-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan J, Rommens J, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245(4922):1066-1073.
-
(1989)
Science
, vol.245
, Issue.4922
, pp. 1066-1073
-
-
Riordan, J.1
Rommens, J.2
Kerem, B.3
-
2
-
-
84878464222
-
Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis
-
Huaux F, Noel S, Dhooghe B, et al. Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis. PLoS One. 2013;8(5):e64341.
-
(2013)
Plos One
, vol.8
, Issue.5
-
-
Huaux, F.1
Noel, S.2
Dhooghe, B.3
-
3
-
-
84921029693
-
Cystic fibrosis transmembrane conductance regulator protein (CFTR) expression in the developing human brain: Comparative immunohis-tochemical study between patients with normal and mutated CFTR.
-
Marcorelles P, Friocourt G, Uguen A, Ledé F, Férec C, Laquerrière A. Cystic fibrosis transmembrane conductance regulator protein (CFTR) expression in the developing human brain: comparative immunohis-tochemical study between patients with normal and mutated CFTR. J Histochem Cytochem. 2014;62(11):791-801.
-
(2014)
J Histochem Cytochem
, vol.62
, Issue.11
, pp. 791-801
-
-
Marcorelles, P.1
Friocourt, G.2
Uguen, A.3
Ledé, F.4
Férec, C.5
Laquerrière, A.6
-
4
-
-
33845743367
-
Evidence for cystic fibrosis transmembrane conductance regulator chloride current in swine ventricular myocytes
-
Gao Z, Sun HY, Lau CP, Chin-Wan Fung P, Li GR. Evidence for cystic fibrosis transmembrane conductance regulator chloride current in swine ventricular myocytes. J Mol Cell Cardiol. 2007;42(1):98-105.
-
(2007)
J Mol Cell Cardiol
, vol.42
, Issue.1
, pp. 98-105
-
-
Gao, Z.1
Sun, H.Y.2
Lau, C.P.3
Chin-Wan Fung, P.4
Li, G.R.5
-
5
-
-
84868322168
-
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection
-
Bonfield TL, Hodges CA, Cotton CU, Drumm ML. Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection. J Leukoc Biol. 2012;92(5):1111-1122.
-
(2012)
J Leukoc Biol
, vol.92
, Issue.5
, pp. 1111-1122
-
-
Bonfield, T.L.1
Hodges, C.A.2
Cotton, C.U.3
Drumm, M.L.4
-
6
-
-
33748146503
-
CFTR regulates phagosome acidification in macrophages and alters bactericidal activity
-
Di A, Brown ME, Deriy LV, et al. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. Nat Cell Biol. 2006;8(9):933-944.
-
(2006)
Nat Cell Biol
, vol.8
, Issue.9
, pp. 933-944
-
-
Di, A.1
Brown, M.E.2
Deriy, L.V.3
-
7
-
-
33748654201
-
CFTR expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis
-
Painter RG, Valentine VG, Lanson NA, et al. CFTR expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis. Biochemistry. 2006;45(34):10260-10269.
-
(2006)
Biochemistry
, vol.45
, Issue.34
, pp. 10260-10269
-
-
Painter, R.G.1
Valentine, V.G.2
Lanson, N.A.3
-
8
-
-
0023272069
-
Mapping of DNA markers linked to the cystic fibrosis locus on the long arm of chromosome 7
-
Zengerling S, Tsui LC, Grzeschik KH, Olek K, Riordan JR, Buchwald M. Mapping of DNA markers linked to the cystic fibrosis locus on the long arm of chromosome 7. Am J Hum Genet. 1987;40(3):228-236.
-
(1987)
Am J Hum Genet
, vol.40
, Issue.3
, pp. 228-236
-
-
Zengerling, S.1
Tsui, L.C.2
Grzeschik, K.H.3
Olek, K.4
Riordan, J.R.5
Buchwald, M.6
-
9
-
-
0022350960
-
Cystic fibrosis locus defined by a genetically linked polymorphic DNA marker
-
Tsui L, Buchwald M, Barker D, et al. Cystic fibrosis locus defined by a genetically linked polymorphic DNA marker. Science. 1985;230(4729): 1054-1057.
-
(1985)
Science
, vol.230
, Issue.4729
, pp. 1054-1057
-
-
Tsui, L.1
Buchwald, M.2
Barker, D.3
-
10
-
-
33645307384
-
The ABC protein turned chloride channel whose failure causes cystic fibrosis
-
Gadsby DC, Vergani P, Csanady L. The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature. 2006;440(7083): 477-483.
-
(2006)
Nature
, vol.440
, Issue.7083
, pp. 477-483
-
-
Gadsby, D.C.1
Vergani, P.2
Csanady, L.3
-
11
-
-
84880428551
-
Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain
-
Sebastian A, Rishishwar L, Wang J, et al. Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain. Gene. 2013;523(2): 137-146.
-
(2013)
Gene
, vol.523
, Issue.2
, pp. 137-146
-
-
Sebastian, A.1
Rishishwar, L.2
Wang, J.3
-
12
-
-
0025987020
-
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
-
Cheng SH, Rich DP, Marshall J, Gregory RJ, Welsh MJ, Smith AE. Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell. 1991;66(5):1027-1036.
-
(1991)
Cell
, vol.66
, Issue.5
, pp. 1027-1036
-
-
Cheng, S.H.1
Rich, D.P.2
Marshall, J.3
Gregory, R.J.4
Welsh, M.J.5
Smith, A.E.6
-
13
-
-
20844457435
-
Preferential phosphorylation of R-domain Serine 768 dampens activation of CFTR channels by PKA.
-
Csanády L, Seto-Young D, Chan KW, et al. Preferential phosphorylation of R-domain Serine 768 dampens activation of CFTR channels by PKA. J Gen Physiol. 2005;125(2):171-186.
-
(2005)
J Gen Physiol
, vol.125
, Issue.2
, pp. 171-186
-
-
Csanády, L.1
Seto-Young, D.2
Chan, K.W.3
-
14
-
-
84965089611
-
Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation
-
Billet A, Jia Y, Jensen T, Riordan JR, Hanrahan JW. Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation. FASEB J. 2015;29(9):3945-3953.
-
(2015)
FASEB J
, vol.29
, Issue.9
, pp. 3945-3953
-
-
Billet, A.1
Jia, Y.2
Jensen, T.3
Riordan, J.R.4
Hanrahan, J.W.5
-
15
-
-
0037404448
-
Physiological modulation of CFTR activity by AMP-activated protein kinase in polarized T84 cells
-
Hallows KR, Kobinger GP, Wilson JM, Witters LA, Foskett JK. Physiological modulation of CFTR activity by AMP-activated protein kinase in polarized T84 cells. Am J Physiol Cell Physiol. 2003;284(5):C1297-C1308.
-
(2003)
Am J Physiol Cell Physiol
, vol.284
, Issue.5
, pp. C1297-C1308
-
-
Hallows, K.R.1
Kobinger, G.P.2
Wilson, J.M.3
Witters, L.A.4
Foskett, J.K.5
-
16
-
-
57349092525
-
PKC phosphorylation modulates PKA-dependent binding of the R domain to other domains of CFTR
-
Seavilleklein G, Amer N, Evagelidis A, et al. PKC phosphorylation modulates PKA-dependent binding of the R domain to other domains of CFTR. Am J Physiol Cell Physiol. 2008;295(5):C1366-C1375.
-
(2008)
Am J Physiol Cell Physiol
, vol.295
, Issue.5
, pp. C1366-C1375
-
-
Seavilleklein, G.1
Amer, N.2
Evagelidis, A.3
-
17
-
-
0033817333
-
Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains
-
Csanády L, Chan KW, Seto-Young D, Kopsco DC, Nairn AC, Gadsby DC. Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains. J Gen Physiol. 2000;116(3):477-500.
-
(2000)
J Gen Physiol
, vol.116
, Issue.3
, pp. 477-500
-
-
Csanády, L.1
Chan, K.W.2
Seto-Young, D.3
Kopsco, D.C.4
Nairn, A.C.5
Gadsby, D.C.6
-
18
-
-
23844483240
-
Phosphorylation of CFTR by PKA promotes binding of the regulatory domain
-
Chappe V, Irvine T, Liao J, Evagelidis A, Hanrahan JW. Phosphorylation of CFTR by PKA promotes binding of the regulatory domain. EMBO J. 2005;24(15):2730-2740.
-
(2005)
EMBO J
, vol.24
, Issue.15
, pp. 2730-2740
-
-
Chappe, V.1
Irvine, T.2
Liao, J.3
Evagelidis, A.4
Hanrahan, J.W.5
-
19
-
-
65549153992
-
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A
-
Hegedűs T, Aleksandrov A, Mengos A, Cui L, Jensen TJ, Riordan JR. Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A. Biochim Biophys Acta. 2009;1788(6):1341-1349.
-
(2009)
Biochim Biophys Acta
, vol.1788
, Issue.6
, pp. 1341-1349
-
-
Hegedűs, T.1
Aleksandrov, A.2
Mengos, A.3
Cui, L.4
Jensen, T.J.5
Riordan, J.R.6
-
20
-
-
0030816586
-
CFTR activation: Additive effects of stimulatory and inhibitory phosphorylation sites in the R domain
-
Wilkinson DJ, Strong TV, Mansoura MK, et al. CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain. Am J Physiol Lung Cell Mol Physiol. 1997;273(1):L127-L133.
-
(1997)
Am J Physiol Lung Cell Mol Physiol
, vol.273
, Issue.1
, pp. L127-L133
-
-
Wilkinson, D.J.1
Strong, T.V.2
Mansoura, M.K.3
-
21
-
-
4143102419
-
Dibasic phosphorylation sites in the R domain of CFTR have stimulatory and inhibitory effects on channel activation
-
Vais H, Zhang R, Reenstra WW. Dibasic phosphorylation sites in the R domain of CFTR have stimulatory and inhibitory effects on channel activation. Am J Physiol Cell Physiol. 2004;287(3):C737-C745.
-
(2004)
Am J Physiol Cell Physiol
, vol.287
, Issue.3
, pp. C737-C745
-
-
Vais, H.1
Zhang, R.2
Reenstra, W.W.3
-
22
-
-
84888085224
-
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions
-
Bozoky Z, Krzeminski M, Muhandiram R, et al. Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions. Proc Natl Acad Sci USA. 2013;110(47):E4427-E4436.
-
(2013)
Proc Natl Acad Sci USA
, vol.110
, Issue.47
, pp. E4427-E4436
-
-
Bozoky, Z.1
Krzeminski, M.2
Muhandiram, R.3
-
23
-
-
84883454123
-
Structural changes of CFTR R region upon phosphorylation: A plastic platform for intramolecular and intermolecular interactions
-
Bozoky Z, Krzeminski M, Chong PA, Forman-Kay JD. Structural changes of CFTR R region upon phosphorylation: a plastic platform for intramolecular and intermolecular interactions. FEBS J. 2013;280(18): 4407-4416.
-
(2013)
FEBS J
, vol.280
, Issue.18
, pp. 4407-4416
-
-
Bozoky, Z.1
Krzeminski, M.2
Chong, P.A.3
Forman-Kay, J.D.4
-
25
-
-
84901610851
-
On the structural organization of the intracellular domains of CFTR
-
Moran O. On the structural organization of the intracellular domains of CFTR. Int J Biochem Cell Biol. 2014;52:7-14.
-
(2014)
Int J Biochem Cell Biol
, vol.52
, pp. 7-14
-
-
Moran, O.1
-
26
-
-
65749102092
-
Gating of the CFTR Cl(−) channel by ATP-driven nucleotide-binding domain dimerisation
-
Hwang TC, Sheppard DN. Gating of the CFTR Cl(−) channel by ATP-driven nucleotide-binding domain dimerisation. J Physiol. 2009;587(pt 10):2151-2161.
-
(2009)
J Physiol
, vol.587
, pp. 2151-2161
-
-
Hwang, T.C.1
Sheppard, D.N.2
-
27
-
-
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(2):282-293.
-
(2004)
EMBO J
, vol.23
, Issue.2
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
-
28
-
-
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(7028):876-880.
-
(2005)
Nature
, vol.433
, Issue.7028
, pp. 876-880
-
-
Vergani, P.1
Lockless, S.W.2
Nairn, A.C.3
Gadsby, D.C.4
-
29
-
-
33749039521
-
The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics
-
Zhou Z, Wang X, Liu HY, Zou X, Li M, Hwang TC. The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics. J Gen Physiol. 2006;128(4):413-422.
-
(2006)
J Gen Physiol
, vol.128
, Issue.4
, pp. 413-422
-
-
Zhou, Z.1
Wang, X.2
Liu, H.Y.3
Zou, X.4
Li, M.5
Hwang, T.C.6
-
30
-
-
0025868103
-
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity
-
Anderson M, Gregory R, Thompson S, et al. Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science. 1991;253(5016):202-205.
-
(1991)
Science
, vol.253
, Issue.5016
, pp. 202-205
-
-
Anderson, M.1
Gregory, R.2
Thompson, S.3
-
31
-
-
32544435783
-
Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channel
-
Linsdell P. Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channel. Exp Physiol. 2006;91(1):123-129.
-
(2006)
Exp Physiol
, vol.91
, Issue.1
, pp. 123-129
-
-
Linsdell, P.1
-
32
-
-
0031954021
-
Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel
-
Linsdell P, Hanrahan JW. Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel. J Gen Physiol. 1998;111(4):601-614.
-
(1998)
J Gen Physiol
, vol.111
, Issue.4
, pp. 601-614
-
-
Linsdell, P.1
Hanrahan, J.W.2
-
33
-
-
84863395512
-
CFTR is the primary known apical glutathione transporter involved in cigarette smoke induced adaptive responses in the lung
-
Gould NS, Min E, Martin RJ, Day BJ. CFTR is the primary known apical glutathione transporter involved in cigarette smoke induced adaptive responses in the lung. Free Radic Biol Med. 2012;52(7):1201-1206.
-
(2012)
Free Radic Biol Med
, vol.52
, Issue.7
, pp. 1201-1206
-
-
Gould, N.S.1
Min, E.2
Martin, R.J.3
Day, B.J.4
-
35
-
-
60549083963
-
Mechanism of direct bicarbonate transport by the CFTR anion channel
-
Tang L, Fatehi M, Linsdell P. Mechanism of direct bicarbonate transport by the CFTR anion channel. J Cyst Fibros. 2009;8(2):115-121.
-
(2009)
J Cyst Fibros
, vol.8
, Issue.2
, pp. 115-121
-
-
Tang, L.1
Fatehi, M.2
Linsdell, P.3
-
36
-
-
0020700816
-
Chloride impermeability in cystic fibrosis
-
Quinton PM. Chloride impermeability in cystic fibrosis. Nature. 1983;301(5899):421-422.
-
(1983)
Nature
, vol.301
, Issue.5899
, pp. 421-422
-
-
Quinton, P.M.1
-
37
-
-
48149090717
-
Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis
-
Quinton PM. Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis. Lancet. 2008;372(9636):415-417.
-
(2008)
Lancet
, vol.372
, Issue.9636
, pp. 415-417
-
-
Quinton, P.M.1
-
38
-
-
0019809960
-
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
-
Knowles M, Gatzy J, Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981;305(25):1489-1495.
-
(1981)
N Engl J Med
, vol.305
, Issue.25
, pp. 1489-1495
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
39
-
-
84942123413
-
Targeting ENaC as a molecular suspect in cystic fibrosis
-
Bangel-Ruland N, Tomczak K, Weber WM. Targeting ENaC as a molecular suspect in cystic fibrosis. Curr Drug Targets. 2015;16(9):951-957.
-
(2015)
Curr Drug Targets
, vol.16
, Issue.9
, pp. 951-957
-
-
Bangel-Ruland, N.1
Tomczak, K.2
Weber, W.M.3
-
40
-
-
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(7):1005-1015.
-
(1998)
Cell
, vol.95
, Issue.7
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
-
41
-
-
84861879494
-
The CFTR and ENaC debate: How important is ENaC in CF lung disease?
-
Collawn JF, Lazrak A, Bebok Z, Matalon S. The CFTR and ENaC debate: how important is ENaC in CF lung disease? Am J Physiol Lung Cell Mol Physiol. 2012;302(11):L1141-L1146.
-
(2012)
Am J Physiol Lung Cell Mol Physiol
, vol.302
, Issue.11
, pp. L1141-L1146
-
-
Collawn, J.F.1
Lazrak, A.2
Bebok, Z.3
Matalon, S.4
-
42
-
-
84884284486
-
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?
-
Hobbs CA, Da Tan C, Tarran R. Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease? J Physiol. 2013;591(pt 18):4377-4387.
-
(2013)
J Physiol
, vol.591
, pp. 4377-4387
-
-
Hobbs, C.A.1
Da Tan, C.2
Tarran, R.3
-
43
-
-
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(50):36481-36488.
-
(2007)
J Biol Chem
, vol.282
, Issue.50
, pp. 36481-36488
-
-
Berdiev, B.K.1
Cormet-Boyaka, E.2
Tousson, A.3
-
44
-
-
0034623128
-
The cytosolic termini of the β- and γ- ENaC subunits are involved in the functional interactions between CFTR and ENaC
-
Ji HL, Chalfant ML, Jovov B, et al. The cytosolic termini of the β- and γ- ENaC subunits are involved in the functional interactions between CFTR and ENaC. J Biol Chem. 2000;275(36):27947-27956.
-
(2000)
J Biol Chem
, vol.275
, Issue.36
, pp. 27947-27956
-
-
Ji, H.L.1
Chalfant, M.L.2
Jovov, B.3
-
45
-
-
0031024767
-
Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator
-
Kunzelmann K, Kiser GL, Schreiber R, Riordan JR. Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 1997;400(3):341-344.
-
(1997)
FEBS Lett
, vol.400
, Issue.3
, pp. 341-344
-
-
Kunzelmann, K.1
Kiser, G.L.2
Schreiber, R.3
Riordan, J.R.4
-
46
-
-
0037283010
-
CAMP-dependent activation of CFTR inhibits the epithelial sodium channel (ENaC) without affecting its surface expression
-
Konstas AA, Koch JP, Korbmacher C. cAMP-dependent activation of CFTR inhibits the epithelial sodium channel (ENaC) without affecting its surface expression. Pflügers Arch. 2003;445(4):513-521.
-
(2003)
Pflügers Arch
, vol.445
, Issue.4
, pp. 513-521
-
-
Konstas, A.A.1
Koch, J.P.2
Korbmacher, C.3
-
47
-
-
0028276637
-
Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: A patch-clamp study
-
Chinet TC, Fullton JM, Yankaskas JR, Boucher RC, Stutts MJ. Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: a patch-clamp study. Am J Physiol Cell Physiol. 1994;266(4):C1061-C1068.
-
(1994)
Am J Physiol Cell Physiol
, vol.266
, Issue.4
, pp. C1061-C1068
-
-
Chinet, T.C.1
Fullton, J.M.2
Yankaskas, J.R.3
Boucher, R.C.4
Stutts, M.J.5
-
48
-
-
77957767326
-
The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na(+) channel
-
Gentzsch M, Dang H, Dang Y, et al. The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na(+) channel. J Biol Chem. 2010;285(42):32227-32232.
-
(2010)
J Biol Chem
, vol.285
, Issue.42
, pp. 32227-32232
-
-
Gentzsch, M.1
Dang, H.2
Dang, Y.3
-
49
-
-
35648949295
-
CFTR stabilizes ENaC at the plasma membrane
-
Lu C, Jiang C, Pribanic S, Rotin D. CFTR stabilizes ENaC at the plasma membrane. J Cyst Fibros. 2007;6(6):419-422.
-
(2007)
J Cyst Fibros
, vol.6
, Issue.6
, pp. 419-422
-
-
Lu, C.1
Jiang, C.2
Pribanic, S.3
Rotin, D.4
-
50
-
-
0037274990
-
ENaC-CFTR interactions: The role of electrical coupling of ion fluxes explored in an epithelial cell model
-
Horisberger J-D. ENaC-CFTR interactions: the role of electrical coupling of ion fluxes explored in an epithelial cell model. Pflügers Arch. 2003;445(4):522-528.
-
(2003)
Pflügers Arch
, vol.445
, Issue.4
, pp. 522-528
-
-
Horisberger, J.-D.1
-
51
-
-
0037279932
-
- channels
-
- channels. Pflügers Arch. 2003;445(4):504-512.
-
(2003)
Pflügers Arch
, vol.445
, Issue.4
, pp. 504-512
-
-
Kunzelmann, K.1
-
52
-
-
84864521244
-
Airway epithelial cells - Functional links between CFTR and anoctamin dependent Cl− secretion
-
Kunzelmann K, Tian Y, Martins JR, et al. Airway epithelial cells - functional links between CFTR and anoctamin dependent Cl− secretion. Int J Biochem Cell Biol. 2012;44(11):1897-1900.
-
(2012)
Int J Biochem Cell Biol
, vol.44
, Issue.11
, pp. 1897-1900
-
-
Kunzelmann, K.1
Tian, Y.2
Martins, J.R.3
-
53
-
-
84901635712
-
Functional interaction of the cystic fibrosis transmembrane conductance regulator with members of the SLC26 family of anion transporters (SLC26A8 and SLC26A9): Physiological and pathophysiological relevance
-
El Khouri E, Touré A. Functional interaction of the cystic fibrosis transmembrane conductance regulator with members of the SLC26 family of anion transporters (SLC26A8 and SLC26A9): physiological and pathophysiological relevance. Int J Biochem Cell Biol. 2014;52:58-67.
-
(2014)
Int J Biochem Cell Biol
, vol.52
, pp. 58-67
-
-
El Khouri, E.1
Touré, A.2
-
54
-
-
51149118485
-
Characterization of SLC26A9, facilitation of Cl- transport by bicarbonate
-
Loriol C, Dulong S, Avella M, et al. Characterization of SLC26A9, facilitation of Cl- transport by bicarbonate. Cell Physiol Biochem. 2008;22(1-4):015-030.
-
(2008)
Cell Physiol Biochem
, vol.22
, Issue.1-4
, pp. 015-030
-
-
Loriol, C.1
Dulong, S.2
Avella, M.3
-
55
-
-
64549085967
-
SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia
-
Bertrand CA, Zhang R, Pilewski JM, Frizzell RA. SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia. J Gen Physiol. 2009;133(4):421-438.
-
(2009)
J Gen Physiol
, vol.133
, Issue.4
, pp. 421-438
-
-
Bertrand, C.A.1
Zhang, R.2
Pilewski, J.M.3
Frizzell, R.A.4
-
56
-
-
84857478183
-
Differential contribution of SLC26A9 to Cl− conductance in polarized and non-polarized epithelial cells
-
Ousingsawat J, Schreiber R, Kunzelmann K. Differential contribution of SLC26A9 to Cl− conductance in polarized and non-polarized epithelial cells. J Gen Physiol. 2012;227(6):2323-2329.
-
(2012)
J Gen Physiol
, vol.227
, Issue.6
, pp. 2323-2329
-
-
Ousingsawat, J.1
Schreiber, R.2
Kunzelmann, K.3
-
57
-
-
0033618404
-
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis: A novel class of mutation
-
Haardt M, Benharouga M, Lechardeur D, Kartner N, Lukacs GL. C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis: a novel class of mutation. J Biol Chem. 1999;274(31):21873-21877.
-
(1999)
J Biol Chem
, vol.274
, Issue.31
, pp. 21873-21877
-
-
Haardt, M.1
Benharouga, M.2
Lechardeur, D.3
Kartner, N.4
Lukacs, G.L.5
-
58
-
-
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(7):1251-1254.
-
(1993)
Cell
, vol.73
, Issue.7
, pp. 1251-1254
-
-
Welsh, M.J.1
Smith, A.E.2
-
59
-
-
22044440151
-
Mutation spectrum in Jewish cystic fibrosis patients in Israel: Implication to carrier screening
-
Quint A, Lerer I, Sagi M, Abeliovich D. Mutation spectrum in Jewish cystic fibrosis patients in Israel: implication to carrier screening. Am J Med Genet A. 2005;136A(3):246-248.
-
(2005)
Am J Med Genet A
, vol.136A
, Issue.3
, pp. 246-248
-
-
Quint, A.1
Lerer, I.2
Sagi, M.3
Abeliovich, D.4
-
60
-
-
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(4):827-834.
-
(1990)
Cell
, vol.63
, Issue.4
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
-
61
-
-
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(6389):761-764.
-
(1992)
Nature
, vol.358
, Issue.6389
, pp. 761-764
-
-
Denning, G.M.1
Erson, M.P.2
Amara, J.F.3
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
62
-
-
0036258208
-
Cystic fibrosis: A worldwide analysis of CFTR mutations - Correlation with incidence data and application to screening
-
Bobadilla JL, Macek M, Fine JP, Farrell PM. Cystic fibrosis: a worldwide analysis of CFTR mutations - correlation with incidence data and application to screening. Hum Mutat. 2002;19(6):575-606.
-
(2002)
Hum Mutat
, vol.19
, Issue.6
, pp. 575-606
-
-
Bobadilla, J.L.1
Macek, M.2
Fine, J.P.3
Farrell, P.M.4
-
63
-
-
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(6354):526-528.
-
(1991)
Nature
, vol.354
, Issue.6354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
-
64
-
-
41149113942
-
Enhanced cell surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator by pharmacological chaperones
-
Varga K, Goldstein RF, Jurkuvenaite A, et al. Enhanced cell surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator by pharmacological chaperones. Biochem J. 2008;410(3):555-564.
-
(2008)
Biochem J
, vol.410
, Issue.3
, pp. 555-564
-
-
Varga, K.1
Goldstein, R.F.2
Jurkuvenaite, A.3
-
65
-
-
84992067869
-
Natural compounds as therapeutic agents in the treatment cystic fibrosis
-
Dey I, Shah K, Bradbury NA. Natural compounds as therapeutic agents in the treatment cystic fibrosis. J Genet Syndr Gene Ther. 2016;7(1):284.
-
(2016)
J Genet Syndr Gene Ther
, vol.7
, Issue.1
, pp. 284
-
-
Dey, I.1
Shah, K.2
Bradbury, N.A.3
-
66
-
-
0026337305
-
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes
-
Drumm M, Wilkinson D, Smit L, et al. Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. Science. 1991;254(5039):1797-1799.
-
(1991)
Science
, vol.254
, Issue.5039
, pp. 1797-1799
-
-
Drumm, M.1
Wilkinson, D.2
Smit, L.3
-
67
-
-
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 USA. 1994;91(19):9160-9164.
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, Issue.19
, pp. 9160-9164
-
-
Becq, F.1
Jensen, T.J.2
Chang, X.B.3
-
68
-
-
0027220387
-
Possible regulation of CFTR-chloride channels by membrane-bound phosphatases in pancreatic duct cells
-
Becq F, Fanjul M, Merten M, Figarella C, Hollande E, Gola M. Possible regulation of CFTR-chloride channels by membrane-bound phosphatases in pancreatic duct cells. FEBS Lett. 1993;327(3):337-342.
-
(1993)
FEBS Lett
, vol.327
, Issue.3
, pp. 337-342
-
-
Becq, F.1
Fanjul, M.2
Merten, M.3
Figarella, C.4
Hollande, E.5
Gola, M.6
-
69
-
-
0031990988
-
Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel
-
Chappe V, Mettey Y, Vierfond JM, et al. Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel. Br J Pharmacol. 1998;123(4):683-693.
-
(1998)
Br J Pharmacol
, vol.123
, Issue.4
, pp. 683-693
-
-
Chappe, V.1
Mettey, Y.2
Vierfond, J.M.3
-
70
-
-
0030943895
-
8-Cyclopentyl-1,3-dipropylx-anthine and other xanthines differentially bind to the wild-type and ΔF508 mutant first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator
-
Cohen BE, Lee G, Jacobson KA, et al. 8-Cyclopentyl-1,3-dipropylx-anthine and other xanthines differentially bind to the wild-type and ΔF508 mutant first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator. Biochemistry. 1997;36(21):6455-6461.
-
(1997)
Biochemistry
, vol.36
, Issue.21
, pp. 6455-6461
-
-
Cohen, B.E.1
Lee, G.2
Jacobson, K.A.3
-
71
-
-
0031763141
-
Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo
-
Illek B, Fischer H. Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo. Am J Physiol Lung Cell Mol Physiol. 1998;275(5):L902-L910.
-
(1998)
Am J Physiol Lung Cell Mol Physiol
, vol.275
, Issue.5
, pp. L902-L910
-
-
Illek, B.1
Fischer, H.2
-
72
-
-
0030850643
-
Genistein activates CFTR Cl- channels via a tyrosine kinase- and protein phosphatase-independent mechanism
-
French PJ, Bijman J, Bot AG, Boomaars WE, Scholte BJ, de Jonge HR. Genistein activates CFTR Cl- channels via a tyrosine kinase- and protein phosphatase-independent mechanism. Am J Physiol Cell Physiol. 1997;273(2):C747-C753.
-
(1997)
Am J Physiol Cell Physiol
, vol.273
, Issue.2
, pp. C747-C753
-
-
French, P.J.1
Bijman, J.2
Bot, A.G.3
Boomaars, W.E.4
Scholte, B.J.5
De Jonge, H.R.6
-
73
-
-
0028928015
-
CAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein
-
Illek B, Fischer H, Santos GF, Widdicombe JH, Machen TE, Reenstra WW. cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein. Am J Physiol Cell Physiol. 1995;268(4):C886-C893.
-
(1995)
Am J Physiol Cell Physiol
, vol.268
, Issue.4
, pp. C886-C893
-
-
Illek, B.1
Fischer, H.2
Santos, G.F.3
Widdicombe, J.H.4
Machen, T.E.5
Reenstra, W.W.6
-
74
-
-
0030773897
-
Genistein potentiates wild-type and delta F508-CFTR channel activity
-
Hwang TC, Wang F, Yang IC, Reenstra WW. Genistein potentiates wild-type and delta F508-CFTR channel activity. Am J Physiol. 1997;273(3 pt 1):C988-C998.
-
(1997)
Am J Physiol
, vol.273
, Issue.3
, pp. C988-C998
-
-
Hwang, T.C.1
Wang, F.2
Yang, I.C.3
Reenstra, W.W.4
-
75
-
-
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 Cell Physiol. 1999;277(4): C833-C839.
-
(1999)
Am J Physiol Cell Physiol
, vol.277
, Issue.4
, pp. C833-C839
-
-
Illek, B.1
Zhang, L.2
Lewis, N.C.3
Moss, R.B.4
Dong, J.Y.5
Fischer, H.6
-
76
-
-
15044353957
-
Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains
-
Moran O, Galietta LJV, 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(4):446-460.
-
(2005)
Cell Mol Life Sci
, vol.62
, Issue.4
, pp. 446-460
-
-
Moran, O.1
Galietta, L.2
Zegarra-Moran, O.3
-
77
-
-
33947543364
-
Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains
-
Wang W, Bernard K, Li G, Kirk KL. Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains. J Biol Chem. 2007;282(7):4533-4544.
-
(2007)
J Biol Chem
, vol.282
, Issue.7
, pp. 4533-4544
-
-
Wang, W.1
Bernard, K.2
Li, G.3
Kirk, K.L.4
-
78
-
-
0036115199
-
Calcium-pump inhibitors induce functional surface expression of delF508-CFTR protein in cystic fibrosis epithelial cells
-
Egan ME, Glockner-Pagel J, Ambrose CA, et al. Calcium-pump inhibitors induce functional surface expression of delF508-CFTR protein in cystic fibrosis epithelial cells. Nat Med. 2002;8(5):485-492.
-
(2002)
Nat Med
, vol.8
, Issue.5
, pp. 485-492
-
-
Egan, M.E.1
Glockner-Pagel, J.2
Ambrose, C.A.3
-
79
-
-
33645235405
-
Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells
-
Norez C, Antigny F, Becq F, Vandebrouck C. Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells. Traffic. 2006;7(5): 562-573.
-
(2006)
Traffic
, vol.7
, Issue.5
, pp. 562-573
-
-
Norez, C.1
Antigny, F.2
Becq, F.3
Vandebrouck, C.4
-
80
-
-
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(5670): 600-602.
-
(2004)
Science
, vol.304
, Issue.5670
, pp. 600-602
-
-
Egan, M.E.1
Pearson, M.2
Weiner, S.A.3
-
81
-
-
4644360693
-
Evidence against the rescue of defective ΔF508-CFTR cellular processing by curcumin in cell culture and mouse models
-
Song Y, Sonawane ND, Salinas D, et al. Evidence against the rescue of defective ΔF508-CFTR cellular processing by curcumin in cell culture and mouse models. J Biol Chem. 2004;279(39):40629-40633.
-
(2004)
J Biol Chem
, vol.279
, Issue.39
, pp. 40629-40633
-
-
Song, Y.1
Sonawane, N.D.2
Salinas, D.3
-
82
-
-
33644625310
-
SERCA pump inhibitors do not correct biosynthetic arrest of ΔF508 CFTR in cystic fibrosis
-
Grubb BR, Gabriel SE, Mengos A, et al. SERCA pump inhibitors do not correct biosynthetic arrest of ΔF508 CFTR in cystic fibrosis. Am J Respir Cell Mol Biol. 2006;34(3):355-363.
-
(2006)
Am J Respir Cell Mol Biol
, vol.34
, Issue.3
, pp. 355-363
-
-
Grubb, B.R.1
Gabriel, S.E.2
Mengos, A.3
-
83
-
-
84884732389
-
CFTR chloride channel is a molecular target of the natural cancer preventive agent resveratrol
-
Yang S, Yu B, Sui Y, et al. CFTR chloride channel is a molecular target of the natural cancer preventive agent resveratrol. Pharmazie. 2013;68(9):772-776.
-
(2013)
Pharmazie
, vol.68
, Issue.9
, pp. 772-776
-
-
Yang, S.1
Yu, B.2
Sui, Y.3
-
84
-
-
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(6):C1838-C1846.
-
(2000)
Am J Physiol Cell Physiol
, vol.279
, Issue.6
, pp. C1838-C1846
-
-
Illek, B.1
Lizarzaburu, M.E.2
Lee, V.3
Nantz, M.H.4
Kurth, M.J.5
Fischer, H.6
-
85
-
-
79958270724
-
Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium
-
Alexander NS, Hatch N, Zhang S, et al. Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium. Laryngoscope. 2011;121(6):1313-1319.
-
(2011)
Laryngoscope
, vol.121
, Issue.6
, pp. 1313-1319
-
-
Alexander, N.S.1
Hatch, N.2
Zhang, S.3
-
86
-
-
84979769205
-
Resveratrol increases F508del-CFTR dependent salivary secretion in cystic fibrosis mice
-
Dhooghe B, Bouckaert C, Capron A, Wallemacq P, Leal T, Noel S. Resveratrol increases F508del-CFTR dependent salivary secretion in cystic fibrosis mice. Biol Open. 2015;4(7):929-936.
-
(2015)
Biol Open
, vol.4
, Issue.7
, pp. 929-936
-
-
Dhooghe, B.1
Bouckaert, C.2
Capron, A.3
Wallemacq, P.4
Leal, T.5
Noel, S.6
-
87
-
-
84942191864
-
Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia-induced model of acquired CFTR deficiency
-
Woodworth BA. Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia-induced model of acquired CFTR deficiency. Laryngoscope. 2015;125(suppl 7):S1-S13.
-
(2015)
Laryngoscope
, vol.125
, pp. SS1-S13
-
-
Woodworth, B.A.1
-
88
-
-
79958046677
-
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1
-
Hamdaoui N, Baudoin-Legros M, Kelly M, et al. Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1. Br J Pharmacol. 2011;163(4):876-886.
-
(2011)
Br J Pharmacol
, vol.163
, Issue.4
, pp. 876-886
-
-
Hamdaoui, N.1
Baudoin-Legros, M.2
Kelly, M.3
-
89
-
-
84941595661
-
Evidence against resveratrol as a viable therapy for the rescue of defective ΔF508 CFTR
-
Jai Y, Shah K, Bridges RJ, Bradbury NA. Evidence against resveratrol as a viable therapy for the rescue of defective ΔF508 CFTR. Biochim Biophys Acta. 2015;1850(11):2377-2384.
-
(2015)
Biochim Biophys Acta
, vol.1850
, Issue.11
, pp. 2377-2384
-
-
Jai, Y.1
Shah, K.2
Bridges, R.J.3
Bradbury, N.A.4
-
91
-
-
0037180511
-
Chemical chaperones increase the cellular activity of N370S β-glucosidase: A therapeutic strategy for Gaucher disease
-
Sawkar AR, Cheng WC, Beutler E, Wong CH, Balch WE, Kelly JW. Chemical chaperones increase the cellular activity of N370S β-glucosidase: a therapeutic strategy for Gaucher disease. Proc Natl Acad Sci USA. 2002;99(24):15428-15433.
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, Issue.24
, pp. 15428-15433
-
-
Sawkar, A.R.1
Cheng, W.C.2
Beutler, E.3
Wong, C.H.4
Balch, W.E.5
Kelly, J.W.6
-
92
-
-
44249097535
-
Parallel improvement of sodium and chloride transport defects by miglustat (N-butyl-deoxynojyrimicin) in cystic fibrosis epithelial cells
-
Noël S, Wilke M, Bot AGM, De Jonge HR, Becq F. Parallel improvement of sodium and chloride transport defects by miglustat (n-butyl-deoxynojyrimicin) in cystic fibrosis epithelial cells. J Pharmacol Exp Ther. 2008;325(3):1016-1023.
-
(2008)
J Pharmacol Exp Ther
, vol.325
, Issue.3
, pp. 1016-1023
-
-
Noël, S.1
Wilke, M.2
Bot, A.3
De Jonge, H.R.4
Becq, F.5
-
93
-
-
33645211759
-
Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the α-glucosidase inhibitor miglustat
-
Norez C, Noel S, Wilke M, et al. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the α-glucosidase inhibitor miglustat. FEBS Lett. 2006;580(8):2081-2086.
-
(2006)
FEBS Lett
, vol.580
, Issue.8
, pp. 2081-2086
-
-
Norez, C.1
Noel, S.2
Wilke, M.3
-
94
-
-
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(11): 1022-1028.
-
(2009)
Am J Respir Crit Care Med
, vol.179
, Issue.11
, pp. 1022-1028
-
-
Lubamba, B.1
Lebacq, J.2
Lebecque, P.3
-
95
-
-
84860605804
-
Randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference
-
Leonard A, Lebecque P, Dingemanse J, Leal T. A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference. J Cyst Fibros. 2012;11(3):231-236.
-
(2012)
J Cyst Fibros
, vol.11
, Issue.3
, pp. 231-236
-
-
Leonard, A.1
Lebecque, P.2
Dingemanse, J.3
Leal, T.A.4
-
96
-
-
84930015224
-
Emerging drugs for the treatment of erectile dysfunction
-
Peak TC, Yafi FA, Sangkum P, Hellstrom WJG. Emerging drugs for the treatment of erectile dysfunction. Expert Opin Emerg Drugs. 2015;20(2):263-275.
-
(2015)
Expert Opin Emerg Drugs
, vol.20
, Issue.2
, pp. 263-275
-
-
Peak, T.C.1
Yafi, F.A.2
Sangkum, P.3
Hellstrom, W.4
-
97
-
-
84949085676
-
Riociguat for the treatment of pulmonary hypertension
-
Hambly N, Granton J. Riociguat for the treatment of pulmonary hypertension. Expert Rev Respir Med. 2015;9(6):679-695.
-
(2015)
Expert Rev Respir Med
, vol.9
, Issue.6
, pp. 679-695
-
-
Hambly, N.1
Granton, J.2
-
98
-
-
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(5-6):775-790.
-
(2012)
Cell Physiol Biochem
, vol.29
, Issue.5-6
, pp. 775-790
-
-
Leier, G.1
Bangel-Ruland, N.2
Sobczak, K.3
Knieper, Y.4
Weber, W.M.5
-
99
-
-
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(1):55-59.
-
(2005)
Thorax
, vol.60
, Issue.1
, pp. 55-59
-
-
Dormer, R.L.1
Harris, C.M.2
Clark, Z.3
-
100
-
-
84886248189
-
Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice
-
Dhooghe B, Noël S, Bouzin C, Behets-Wydemans G, Leal T. Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice. PLoS One. 2013;8(10):e77314.
-
(2013)
Plos One
, vol.8
, Issue.10
-
-
Dhooghe, B.1
Noël, S.2
Bouzin, C.3
Behets-Wydemans, G.4
Leal, T.5
-
101
-
-
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(5):506-515.
-
(2008)
Am J Respir Crit Care Med
, vol.177
, Issue.5
, pp. 506-515
-
-
Lubamba, B.1
Lecourt, H.2
Lebacq, J.3
-
102
-
-
84924257957
-
Pharmacokinetics and tolerability of oral sildenafil in adults with cystic fibrosis lung disease
-
Taylor-Cousar JL, Wiley C, Felton LA, et al. Pharmacokinetics and tolerability of oral sildenafil in adults with cystic fibrosis lung disease. J Cyst Fibros. 2015;14(2):228-236.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.2
, pp. 228-236
-
-
Taylor-Cousar, J.L.1
Wiley, C.2
Felton, L.A.3
-
103
-
-
84880721470
-
Riociguat for the treatment of pulmonary arterial hypertension
-
Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4): 330-340.
-
(2013)
N Engl J Med
, vol.369
, Issue.4
, pp. 330-340
-
-
Ghofrani, H.A.1
Galiè, N.2
Grimminger, F.3
-
104
-
-
67651094166
-
Discovery of riociguat (BAY 63-2521): A potent, oral stimulator of soluble guanylate cyclase for the treatment of pulmonary hypertension
-
Mittendorf J, Weigand S, Alonso-Alija C, et al. Discovery of riociguat (BAY 63-2521): a potent, oral stimulator of soluble guanylate cyclase for the treatment of pulmonary hypertension. ChemMedChem. 2009;4(5):853-865.
-
(2009)
Chemmedchem
, vol.4
, Issue.5
, pp. 853-865
-
-
Mittendorf, J.1
Weigand, S.2
Alonso-Alija, C.3
-
105
-
-
36848999782
-
Clinical use of ibuprofen is associated with slower FEV(1) decline in children with cystic fibrosis
-
Konstan MW, Schluchter MD, Xue W, Davis PB. Clinical use of ibuprofen is associated with slower FEV(1) decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2007;176(11): 1084-1089.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, Issue.11
, pp. 1084-1089
-
-
Konstan, M.W.1
Schluchter, M.D.2
Xue, W.3
Davis, P.B.4
-
106
-
-
84918785468
-
Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking
-
Carlile GW, Robert R, Goepp J, et al. Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking. J Cyst Fibros. 2015;14(1):16-25.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.1
, pp. 16-25
-
-
Carlile, G.W.1
Robert, R.2
Goepp, J.3
-
107
-
-
0347320495
-
Early oral cysteamine therapy for nephropathic cystinosis
-
Gahl WA. Early oral cysteamine therapy for nephropathic cystinosis. Eur J Pediatr. 2003;162(1):S38-S41.
-
(2003)
Eur J Pediatr
, vol.162
, Issue.1
, pp. S38-S41
-
-
Gahl, W.A.1
-
108
-
-
77956396747
-
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition
-
Luciani A, Villella VR, Esposito S, et al. Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition. Nat Cell Biol. 2010;12(9):863-875.
-
(2010)
Nat Cell Biol
, vol.12
, Issue.9
, pp. 863-875
-
-
Luciani, A.1
Villella, V.R.2
Esposito, S.3
-
109
-
-
80655134731
-
Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis
-
Abdulrahman BA, Khweek AA, Akhter A, et al. Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis. Autophagy. 2011;7(11):1359-1370.
-
(2011)
Autophagy
, vol.7
, Issue.11
, pp. 1359-1370
-
-
Abdulrahman, B.A.1
Khweek, A.A.2
Akhter, A.3
-
110
-
-
84869389296
-
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator
-
Luciani A, Villella VR, Esposito S, et al. Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator. Autophagy. 2012;8(11):1657-1672.
-
(2012)
Autophagy
, vol.8
, Issue.11
, pp. 1657-1672
-
-
Luciani, A.1
Villella, V.R.2
Esposito, S.3
-
111
-
-
84919764939
-
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation
-
Stefano DD, Villella VR, Esposito S, et al. Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Autophagy. 2014;10(11):2053-2074.
-
(2014)
Autophagy
, vol.10
, Issue.11
, pp. 2053-2074
-
-
Stefano, D.D.1
Villella, V.R.2
Esposito, S.3
-
112
-
-
84962121102
-
A novel treatment of cystic fibrosis acting on-target: Cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR
-
Epub, Jul 22
-
Tosco A, De Gregorio F, Esposito S, et al. A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR. Cell Death Differ. Epub 2016 Jul 22.
-
(2016)
Cell Death Differ
-
-
Tosco, A.1
De Gregorio, F.2
Esposito, S.3
-
113
-
-
0034828555
-
Aescin: Pharmacology, pharmacokinetics and therapeutic profile
-
Sirtori CR. Aescin: pharmacology, pharmacokinetics and therapeutic profile. Pharmacol Res. 2001;44(3):183-193.
-
(2001)
Pharmacol Res
, vol.44
, Issue.3
, pp. 183-193
-
-
Sirtori, C.R.1
-
114
-
-
84969178328
-
Discovery of clinically approved agents that promote suppression of CFTR nonsense mutations
-
Apr 22
-
Mutyam V, Du M, Xue X, et al. Discovery of clinically approved agents that promote suppression of CFTR nonsense mutations. Am J Respir Crit Care Med. Epub 2016 Apr 22.
-
(2016)
Am J Respir Crit Care Med. Epub
-
-
Mutyam, V.1
Du, M.2
Xue, X.3
-
115
-
-
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(40):37235-37241.
-
(2002)
J Biol Chem
, vol.277
, Issue.40
, pp. 37235-37241
-
-
Ma, T.1
Vetrivel, L.2
Yang, H.3
-
116
-
-
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(9):2564-2571.
-
(2005)
J Clin Invest
, vol.115
, Issue.9
, pp. 2564-2571
-
-
Pedemonte, N.1
Lukacs, G.L.2
Du, K.3
-
117
-
-
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(6): L1117-L1130.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
, Issue.6
, pp. L1117-L1130
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
-
118
-
-
34247200483
-
Vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: A pilot study
-
Sermet-Gaudelus I, Renouil M, Fajac A, et al. In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study. BMC Med. 2007;5:5-5.
-
(2007)
BMC Med
, vol.5
-
-
Sermet-Gaudelus, I.1
Renouil, M.2
Fajac, A.3
-
119
-
-
0029994529
-
Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations
-
Howard M, Frizzell RA, Bedwell DM. Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations. Nat Med. 1996;2(4):467-469.
-
(1996)
Nat Med
, vol.2
, Issue.4
, pp. 467-469
-
-
Howard, M.1
Frizzell, R.A.2
Bedwell, D.M.3
-
120
-
-
0030702773
-
Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line
-
Bedwell DM, Kaenjak A, Benos DJ, et al. Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nat Med. 1997;3(11):1280-1284.
-
(1997)
Nat Med
, vol.3
, Issue.11
, pp. 1280-1284
-
-
Bedwell, D.M.1
Kaenjak, A.2
Benos, D.J.3
-
121
-
-
0141863491
-
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
-
Wilschanski M, Yahav Y, Yaacov Y, et al. Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N Engl J Med. 2003;349(15):1433-1441.
-
(2003)
N Engl J Med
, vol.349
, Issue.15
, pp. 1433-1441
-
-
Wilschanski, M.1
Yahav, Y.2
Yaacov, Y.3
-
122
-
-
0034073736
-
A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations
-
Wilschanski M, Famini C, Blau H, et al. A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations. Am J Respir Crit Care Med. 2000;161(3):860-865.
-
(2000)
Am J Respir Crit Care Med
, vol.161
, Issue.3
, pp. 860-865
-
-
Wilschanski, M.1
Famini, C.2
Blau, H.3
-
123
-
-
0034961464
-
Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis
-
Clancy JP, Bebök Z, Ruiz F, et al. Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis. Am J Respir Crit Care Med. 2001;163(7):1683-1692.
-
(2001)
Am J Respir Crit Care Med
, vol.163
, Issue.7
, pp. 1683-1692
-
-
Clancy, J.P.1
Bebök, Z.2
Ruiz, F.3
-
124
-
-
34347224756
-
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
-
Clancy JP, Rowe SM, Bebok Z, et al. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol. 2007;37(1):57-66.
-
(2007)
Am J Respir Cell Mol Biol
, vol.37
, Issue.1
, pp. 57-66
-
-
Clancy, J.P.1
Rowe, S.M.2
Bebok, Z.3
-
125
-
-
65649136885
-
Development of novel aminoglycoside (NB54) with reduced toxicity and enhanced suppression of disease-causing premature stop mutations
-
Nudelman I, Rebibo-Sabbah A, Cherniavsky M, et al. Development of novel aminoglycoside (NB54) with reduced toxicity and enhanced suppression of disease-causing premature stop mutations. J Med Chem. 2009;52(9):2836-2845.
-
(2009)
J Med Chem
, vol.52
, Issue.9
, pp. 2836-2845
-
-
Nudelman, I.1
Rebibo-Sabbah, A.2
Cherniavsky, M.3
-
126
-
-
84965035945
-
Design of novel aminoglycoside derivatives with enhanced suppression of diseases-causing nonsense mutations
-
Sabbavarapu NM, Shavit M, Degani Y, Smolkin B, Belakhov V, Baasov T. Design of novel aminoglycoside derivatives with enhanced suppression of diseases-causing nonsense mutations. ACS Med Chem Lett. 2016;7(4):418-423.
-
(2016)
ACS Med Chem Lett
, vol.7
, Issue.4
, pp. 418-423
-
-
Sabbavarapu, N.M.1
Shavit, M.2
Degani, Y.3
Smolkin, B.4
Belakhov, V.5
Baasov, T.6
-
127
-
-
84898715557
-
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor
-
Xue X, Mutyam V, Tang L, et al. Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. Am J Respir Cell Mol Biol. 2014;50(4):805-816.
-
(2014)
Am J Respir Cell Mol Biol
, vol.50
, Issue.4
, pp. 805-816
-
-
Xue, X.1
Mutyam, V.2
Tang, L.3
-
128
-
-
80755133472
-
Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54
-
Rowe SM, Sloane P, Tang LP, et al. Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54. J Mol Med (Berl). 2011;89(11): 1149-1161.
-
(2011)
J Mol Med (Berl)
, vol.89
, Issue.11
, pp. 1149-1161
-
-
Rowe, S.M.1
Sloane, P.2
Tang, L.P.3
-
129
-
-
0036379141
-
-/- mouse carrying a human CFTR-G542X transgene
-
-/- mouse carrying a human CFTR-G542X transgene. J Mol Med (Berl). 2002;80(9):595-604.
-
(2002)
J Mol Med (Berl)
, vol.80
, Issue.9
, pp. 595-604
-
-
Du, M.1
Jones, J.R.2
Lanier, J.3
-
130
-
-
33751064254
-
Drug evaluation: PTC-124: A potential treatment of cystic fibrosis and Duchenne muscular dystrophy
-
Hamed S. Drug evaluation: PTC-124: a potential treatment of cystic fibrosis and Duchenne muscular dystrophy. IDrugs. 2006;9(11):783-789.
-
(2006)
Idrugs
, vol.9
, Issue.11
, pp. 783-789
-
-
Hamed, S.1
-
131
-
-
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(9640):719-727.
-
(2008)
Lancet
, vol.372
, Issue.9640
, pp. 719-727
-
-
Kerem, E.1
Hirawat, S.2
Armoni, S.3
-
132
-
-
78349290383
-
Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis
-
Sermet-Gaudelus I, De Boeck K, Casimir GJ, et al. Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis. Am J Respir Crit Care Med. 2010;182(10):1262-1272.
-
(2010)
Am J Respir Crit Care Med
, vol.182
, Issue.10
, pp. 1262-1272
-
-
Sermet-Gaudelus, I.1
De Boeck, K.2
Casimir, G.J.3
-
133
-
-
84904016376
-
Cystic Fibrosis Ataluren Study Group. Ataluren for the treatment of nonsense-mutation cystic fibrosis: A randomised, double-blind, placebo-controlled phase 3 trial
-
Kerem E, Konstan MW, De Boeck K, et al; Cystic Fibrosis Ataluren Study Group. Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial. Lancet Respir Med. 2014;2(7):539-547.
-
(2014)
Lancet Respir Med
, vol.2
, Issue.7
, pp. 539-547
-
-
Kerem, E.1
Konstan, M.W.2
De Boeck, K.3
-
134
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the ΔF508 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 ΔF508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones. 1996;1(2):117-125.
-
(1996)
Cell Stress Chaperones
, vol.1
, Issue.2
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
Verkman, A.S.4
Welch, W.J.5
-
135
-
-
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(2):635-638.
-
(1996)
J Biol Chem
, vol.271
, Issue.2
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
136
-
-
0032871691
-
Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells
-
Moyer BD, Loffing-Cueni D, Loffing J, Reynolds D, Stanton BA. Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells. Am J Physiol. 1999;277(2):F271-F276.
-
(1999)
Am J Physiol
, vol.277
, Issue.2
, pp. F271-F276
-
-
Moyer, B.D.1
Loffing-Cueni, D.2
Loffing, J.3
Reynolds, D.4
Stanton, B.A.5
-
137
-
-
0030809817
-
Vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
-
Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest. 1997;100(10):2457-2465.
-
(1997)
J Clin Invest
, vol.100
, Issue.10
, pp. 2457-2465
-
-
Rubenstein, R.C.1
Egan, M.E.2
Zeitlin, P.L.3
-
138
-
-
0034099743
-
Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of ΔF508-CFTR
-
Rubenstein RC, Zeitlin PL. Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of ΔF508-CFTR. Am J Physiol Cell Physiol. 2000;278(2):C259-C267.
-
(2000)
Am J Physiol Cell Physiol
, vol.278
, Issue.2
, pp. C259-C267
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
139
-
-
0031889082
-
Pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in Δ F508-homozygous cystic fibrosis patients
-
Rubenstein R, Zeitlin P. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in Δ F508-homozygous cystic fibrosis patients. Am J Respir Crit Care Med. 1998;157(2):484-490.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, Issue.2
, pp. 484-490
-
-
Rubenstein, R.1
Zeitlin, P.A.2
-
140
-
-
46249118125
-
Correctors promote folding of the CFTR in the endoplasmic reticulum
-
Loo T, Bartlett M, Clarke D. Correctors promote folding of the CFTR in the endoplasmic reticulum. Biochem J. 2008;413(1):29-36.
-
(2008)
Biochem J
, vol.413
, Issue.1
, pp. 29-36
-
-
Loo, T.1
Bartlett, M.2
Clarke, D.3
-
141
-
-
33645530653
-
The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants
-
Loo T, Bartlett M, Wang Y, Clarke D. The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants. Biochem J. 2006;395(pt 3):537-542.
-
(2006)
Biochem J
, vol.395
, pp. 537-542
-
-
Loo, T.1
Bartlett, M.2
Wang, Y.3
Clarke, D.4
-
142
-
-
77950428804
-
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis
-
Hutt DM, Herman D, Rodrigues APC, et al. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol. 2010;6(1):25-33.
-
(2010)
Nat Chem Biol
, vol.6
, Issue.1
, pp. 25-33
-
-
Hutt, D.M.1
Herman, D.2
Rodrigues, A.3
-
143
-
-
79951829938
-
Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR
-
Sampson H, Robert R, Liao J, et al. Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR. Chem Biol. 2011;18(2):231-242.
-
(2011)
Chem Biol
, vol.18
, Issue.2
, pp. 231-242
-
-
Sampson, H.1
Robert, R.2
Liao, J.3
-
144
-
-
84884773595
-
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
-
Ren HY, Grove DE, De La Rosa O, et al. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol Biol Cell. 2013;24(19):3016-3024.
-
(2013)
Mol Biol Cell
, vol.24
, Issue.19
, pp. 3016-3024
-
-
Ren, H.Y.1
Grove, D.E.2
De La Rosa, O.3
-
145
-
-
81755163563
-
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
-
Van Goor F, Hadida S, Grootenhuis PDJ, 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(46):18843-18848.
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, Issue.46
, pp. 18843-18848
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.3
-
146
-
-
84855202429
-
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound
-
Clancy JP, Rowe SM, Accurso FJ, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax. 2012;67(1):12-18.
-
(2012)
Thorax
, vol.67
, Issue.1
, pp. 12-18
-
-
Clancy, J.P.1
Rowe, S.M.2
Accurso, F.J.3
-
147
-
-
84904002908
-
VX09-809-102 Study Group. A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: A phase 2 randomised controlled trial
-
Boyle MP, Bell SC, Konstan MW, et al; VX09-809-102 Study Group. A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir Med. 2014;2(7):527-538.
-
(2014)
Lancet Respir Med
, vol.2
, Issue.7
, pp. 527-538
-
-
Boyle, M.P.1
Bell, S.C.2
Konstan, M.W.3
-
148
-
-
84937035647
-
Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR.
-
Wainwright CE, Elborn JS, Ramsey BW, et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373(3):220-231.
-
(2015)
N Engl J Med
, vol.373
, Issue.3
, pp. 220-231
-
-
Wainwright, C.E.1
Elborn, J.S.2
Ramsey, B.W.3
-
149
-
-
84939225425
-
Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: Should we curb our enthusiasm?
-
Jones AM, Barry PJ. Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm? Thorax. 2015;70(7):615-616.
-
(2015)
Thorax
, vol.70
, Issue.7
, pp. 615-616
-
-
Jones, A.M.1
Barry, P.J.2
-
151
-
-
84907223425
-
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
-
Cholon DM, Quinney NL, Fulcher ML, et al. Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis. Sci Transl Med. 2014;6(246):246ra296.
-
(2014)
Sci Transl Med
, vol.6
, Issue.246
-
-
Cholon, D.M.1
Quinney, N.L.2
Fulcher, M.L.3
-
152
-
-
84907211027
-
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression
-
Veit G, Avramescu RG, Perdomo D, et al. Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression. Sci Transl Med. 2014;6(246):246ra297.
-
(2014)
Sci Transl Med
, vol.6
, Issue.246
-
-
Veit, G.1
Avramescu, R.G.2
Perdomo, D.3
-
153
-
-
84952021538
-
WS01.4 VX-661 in combination with ivacaftor in patients with cystic fibrosis and the F508del-CFTR mutation
-
Pilewski JM, Cooke J, Lekstrom-Himes J, Donaldson S. WS01.4 VX-661 in combination with ivacaftor in patients with cystic fibrosis and the F508del-CFTR mutation. J Cyst Fibros. 2015;14:S1.
-
(2015)
J Cyst Fibros
, vol.14
-
-
Pilewski, J.M.1
Cooke, J.2
Lekstrom-Himes, J.3
Donaldson, S.4
-
154
-
-
84982170519
-
WS01.2 QR-010, an RNA therapy, restores CFTR function using in vitro and in vivo models of delF508 CFTR
-
Beumer W, Swildens J, Henig N, et al. WS01.2 QR-010, an RNA therapy, restores CFTR function using in vitro and in vivo models of delF508 CFTR. J Cyst Fibros. 2015;14:S1.
-
(2015)
J Cyst Fibros
, vol.14
-
-
Beumer, W.1
Swildens, J.2
Henig, N.3
-
155
-
-
85073736996
-
QR-010, an RNA Therapy, Restores CFTR Function in the Saliva Secretion Assay
-
New York, NY: American Thoracic Society
-
Henig N, Beumer W, Anthonijsz H, et al. QR-010, an RNA Therapy, Restores CFTR Function in the Saliva Secretion Assay. A37. It Won’t be Long: Advances in Adult Cystic Fibrosis. New York, NY: American Thoracic Society; 2015:A1449-A1449.
-
(2015)
A37. It Won’t Be Long: Advances in Adult Cystic Fibrosis
, pp. A1449-A1449
-
-
Henig, N.1
Beumer, W.2
Anthonijsz, H.3
-
156
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
Van Goor F, Hadida S, Grootenhuis PDJ, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci USA. 2009;106(44):18825-18830.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, Issue.44
, pp. 18825-18830
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.3
-
157
-
-
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(21):1991-2003.
-
(2010)
N Engl J Med
, vol.363
, Issue.21
, pp. 1991-2003
-
-
Accurso, F.J.1
Rowe, S.M.2
Clancy, J.P.3
-
158
-
-
84885071273
-
Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: A randomised controlled trial
-
Davies J, Sheridan H, Bell N, et al. Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. Lancet Respir Med. 2013;1(8):630-638.
-
(2013)
Lancet Respir Med
, vol.1
, Issue.8
, pp. 630-638
-
-
Davies, J.1
Sheridan, H.2
Bell, N.3
-
159
-
-
80455162465
-
VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365(18):1663-1672.
-
(2011)
N Engl J Med
, vol.365
, Issue.18
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
-
160
-
-
84911493956
-
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation
-
De Boeck K, Munck A, Walker S, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros. 2014;13(6):674-680.
-
(2014)
J Cyst Fibros
, vol.13
, Issue.6
, pp. 674-680
-
-
De Boeck, K.1
Munck, A.2
Walker, S.3
-
161
-
-
84958107856
-
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): An open-label, single-arm study
-
Davies JC, Cunningham S, Harris WT, et al. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Lancet Respir Med. 2016;4(2):107-115.
-
(2016)
Lancet Respir Med
, vol.4
, Issue.2
, pp. 107-115
-
-
Davies, J.C.1
Cunningham, S.2
Harris, W.T.3
-
162
-
-
84865853894
-
VX 08-770-104 Study Group. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
-
Flume PA, Liou TG, Borowitz DS, et al; VX 08-770-104 Study Group. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Chest. 2012;142(3):718-724.
-
(2012)
Chest
, vol.142
, Issue.3
, pp. 718-724
-
-
Flume, P.A.1
Liou, T.G.2
Borowitz, D.S.3
-
163
-
-
84992100293
-
WS13.6 CTP-656 tablet confirmed superiority of pharmacokinetic profile relative to Kalydeco® in phase I clinical studies
-
Uttamsineh V, Pilja L, Grotbeck B, et al. WS13.6 CTP-656 tablet confirmed superiority of pharmacokinetic profile relative to Kalydeco® in phase I clinical studies. J Cyst Fibros. 2016; 15(S1):S22.
-
(2016)
J Cyst Fibros
, vol.15
-
-
Uttamsineh, V.1
Pilja, L.2
Grotbeck, B.3
-
164
-
-
85043640362
-
QBW251 is a safe and efficacious CFTR potentiator for patients with cystic fibrosis
-
Shamsah K, Jose A, Laurie D, et al. QBW251 is a safe and efficacious CFTR potentiator for patients with cystic fibrosis. Am J Respir Crit Care Med. 2016;193:A7789.
-
(2016)
Am J Respir Crit Care Med
, vol.193
-
-
Shamsah, K.1
Jose, A.2
Laurie, D.3
-
165
-
-
85043637298
-
Safety, tolerability and pharmacokinetics of a novel CFTR potentiator GPLG1837 in healthy volunteers
-
Vanhoutte FP, Gouy M, Haazen W, et al. Safety, tolerability and pharmacokinetics of a novel CFTR potentiator GPLG1837 in healthy volunteers. Pediatr Pulmonol. 2015;50(S41):S289.
-
(2015)
Pediatr Pulmonol
, vol.50
-
-
Vanhoutte, F.P.1
Gouy, M.2
Haazen, W.3
-
166
-
-
77954939833
-
Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy
-
Marozkina NV, Yemen S, Borowitz M, et al. Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy. Proc Natl Acad Sci USA. 2010;107(25):11393-11398.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, Issue.25
, pp. 11393-11398
-
-
Marozkina, N.V.1
Yemen, S.2
Borowitz, M.3
-
167
-
-
84956899678
-
Augmentation of CFTR maturation by S-nitrosoglutathione reductase
-
Zaman K, Sawczak V, Zaidi A, et al. Augmentation of CFTR maturation by S-nitrosoglutathione reductase. Am J Physiol Lung Cell Mol Physiol. 2016;310(3):L263-L270.
-
(2016)
Am J Physiol Lung Cell Mol Physiol
, vol.310
, Issue.3
, pp. L263-L270
-
-
Zaman, K.1
Sawczak, V.2
Zaidi, A.3
-
168
-
-
33748944330
-
S-nitrosylating agents: A novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells
-
Zaman K, Carraro S, Doherty J, et al. S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells. Mol Pharmacol. 2006;70(4):1435-1442.
-
(2006)
Mol Pharmacol
, vol.70
, Issue.4
, pp. 1435-1442
-
-
Zaman, K.1
Carraro, S.2
Doherty, J.3
-
169
-
-
84969191949
-
The pharmacokinetics of N91115, an inhibitor of S-nitrosoglutathione reductase in cystic fibrosis patients
-
Taylor-Cousar J, Zemanick E, Solomon G. The pharmacokinetics of N91115, an inhibitor of S-nitrosoglutathione reductase in cystic fibrosis patients. Pediatr Pulmonol. 2015;50(S41):S285-S286.
-
(2015)
Pediatr Pulmonol
, vol.50
, pp. S285-S286
-
-
Taylor-Cousar, J.1
Zemanick, E.2
Solomon, G.3
-
170
-
-
85043642209
-
Amplifiers are a new class of CFTR modulators that increase the abundance of CFTR protein and combined with potentiators and correctors enhance chloride transport activity
-
Miller J, Drew L, Green O, et al. Amplifiers are a new class of CFTR modulators that increase the abundance of CFTR protein and combined with potentiators and correctors enhance chloride transport activity. Pediatr Pulmonol. 2015;50(S41):S265.
-
(2015)
Pediatr Pulmonol
, vol.50
-
-
Miller, J.1
Drew, L.2
Green, O.3
-
171
-
-
84961828393
-
Strategies in early clinical development for the treatment of basic defects of cystic fibrosis
-
Dhooghe B, Haaf JB, Noel S, Leal T. Strategies in early clinical development for the treatment of basic defects of cystic fibrosis. Expert Opin Investig Drugs. 2016;25(4):423-436.
-
(2016)
Expert Opin Investig Drugs
, vol.25
, Issue.4
, pp. 423-436
-
-
Dhooghe, B.1
Haaf, J.B.2
Noel, S.3
Leal, T.4
|