-
Phosphatase inhibitors activate normal and defective CFTR chloride channels
-
Becq F, Jensen TJ, Chang XB, et al. Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc Natl Acad Sci U S A 1994, 91:9160-9164.
-
(1994)
Proc Natl Acad Sci U S A
, vol.91
, pp. 9160-9164
-
-
Becq, F.1
Jensen, T.J.2
Chang, X.B.3
-
31
-
-
0031881489
-
Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating: evidence for two binding sites with opposite effects
-
Wang F, Zeltwanger S, Yang IC, Nairn AC, Hwang TC Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating: evidence for two binding sites with opposite effects. J Gen Physiol 1998, 111:477-490.
-
(1998)
J Gen Physiol
, vol.111
, pp. 477-490
-
-
Wang, F.1
Zeltwanger, S.2
Yang, I.C.3
Nairn, A.C.4
Hwang, T.C.5
-
33
-
-
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 U S A 2009, 106:18825-18830.
-
(2009)
Proc Natl Acad Sci U S A
, vol.106
, pp. 18825-18830
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
34
-
-
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
-
36
-
-
14544300522
-
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains
-
Vergani P, Lockless SW, Nairn AC, Gadsby DC CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature 2005, 433:876-880.
-
(2005)
Nature
, vol.433
, pp. 876-880
-
-
Vergani, P.1
Lockless, S.W.2
Nairn, A.C.3
Gadsby, D.C.4
-
37
-
-
10744230777
-
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
-
Lewis HA, Buchanan SG, Burley SK, et al. Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J 2004, 23:282-293.
-
(2004)
EMBO J
, vol.23
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
-
38
-
-
0037013262
-
The first nucleotide binding domain of cystic fibrosis transmembrane conductance regulator is a site of stable nucleotide interaction, whereas the second is a site of rapid turnover
-
Aleksandrov L, Aleksandrov AA, Chang XB, Riordan JR The first nucleotide binding domain of cystic fibrosis transmembrane conductance regulator is a site of stable nucleotide interaction, whereas the second is a site of rapid turnover. J Biol Chem 2002, 277:15419-15425.
-
(2002)
J Biol Chem
, vol.277
, pp. 15419-15425
-
-
Aleksandrov, L.1
Aleksandrov, A.A.2
Chang, X.B.3
Riordan, J.R.4
-
39
-
-
2442669014
-
Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents
-
Ai T, Bompadre SG, Wang X, Hu S, Li M, Hwang TC Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents. Mol Pharmacol 2004, 65:1415-1426.
-
(2004)
Mol Pharmacol
, vol.65
, pp. 1415-1426
-
-
Ai, T.1
Bompadre, S.G.2
Wang, X.3
Hu, S.4
Li, M.5
Hwang, T.C.6
-
40
-
-
15044353957
-
Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains
-
Moran O, Galietta LJ, Zegarra-Moran O Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains. Cell Mol Life Sci 2005, 62:446-460.
-
(2005)
Cell Mol Life Sci
, vol.62
, pp. 446-460
-
-
Moran, O.1
Galietta, L.J.2
Zegarra-Moran, O.3
-
41
-
-
34247846123
-
Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators: interaction between activation and inhibition
-
Zegarra-Moran O, Monteverde M, Galietta LJ, Moran O Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators: interaction between activation and inhibition. J Biol Chem 2007, 282:9098-9104.
-
(2007)
J Biol Chem
, vol.282
, pp. 9098-9104
-
-
Zegarra-Moran, O.1
Monteverde, M.2
Galietta, L.J.3
Moran, O.4
-
42
-
-
58449116969
-
+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease
-
+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease. Exp Physiol 2009, 94:171-174.
-
(2009)
Exp Physiol
, vol.94
, pp. 171-174
-
-
Mall, M.A.1
-
44
-
-
0032940125
-
Pharmacology of CFTR chloride channel activity
-
Schultz BD, Singh AK, Devor DC, Bridges RJ Pharmacology of CFTR chloride channel activity. Physiol Rev 1999, 79(1 Suppl):S109-S144.
-
(1999)
Physiol Rev
, vol.79
, Issue.1 SUPPL.
-
-
Schultz, B.D.1
Singh, A.K.2
Devor, D.C.3
Bridges, R.J.4
-
45
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts MJ, Canessa CM, Olsen JC, et al. CFTR as a cAMP-dependent regulator of sodium channels. Science 1995, 269:847-850.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
-
46
-
-
0032127980
-
+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways
-
+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways. J Clin Invest 1998, 102:15-21.
-
(1998)
J Clin Invest
, vol.102
, pp. 15-21
-
-
Mall, M.1
Bleich, M.2
Greger, R.3
Schreiber, R.4
Kunzelmann, K.5
-
47
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
Matsui H, Grubb BR, Tarran R, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998, 95:1005-1015.
-
(1998)
Cell
, vol.95
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
-
48
-
-
0030970422
-
Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics
-
Stutts MJ, Rossier BC, Boucher RC Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics. J Biol Chem 1997, 272:14037-14040.
-
(1997)
J Biol Chem
, vol.272
, pp. 14037-14040
-
-
Stutts, M.J.1
Rossier, B.C.2
Boucher, R.C.3
-
49
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
Knowles MR, Stutts MJ, Spock A, Fischer N, Gatzy JT, Boucher RC Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 1983, 221:1067-1070.
-
(1983)
Science
, vol.221
, pp. 1067-1070
-
-
Knowles, M.R.1
Stutts, M.J.2
Spock, A.3
Fischer, N.4
Gatzy, J.T.5
Boucher, R.C.6
-
52
-
-
57149104917
-
Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in βENaC-overexpressing mice
-
Zhou Z, Treis D, Schubert SC, et al. Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in βENaC-overexpressing mice. Am J Respir Crit Care Med 2008, 178:1245-1256.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, pp. 1245-1256
-
-
Zhou, Z.1
Treis, D.2
Schubert, S.C.3
-
53
-
-
0025236867
-
A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
-
Knowles MR, Church NL, Waltner WE, et al. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 1990, 322:1189-1194.
-
(1990)
N Engl J Med
, vol.322
, pp. 1189-1194
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
-
54
-
-
0022988338
-
Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibröses
-
Köhler D, App E, Schmitz-Schumann M, Würtemberger G, Matthys H Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibröses. Eur J Respir Dis Suppl 1986, 146:319-326.
-
(1986)
Eur J Respir Dis Suppl
, vol.146
, pp. 319-326
-
-
Köhler, D.1
App, E.2
Schmitz-Schumann, M.3
Würtemberger, G.4
Matthys, H.5
-
55
-
-
0025274624
-
Acute and long-term amiloride inhalation in cystic fibrosis lung disease: a rational approach to cystic fibrosis therapy
-
App EM, King M, Helfesrieder R, Köhler D, Matthys H Acute and long-term amiloride inhalation in cystic fibrosis lung disease: a rational approach to cystic fibrosis therapy. Am Rev Respir Dis 1990, 141:605-612.
-
(1990)
Am Rev Respir Dis
, vol.141
, pp. 605-612
-
-
App, E.M.1
King, M.2
Helfesrieder, R.3
Köhler, D.4
Matthys, H.5
-
56
-
-
0027486593
-
No added benefit from nebulized amiloride in patients with cystic fibrosis
-
Graham A, Hasani A, Alton EW, et al. No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur Respir J 1993, 6:1243-1248.
-
(1993)
Eur Respir J
, vol.6
, pp. 1243-1248
-
-
Graham, A.1
Hasani, A.2
Alton, E.W.3
-
57
-
-
0028787073
-
Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial
-
Bowler IM, Kelman B, Worthington D, et al. Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial. Arch Dis Child 1995, 73:427-430.
-
(1995)
Arch Dis Child
, vol.73
, pp. 427-430
-
-
Bowler, I.M.1
Kelman, B.2
Worthington, D.3
-
58
-
-
0034047238
-
French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients: the Amiloride-AFLM Collaborative Study Group
-
Pons G, Marchand MC, d'Athis P, et al. French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients: the Amiloride-AFLM Collaborative Study Group. Pediatr Pulmonol 2000, 30:25-31.
-
(2000)
Pediatr Pulmonol
, vol.30
, pp. 25-31
-
-
Pons, G.1
Marchand, M.C.2
d'Athis, P.3
-
59
-
-
45949094270
-
Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models
-
Mall MA Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models. J Aerosol Med Pulm Drug Deliv 2008, 21:13-24.
-
(2008)
J Aerosol Med Pulm Drug Deliv
, vol.21
, pp. 13-24
-
-
Mall, M.A.1
-
60
-
-
0037027952
-
Altering airway surface liquid volume: inhalation therapy with amiloride and hyperosmotic agents
-
Hirsh AJ Altering airway surface liquid volume: inhalation therapy with amiloride and hyperosmotic agents. Adv Drug Deliv Rev 2002, 54:1445-1462.
-
(2002)
Adv Drug Deliv Rev
, vol.54
, pp. 1445-1462
-
-
Hirsh, A.J.1
-
61
-
-
41149164824
-
Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease
-
Hirsh AJ, Zhang J, Zamurs A, et al. Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease. J Pharmacol Exp Ther 2008, 325:77-88.
-
(2008)
J Pharmacol Exp Ther
, vol.325
, pp. 77-88
-
-
Hirsh, A.J.1
Zhang, J.2
Zamurs, A.3
-
62
-
-
56749173582
-
The guinea-pig trachéal potential difference as an in vivo model for the study of epithelial sodium channel function in the airways
-
Coote KJ, Atherton H, Young A, et al. The guinea-pig trachéal potential difference as an in vivo model for the study of epithelial sodium channel function in the airways. Br J Pharmacol 2008, 155:1025-1033.
-
(2008)
Br J Pharmacol
, vol.155
, pp. 1025-1033
-
-
Coote, K.J.1
Atherton, H.2
Young, A.3
-
63
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006, 354:241-250.
-
(2006)
N Engl J Med
, vol.354
, pp. 241-250
-
-
Donaldson, S.H.1
Bennett, W.D.2
Zeman, K.L.3
Knowles, M.R.4
Tarran, R.5
Boucher, R.C.6
-
66
-
-
0037040995
-
Regulation of the epithelial sodium channel by serine proteases in human airways
-
Donaldson SH, Hirsh A, Li DC, et al. Regulation of the epithelial sodium channel by serine proteases in human airways. J Biol Chem 2002, 277:8338-8345.
-
(2002)
J Biol Chem
, vol.277
, pp. 8338-8345
-
-
Donaldson, S.H.1
Hirsh, A.2
Li, D.C.3
-
67
-
-
65649148597
-
Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease
-
Coote K, Atherton-Watson HC, Sugar R, et al. Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease. J Pharmacol Exp Ther 2009, 329:764-774.
-
(2009)
J Pharmacol Exp Ther
, vol.329
, pp. 764-774
-
-
Coote, K.1
Atherton-Watson, H.C.2
Sugar, R.3
-
69
-
-
59149088885
-
Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA
-
Caci E, Melani R, Pedemonte N, et al. Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA. Am J Respir Cell Mol Biol 2009, 40:211-216.
-
(2009)
Am J Respir Cell Mol Biol
, vol.40
, pp. 211-216
-
-
Caci, E.1
Melani, R.2
Pedemonte, N.3
-
70
-
-
33646576255
-
RNA interference for a-ENaC inhibits rat lung fluid absorption in vivo
-
Li T, Folkesson HG RNA interference for a-ENaC inhibits rat lung fluid absorption in vivo. Am J Physiol Lung Cell Mol Physiol 2006, 290:L649-L660.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
-
-
Li, T.1
Folkesson, H.G.2
-
71
-
-
52649120753
-
RNA interference for CFTR attenuates lung fluid absorption at birth in rats
-
Li T, Koshy S, Folkesson HG RNA interference for CFTR attenuates lung fluid absorption at birth in rats. Respir Res 2008, 9:55.
-
(2008)
Respir Res
, vol.9
, pp. 55
-
-
Li, T.1
Koshy, S.2
Folkesson, H.G.3
-
72
-
-
33645382880
-
Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo
-
Griesenbach U, Kitson C, Escudero Garcia S, et al. Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo. Respir Res 2006, 7:26.
-
(2006)
Respir Res
, vol.7
, pp. 26
-
-
Griesenbach, U.1
Kitson, C.2
Escudero Garcia, S.3
-
73
-
-
0026651508
-
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia
-
Anderson MP, Sheppard DN, Berger HA, Welsh MJ Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am J Physiol 1992, 263:L1-14.
-
(1992)
Am J Physiol
, vol.263
-
-
Anderson, M.P.1
Sheppard, D.N.2
Berger, H.A.3
Welsh, M.J.4
-
74
-
-
0025874599
-
Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
-
Knowles MR, Clarke LL, Boucher RC Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991, 325:533-538.
-
(1991)
N Engl J Med
, vol.325
, pp. 533-538
-
-
Knowles, M.R.1
Clarke, L.L.2
Boucher, R.C.3
-
75
-
-
0025845558
-
Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium
-
Mason SJ, Paradiso AM, Boucher RC Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium. Br J Pharmacol 1991, 103:1649-1656.
-
(1991)
Br J Pharmacol
, vol.103
, pp. 1649-1656
-
-
Mason, S.J.1
Paradiso, A.M.2
Boucher, R.C.3
-
76
-
-
0034537007
-
+ absorption by extracellular nucleotides in human normal and cystic fibrosis airways
-
+ absorption by extracellular nucleotides in human normal and cystic fibrosis airways. Am J Respir Cell Mol Biol 2000, 23:755-761.
-
(2000)
Am J Respir Cell Mol Biol
, vol.23
, pp. 755-761
-
-
Mall, M.1
Wissner, A.2
Gonska, T.3
-
77
-
-
0034885663
-
The CF salt controversy: in vivo observations and therapeutic approaches
-
Tarran R, Grubb BR, Parsons D, et al. The CF salt controversy: in vivo observations and therapeutic approaches. Mol Cell 2001, 8:149-158.
-
(2001)
Mol Cell
, vol.8
, pp. 149-158
-
-
Tarran, R.1
Grubb, B.R.2
Parsons, D.3
-
79
-
-
54949112835
-
TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity
-
Caputo A, Caci E, Ferrera L, et al. TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity. Science 2008, 322:5904.
-
(2008)
Science
, vol.322
, pp. 5904
-
-
Caputo, A.1
Caci, E.2
Ferrera, L.3
-
80
-
-
51549120559
-
Expression cloning of TMEM16A as a calcium-activated chloride channel subunit
-
Schroeder BC, Cheng T, Jan YN, Jan LY Expression cloning of TMEM16A as a calcium-activated chloride channel subunit. Cell 2008, 134:1019-1029.
-
(2008)
Cell
, vol.134
, pp. 1019-1029
-
-
Schroeder, B.C.1
Cheng, T.2
Jan, Y.N.3
Jan, L.Y.4
-
81
-
-
55249091085
-
TMEM16A confers receptor-activated calcium-dependent chloride conductance
-
Yang YD, Cho H, Koo JY, et al. TMEM16A confers receptor-activated calcium-dependent chloride conductance. Nature 2008, 455:1210-1215.
-
(2008)
Nature
, vol.455
, pp. 1210-1215
-
-
Yang, Y.D.1
Cho, H.2
Koo, J.Y.3
-
82
-
-
78751478642
-
TMEM16A inhibitors reveal TMEM16A as a minor component of CaCC conductance in airway and intestinal epithelial cells
-
Namkung W, Phuan PW, Verkman AS TMEM16A inhibitors reveal TMEM16A as a minor component of CaCC conductance in airway and intestinal epithelial cells. J Biol Chem 2011, 286:2365-2374.
-
(2011)
J Biol Chem
, vol.286
, pp. 2365-2374
-
-
Namkung, W.1
Phuan, P.W.2
Verkman, A.S.3
-
83
-
-
77249176209
-
Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date
-
Becq F Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date. Drugs 2010, 70:241-259.
-
(2010)
Drugs
, vol.70
, pp. 241-259
-
-
Becq, F.1
-
85
-
-
79958134728
-
Mouse models of cystic fibrosis: Phenotypic analysis and research applications
-
Wilke M, Buijs-Offerman RM, Aarbiou J, et al. Mouse models of cystic fibrosis: Phenotypic analysis and research applications. J Cyst Fibros 2011, 10(Suppl 2):152-171.
-
(2011)
J Cyst Fibros
, vol.10
, Issue.SUPPL. 2
, pp. 152-171
-
-
Wilke, M.1
Buijs-Offerman, R.M.2
Aarbiou, J.3
-
87
-
-
77956249370
-
Responsiveness of mouse versus human CFTR-ΔF508 to small molecule correctors and potentiators
-
de Jonge H, Wilke M, Bot A, Sheppard DN Responsiveness of mouse versus human CFTR-ΔF508 to small molecule correctors and potentiators. Pediatr Pulmonol Suppl 2009, 32:291-292.
-
(2009)
Pediatr Pulmonol Suppl
, vol.32
, pp. 291-292
-
-
de Jonge, H.1
Wilke, M.2
Bot, A.3
Sheppard, D.N.4
-
88
-
-
52949154301
-
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs
-
Rogers CS, Stoltz DA, Meyerholz DK, et al. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science 2008, 321:1837-1841.
-
(2008)
Science
, vol.321
, pp. 1837-1841
-
-
Rogers, C.S.1
Stoltz, D.A.2
Meyerholz, D.K.3
-
89
-
-
78649908972
-
Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries
-
European Registry Working Group
-
Mehta G, Macek M, Mehta A, European Registry Working Group Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. J Cyst Fibros 2010, 9(Suppl 2):S5-21.
-
(2010)
J Cyst Fibros
, vol.9
, Issue.SUPPL. 2
-
-
Mehta, G.1
Macek, M.2
Mehta, A.3
-
90
-
-
79958158650
-
Guideline on the design and conduct of cystic fibrosis clinical trials: The European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN)
-
on behalf of all ECFS-CTN network partners et al.
-
De Boeck K, Bulteel V, Tiddens H, on behalf of all ECFS-CTN network partners, et al. Guideline on the design and conduct of cystic fibrosis clinical trials: The European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN). J Cyst Fibros 2011, 10(Suppl 2):67-74.
-
(2011)
J Cyst Fibros
, vol.10
, Issue.SUPPL. 2
, pp. 67-74
-
-
De Boeck, K.1
Bulteel, V.2
Tiddens, H.3
-
91
-
-
79958113612
-
Developing new products in cystic fibrosis: Needs and obstacles for activities of small and middle-sized companies
-
Schlangen M, Reimann ALG Developing new products in cystic fibrosis: Needs and obstacles for activities of small and middle-sized companies. J Cyst Fibros 2011, 10(Suppl 2):103-109.
-
(2011)
J Cyst Fibros
, vol.10
, Issue.SUPPL. 2
, pp. 103-109
-
-
Schlangen, M.1
Reimann, A.L.G.2
-
92
-
-
0036115199
-
Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells
-
Egan ME, Glöckner-Pagel J, Ambrose C, et al. Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells. Nat Med 2002, 8:485-492.
-
(2002)
Nat Med
, vol.8
, pp. 485-492
-
-
Egan, M.E.1
Glöckner-Pagel, J.2
Ambrose, C.3
-
93
-
-
11144355340
-
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
-
Egan ME, Pearson M, Weiner SA, et al. Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science 2004, 304:600-602.
-
(2004)
Science
, vol.304
, pp. 600-602
-
-
Egan, M.E.1
Pearson, M.2
Weiner, S.A.3
-
94
-
-
33645844261
-
Rescue of ΔF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network
-
Lipecka J, Norez C, Bensalem N, et al. Rescue of ΔF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network. J Pharmacol Exp Ther 2006, 317:500-505.
-
(2006)
J Pharmacol Exp Ther
, vol.317
, pp. 500-505
-
-
Lipecka, J.1
Norez, C.2
Bensalem, N.3
-
95
-
-
33645235405
-
2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells
-
2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells. Traffic 2006, 7:562-573.
-
(2006)
Traffic
, vol.7
, pp. 562-573
-
-
Norez, C.1
Antigny, F.2
Becq, F.3
Vandebrouck, C.4
-
96
-
-
76249093127
-
Partial correction of cystic fibrosis defects with PLGA nanoparticles encapsulating curcumin
-
Cartiera MS, Ferreira EC, Caputo C, Egan ME, Caplan MJ, Saltzman WM Partial correction of cystic fibrosis defects with PLGA nanoparticles encapsulating curcumin. Mol Pharm 2010, 7:86-93.
-
(2010)
Mol Pharm
, vol.7
, pp. 86-93
-
-
Cartiera, M.S.1
Ferreira, E.C.2
Caputo, C.3
Egan, M.E.4
Caplan, M.J.5
Saltzman, W.M.6
-
97
-
-
33847793158
-
Rescue of the F508del CFTR trafficking defect in murine intestine by proteasome inhibitors
-
Wilke M, Bot AG, Jorna H, Marino CR, de Jonge HR Rescue of the F508del CFTR trafficking defect in murine intestine by proteasome inhibitors. Pediatr Pulmonol Suppl 2005, 28:276.
-
(2005)
Pediatr Pulmonol Suppl
, vol.28
, pp. 276
-
-
Wilke, M.1
Bot, A.G.2
Jorna, H.3
Marino, C.R.4
de Jonge, H.R.5
-
98
-
-
33745190973
-
Selective inhibition of endoplasmic reticulum-associated degradation rescues ΔF508-cystic fibrosis trans-membrane regulator and suppresses interleukin-8 levels: therapeutic implications
-
Vij N, Fang S, Zeitlin PL Selective inhibition of endoplasmic reticulum-associated degradation rescues ΔF508-cystic fibrosis trans-membrane regulator and suppresses interleukin-8 levels: therapeutic implications. J Biol Chem 2006, 281:17369-17378.
-
(2006)
J Biol Chem
, vol.281
, pp. 17369-17378
-
-
Vij, N.1
Fang, S.2
Zeitlin, P.L.3
-
99
-
-
65749105978
-
High-content functional screen to identify proteins that correct F508del-CFTR function
-
Trzcinska-Daneluti AM, Ly D, Huynh L, Jiang C, Fladd C, Rotin D High-content functional screen to identify proteins that correct F508del-CFTR function. Mol Cell Proteomics 2009, 8:780-790.
-
(2009)
Mol Cell Proteomics
, vol.8
, pp. 780-790
-
-
Trzcinska-Daneluti, A.M.1
Ly, D.2
Huynh, L.3
Jiang, C.4
Fladd, C.5
Rotin, D.6
-
100
-
-
67651152728
-
A cystic fibrosis respiratory epithelial cell chronically treated by miglustat acquires a non-cystic fibrosis-like phenotype
-
Norez C, Antigny F, Noel S, Vandebrouck C, Becq F A cystic fibrosis respiratory epithelial cell chronically treated by miglustat acquires a non-cystic fibrosis-like phenotype. Am J Respir Cell Mol Biol 2009, 41:217-225.
-
(2009)
Am J Respir Cell Mol Biol
, vol.41
, pp. 217-225
-
-
Norez, C.1
Antigny, F.2
Noel, S.3
Vandebrouck, C.4
Becq, F.5
-
101
-
-
55349097458
-
Anti-inflammatory effect of miglustat in bronchial epithelial cells
-
Dechecchi MC, Nicolis E, Norez C, et al. Anti-inflammatory effect of miglustat in bronchial epithelial cells. J Cyst Fibros 2008, 7:555-565.
-
(2008)
J Cyst Fibros
, vol.7
, pp. 555-565
-
-
Dechecchi, M.C.1
Nicolis, E.2
Norez, C.3
-
102
-
-
66249120003
-
Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice
-
Lubamba B, Lebacq J, Lebecque P, et al. Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice. Am J Respir Crit Care Med 2009, 179:1022-1028.
-
(2009)
Am J Respir Crit Care Med
, vol.179
, pp. 1022-1028
-
-
Lubamba, B.1
Lebacq, J.2
Lebecque, P.3
-
103
-
-
0035203949
-
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells
-
Dormer RL, Dérand R, McNeilly CM, et al. Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J Cell Sei 2001, 114:4073-4081.
-
(2001)
J Cell Sei
, vol.114
, pp. 4073-4081
-
-
Dormer, R.L.1
Dérand, R.2
McNeilly, C.M.3
-
104
-
-
0037427513
-
Benzo(c)quinolizinium drugs inhibit degradation of ΔF508-CFTR cytoplasmic domain
-
Stratford FL, Pereira MM, Becq F, McPherson MA, Dormer RL Benzo(c)quinolizinium drugs inhibit degradation of ΔF508-CFTR cytoplasmic domain. Biochem Biophys Res Commun 2003, 300:524-530.
-
(2003)
Biochem Biophys Res Commun
, vol.300
, pp. 524-530
-
-
Stratford, F.L.1
Pereira, M.M.2
Becq, F.3
McPherson, M.A.4
Dormer, R.L.5
-
105
-
-
34247629486
-
MPB-07 reduces the inflammatory response to Pseudomonas aeruginosa in cystic fibrosis bronchial cells
-
Dechecchi MC, Nicolis E, Bezzerri V, et al. MPB-07 reduces the inflammatory response to Pseudomonas aeruginosa in cystic fibrosis bronchial cells. Am J Respir Cell Mol Biol 2007, 36:615-624.
-
(2007)
Am J Respir Cell Mol Biol
, vol.36
, pp. 615-624
-
-
Dechecchi, M.C.1
Nicolis, E.2
Bezzerri, V.3
-
106
-
-
11944265976
-
Sildenafil (Viagra) corrects ΔF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis
-
Dormer RL, Harris CM, Clark Z, et al. Sildenafil (Viagra) corrects ΔF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis. Thorax 2005, 60:55-59.
-
(2005)
Thorax
, vol.60
, pp. 55-59
-
-
Dormer, R.L.1
Harris, C.M.2
Clark, Z.3
-
107
-
-
40649117683
-
Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis
-
Lubamba B, Lecourt H, Lebacq J, et al. Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med 2008, 177:506-515.
-
(2008)
Am J Respir Crit Care Med
, vol.177
, pp. 506-515
-
-
Lubamba, B.1
Lecourt, H.2
Lebacq, J.3
-
108
-
-
34548407741
-
Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis
-
Poschet JF, Timmins GS, Taylor-Cousar JL, et al. Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 2007, 293:L712-L719.
-
(2007)
Am J Physiol Lung Cell Mol Physiol
, vol.293
-
-
Poschet, J.F.1
Timmins, G.S.2
Taylor-Cousar, J.L.3
-
109
-
-
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, 73:478-489.
-
(2008)
Mol Pharmacol
, vol.73
, pp. 478-489
-
-
Robert, R.1
Carlile, G.W.2
Pavel, C.3
-
110
-
-
53549097399
-
Potent s-cis-locked bithiazole correctors of ΔF508 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 ΔF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy. J Med Chem 2008, 51:6044-6054.
-
(2008)
J Med Chem
, vol.51
, pp. 6044-6054
-
-
Yu, G.J.1
Yoo, C.L.2
Yang, B.3
-
111
-
-
77950428804
-
Reduced histone deacety-lase 7 activity restores function to misfolded CFTR in cystic fibrosis
-
Hutt DM, Herman D, Rodrigues AP, et al. Reduced histone deacety-lase 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
-
112
-
-
0031853136
-
Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by de-oxyspergualin
-
Jiang C, Fang SL, Xiao YF, et al. Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by de-oxyspergualin. Am J Physiol 1998, 275:C171-C178.
-
(1998)
Am J Physiol
, vol.275
-
-
Jiang, C.1
Fang, S.L.2
Xiao, Y.F.3
-
114
-
-
0031889082
-
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function
-
Rubenstein RC, Zeitlin PL A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998, 157:484-490.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 484-490
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
115
-
-
0036665609
-
Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
-
Zeitlin PL, Diener-West M, Rubenstein RC, Boyle MP, Lee CK, Brass-Ernst L Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol Ther 2002, 6:119-126.
-
(2002)
Mol Ther
, vol.6
, pp. 119-126
-
-
Zeitlin, P.L.1
Diener-West, M.2
Rubenstein, R.C.3
Boyle, M.P.4
Lee, C.K.5
Brass-Ernst, L.6
-
116
-
-
0032489513
-
Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-l,3-dipropylxanthine (CPX) and l,3-diallyl-8-cyclohexylxanthine (DAX)
-
Arispe N, Ma J, Jacobson KA, Pollard HB Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-l,3-dipropylxanthine (CPX) and l,3-diallyl-8-cyclohexylxanthine (DAX). J Biol Chem 1998, 273:5727-5734.
-
(1998)
J Biol Chem
, vol.273
, pp. 5727-5734
-
-
Arispe, N.1
Ma, J.2
Jacobson, K.A.3
Pollard, H.B.4
-
117
-
-
0033027269
-
Activation of wild-type and ΔF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms
-
Al-Nakkash L, Hwang TC Activation of wild-type and ΔF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms. Pflugers Arch 1999, 437:553-561.
-
(1999)
Pflugers Arch
, vol.437
, pp. 553-561
-
-
Al-Nakkash, L.1
Hwang, T.C.2
-
118
-
-
0030818110
-
Direct action of genistein on CFTR
-
Weinreich F, Wood PG, Riordan JR, Nagel G Direct action of genistein on CFTR. Pflugers Arch 1997, 434:484-491.
-
(1997)
Pflugers Arch
, vol.434
, pp. 484-491
-
-
Weinreich, F.1
Wood, P.G.2
Riordan, J.R.3
Nagel, G.4
-
120
-
-
0032743305
-
Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein
-
Illek B, Zhang L, Lewis NC, Moss RB, Dong JY, Fischer H Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein. Am J Physiol 1999, 277:C833-C839.
-
(1999)
Am J Physiol
, vol.277
-
-
Illek, B.1
Zhang, L.2
Lewis, N.C.3
Moss, R.B.4
Dong, J.Y.5
Fischer, H.6
-
121
-
-
0035141190
-
A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogs
-
Al-Nakkash L, Hu S, Li M, Hwang TC A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogs. J Pharmacol Exp Ther 2001, 296:464-472.
-
(2001)
J Pharmacol Exp Ther
, vol.296
, pp. 464-472
-
-
Al-Nakkash, L.1
Hu, S.2
Li, M.3
Hwang, T.C.4
-
122
-
-
0036797539
-
Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07
-
Zegarra-Moran O, Romio L, Folli C, et al. Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07. Br J Pharmacol 2002, 137:504-512.
-
(2002)
Br J Pharmacol
, vol.137
, pp. 504-512
-
-
Zegarra-Moran, O.1
Romio, L.2
Folli, C.3
-
124
-
-
0037183975
-
The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory genistein binding site
-
Derand R, Bulteau-Pignoux L, Becq F The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory genistein binding site. J Biol Chem 2002, 277:35999-36004.
-
(2002)
J Biol Chem
, vol.277
, pp. 35999-36004
-
-
Derand, R.1
Bulteau-Pignoux, L.2
Becq, F.3
-
125
-
-
33751074719
-
Dietary genistein stimulates anion secretion across female murine intestine
-
Al-Nakkash L, Clarke LL, Rottinghaus GE, Chen YJ, Cooper K, Rubin LJ Dietary genistein stimulates anion secretion across female murine intestine. J Nutr 2006, 136:2785-2790.
-
(2006)
J Nutr
, vol.136
, pp. 2785-2790
-
-
Al-Nakkash, L.1
Clarke, L.L.2
Rottinghaus, G.E.3
Chen, Y.J.4
Cooper, K.5
Rubin, L.J.6
-
126
-
-
0033636431
-
Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin
-
Illek B, Lizarzaburu ME, Lee V, Nantz MH, Kurth MJ, Fischer H Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin. Am J Physiol Cell Physiol 2000, 279:C1838-C1846.
-
(2000)
Am J Physiol Cell Physiol
, vol.279
-
-
Illek, B.1
Lizarzaburu, M.E.2
Lee, V.3
Nantz, M.H.4
Kurth, M.J.5
Fischer, H.6
-
127
-
-
0033600763
-
Development of substituted benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel
-
Becq F, Mettey Y, Gray MA, et al. Development of substituted benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel. J Biol Chem 1999, 274:27415-27425.
-
(1999)
J Biol Chem
, vol.274
, pp. 27415-27425
-
-
Becq, F.1
Mettey, Y.2
Gray, M.A.3
-
128
-
-
10744229649
-
Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels
-
Marivingt-Mounir C, Norez C, Dérand R, et al. Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels. J Med Chem 2004, 47:962-972.
-
(2004)
J Med Chem
, vol.47
, pp. 962-972
-
-
Marivingt-Mounir, C.1
Norez, C.2
Dérand, R.3
-
129
-
-
0035827680
-
Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds
-
Galietta LJ, Springsteel MF, Eda M, et al. Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds. J Biol Chem 2001, 276:19723-19728.
-
(2001)
J Biol Chem
, vol.276
, pp. 19723-19728
-
-
Galietta, L.J.1
Springsteel, M.F.2
Eda, M.3
-
130
-
-
0037971989
-
3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators
-
Sammelson RE, Ma T, Galietta LJ, Verkman AS, Kurth MJ 3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators. Bioorg Med Chem Lett 2003, 13:2509-2512.
-
(2003)
Bioorg Med Chem Lett
, vol.13
, pp. 2509-2512
-
-
Sammelson, R.E.1
Ma, T.2
Galietta, L.J.3
Verkman, A.S.4
Kurth, M.J.5
-
131
-
-
17044448151
-
CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds
-
Caci E, Folli C, Zegarra-Moran O, et al. CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds. Am J Physiol Lung Cell Mol Physiol 2003, 285:L180-L188.
-
(2003)
Am J Physiol Lung Cell Mol Physiol
, vol.285
-
-
Caci, E.1
Folli, C.2
Zegarra-Moran, O.3
-
132
-
-
0041589194
-
Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain
-
Springsteel MF, Galietta LJ, Ma T, et al. Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain. Bioorg Med Chem 2003, 11:4113-4120.
-
(2003)
Bioorg Med Chem
, vol.11
, pp. 4113-4120
-
-
Springsteel, M.F.1
Galietta, L.J.2
Ma, T.3
-
133
-
-
0037020097
-
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening
-
Ma T, Vetrivel L, Yang H, et al. High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening. J Biol Chem 2002, 277:37235-37241.
-
(2002)
J Biol Chem
, vol.277
, pp. 37235-37241
-
-
Ma, T.1
Vetrivel, L.2
Yang, H.3
-
135
-
-
0037205444
-
Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity
-
Cai Z, Sheppard DN Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity. J Biol Chem 2002, 277:19546-19553.
-
(2002)
J Biol Chem
, vol.277
, pp. 19546-19553
-
-
Cai, Z.1
Sheppard, D.N.2
-
136
-
-
33646455879
-
The glycine residues G551 and G1349 within the ATP-binding cassette signature motifs play critical roles in the activation and inhibition of cystic fibrosis transmembrane conductance regulator channels by phloxine B
-
Melin P, Norez C, Callebaut I, Becq F The glycine residues G551 and G1349 within the ATP-binding cassette signature motifs play critical roles in the activation and inhibition of cystic fibrosis transmembrane conductance regulator channels by phloxine B. J Membr Biol 2005, 208:203-212.
-
(2005)
J Membr Biol
, vol.208
, pp. 203-212
-
-
Melin, P.1
Norez, C.2
Callebaut, I.3
Becq, F.4
-
138
-
-
0042317111
-
Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating
-
Yang H, Shelat AA, Guy RK, et al. Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating. J Biol Chem 2003, 278:35079-35085.
-
(2003)
J Biol Chem
, vol.278
, pp. 35079-35085
-
-
Yang, H.1
Shelat, A.A.2
Guy, R.K.3
-
140
-
-
20944442087
-
Phenylglycine and sulfonamide correctors of defective ΔF508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating
-
Pedemonte N, Sonawane ND, Taddei A, et al. Phenylglycine and sulfonamide correctors of defective ΔF508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating. Mol Pharmacol 2005, 67:1797-1807.
-
(2005)
Mol Pharmacol
, vol.67
, pp. 1797-1807
-
-
Pedemonte, N.1
Sonawane, N.D.2
Taddei, A.3
-
141
-
-
70349120419
-
Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators
-
Caputo A, Hinzpeter A, Caci E, et al. Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators. J Pharmacol Exp Ther 2009, 330:783-791.
-
(2009)
J Pharmacol Exp Ther
, vol.330
, pp. 783-791
-
-
Caputo, A.1
Hinzpeter, A.2
Caci, E.3
-
142
-
-
21244494942
-
Activating cystic fibrosis trans-membrane conductance regulator channels with pore blocker analogs
-
Wang W, Li G, Clancy JP, Kirk KL Activating cystic fibrosis trans-membrane conductance regulator channels with pore blocker analogs. J Biol Chem 2005, 280:23622-23630.
-
(2005)
J Biol Chem
, vol.280
, pp. 23622-23630
-
-
Wang, W.1
Li, G.2
Clancy, J.P.3
Kirk, K.L.4
-
143
-
-
27844445676
-
Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations
-
Pedemonte N, Diena T, Caci E, et al. Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations. Mol Pharmacol 2005, 68:1736-1746.
-
(2005)
Mol Pharmacol
, vol.68
, pp. 1736-1746
-
-
Pedemonte, N.1
Diena, T.2
Caci, E.3
-
144
-
-
34347242438
-
Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel
-
Pedemonte N, Boido D, Moran O, et al. Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel. Mol Pharmacol 2007, 72:197-207.
-
(2007)
Mol Pharmacol
, vol.72
, pp. 197-207
-
-
Pedemonte, N.1
Boido, D.2
Moran, O.3
-
145
-
-
20444409680
-
4-Chlorobenzo[F]isoquinoline (CBIQ), a novel activator of CFTR and ΔF508 CFTR
-
Murthy M, Pedemonte N, MacVinish L, Galietta L, Cuthbert A 4-Chlorobenzo[F]isoquinoline (CBIQ), a novel activator of CFTR and ΔF508 CFTR. Eur J Pharmacol 2005, 516:118-124.
-
(2005)
Eur J Pharmacol
, vol.516
, pp. 118-124
-
-
Murthy, M.1
Pedemonte, N.2
MacVinish, L.3
Galietta, L.4
Cuthbert, A.5
-
146
-
-
66849129301
-
A small-molecule modulator interacts directly with ΔPhe508-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 ΔPhe508-CFTR to modify its ATPase activity and conformational stability. Mol Pharmacol 2009, 75:1430-1438.
-
(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
-
147
-
-
33748987102
-
Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channels
-
Noel S, Faveau C, Norez C, Rogier C, Mettey Y, Becq F Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channels. J Pharmacol Exp Ther 2006, 319:349-359.
-
(2006)
J Pharmacol Exp Ther
, vol.319
, pp. 349-359
-
-
Noel, S.1
Faveau, C.2
Norez, C.3
Rogier, C.4
Mettey, Y.5
Becq, F.6
-
148
-
-
29544440095
-
Sulfamoyl-4-oxoquinoline-3-carboxamides: novel potentiators of defective ΔF508-cystic fibrosis transmembrane conductance regulator chloride channel gating
-
Suen YF, Robins L, Yang B, Verkman AS, Nantz MH, Kurth MJ Sulfamoyl-4-oxoquinoline-3-carboxamides: novel potentiators of defective ΔF508-cystic fibrosis transmembrane conductance regulator chloride channel gating. Bioorg Med Chem Lett 2006, 16:537-540.
-
(2006)
Bioorg Med Chem Lett
, vol.16
, pp. 537-540
-
-
Suen, Y.F.1
Robins, L.2
Yang, B.3
Verkman, A.S.4
Nantz, M.H.5
Kurth, M.J.6
-
149
-
-
77955647867
-
Identification of a novel water-soluble activator of wild-type and F508del CFTR: GPact-1 la
-
Bertrand J, Boucherie B, Billet A, et al. Identification of a novel water-soluble activator of wild-type and F508del CFTR: GPact-1 la. Eur Respir J 2010, 36:311-322.
-
(2010)
Eur Respir J
, vol.36
, pp. 311-322
-
-
Bertrand, J.1
Boucherie, B.2
Billet, A.3
-
150
-
-
0036161237
-
A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis
-
McCarty NA, Standaert TA, Teresi M, et al. A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Pediatr Pulmonol 2002, 33:90-98.
-
(2002)
Pediatr Pulmonol
, vol.33
, pp. 90-98
-
-
McCarty, N.A.1
Standaert, T.A.2
Teresi, M.3
|