-
1
-
-
0028309683
-
Human airway ion transport: Part one
-
Boucher RC. Human airway ion transport: part one. Am. J. Respir. Crit. Care Med. 150, 271-281 (1994).
-
(1994)
Am. J. Respir. Crit. Care Med.
, vol.150
, pp. 271-281
-
-
Boucher, R.C.1
-
2
-
-
0027998618
-
Human airway ion transport: Part two
-
Boucher RC. Human airway ion transport: part two. Am. J. Respir. Crit Care Med. 150, 581-593 (1994).
-
(1994)
Am. J. Respir. Crit. Care Med.
, vol.150
, pp. 581-593
-
-
Boucher, R.C.1
-
3
-
-
0022526266
-
Mechanics of lung fluid balance
-
Lai-Fook SJ. Mechanics of lung fluid balance. Crit. Rev. Biomed. Eng. 13 (3), 171-200 (1986).
-
(1986)
Crit. Rev. Biomed. Eng.
, vol.13
, Issue.3
, pp. 171-200
-
-
Lai-Fook, S.J.1
-
4
-
-
36148956756
-
Alveolar epithelial transport in the adult lung
-
Dobbs LG, Johnson MD. Alveolar epithelial transport in the adult lung. Respir. Physiol. Neurobiol. 159 (3), 283-300 (2007).
-
(2007)
Respir. Physiol. Neurobiol.
, vol.159
, Issue.3
, pp. 283-300
-
-
Dobbs, L.G.1
Johnson, M.D.2
-
6
-
-
33845992179
-
Evidence for airway surface dehydration as the initiating event in CF airway disease
-
• Reviews data supporting the 'low-volume' hypothesis for cystic fibrosis
-
Boucher RC. Evidence for airway surface dehydration as the initiating event in CF airway disease. J. Intern. Med. 261 (1), 5-16 (2007). • Reviews data supporting the 'low-volume' hypothesis for cystic fibrosis.
-
(2007)
J. Intern. Med.
, vol.261
, Issue.1
, pp. 5-16
-
-
Boucher, R.C.1
-
7
-
-
0014108774
-
Studies of pulmonary secretions. II. Osmolarity and the ionic environment of pulmonary secretions from patients with cystic fibrosis, bronchiectasis and laryngectomy
-
Potter JL, Matthews LW, Spector S, Lemm J. Studies of pulmonary secretions. II. Osmolarity and the ionic environment of pulmonary secretions from patients with cystic fibrosis, bronchiectasis and laryngectomy. Am. Rev. Respir. Dis. 96, 83-87 (1967).
-
(1967)
Am. Rev. Respir. Dis.
, vol.96
, pp. 83-87
-
-
Potter, J.L.1
Matthews, L.W.2
Spector, S.3
Lemm, J.4
-
8
-
-
0033565228
-
Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism
-
+ channel ENaC loss-of-function mutations
-
+ channel (ENaC) loss-of-function mutations.
-
(1999)
N. Engl. J. Med.
, vol.341
, Issue.3
, pp. 156-162
-
-
Kerem, E.1
Bistritzer, T.2
Hanukoglu, A.3
-
9
-
-
0029916071
-
Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis
-
Robinson M, Regnis JA, Bailey DL, King M, Bautovich GJ, Bye PT. Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis. Am. J. Respir. Crit. Care Med. 153, 1503-1509 (1996).
-
(1996)
Am. J. Respir. Crit. Care Med.
, vol.153
, pp. 1503-1509
-
-
Robinson, M.1
Regnis, J.A.2
Bailey, D.L.3
King, M.4
Bautovich, G.J.5
Bye, P.T.6
-
10
-
-
0032834277
-
The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: A pilot study
-
Robinson M, Daviskas E, Eberl S et al. The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: a pilot study. Eur. Respir. J. 14 (3), 678-685 (1999).
-
(1999)
Eur. Respir. J.
, vol.14
, Issue.3
, pp. 678-685
-
-
Robinson, M.1
Daviskas, E.2
Eberl, S.3
-
11
-
-
0029921103
-
Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis
-
Eng PA, Morton J, Douglass JA, Riedler J, Wilson J, Robertson CF. Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis. Pediatr. Pulmonol. 21, 77-83 (1996).
-
(1996)
Pediatr. Pulmonol.
, vol.21
, pp. 77-83
-
-
Eng, P.A.1
Morton, J.2
Douglass, J.A.3
Riedler, J.4
Wilson, J.5
Robertson, C.F.6
-
12
-
-
44649166466
-
Inhaled mannitol improves lung function in cystic fibrosis
-
Jaques A, Daviskas E, Turton JA et al Inhaled mannitol improves lung function in cystic fibrosis. Chest 133 (6), 1388-1396 (2008).
-
(2008)
Chest
, vol.133
, Issue.6
, pp. 1388-1396
-
-
Jaques, A.1
Daviskas, E.2
Turton, J.A.3
-
13
-
-
30944466084
-
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. National hypertonic saline in cystic Fibrosis (NHSCF) study group
-
• Important clinical proof of concept for improving airway hydration in cystic fibrosis
-
Elkins MR, Robinson M, Rose BR et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. National Hypertonic Saline in Cystic Fibrosis (NHSCF) study group. N. Engl. J. Med. 354 (3), 229-240 (2006). • Important clinical proof of concept for improving airway hydration in cystic fibrosis.
-
(2006)
N. Engl. J. Med.
, vol.354
, Issue.3
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
-
14
-
-
77952558917
-
Randomized, double blind, placebo controlled Phase III study of inhaled dry powder mannitol (bronchitol) in CF
-
Bilton D, Robinson P, Cooper P, Charlton B. Randomized, double blind, placebo controlled Phase III study of inhaled dry powder mannitol (bronchitol) in CF. Pediatr. Pulmonol. 44 (Suppl. 32), 286 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.32 SUPPL.
, pp. 286
-
-
Bilton, D.1
Robinson, P.2
Cooper, P.3
Charlton, B.4
-
15
-
-
0024453308
-
Identification of the cystic fibrosis gene: Chromosome walking and jumping
-
Rommens JM, Iannuzzi MC, Kerem B et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245 (4922), 1059-1065 (1989).
-
(1989)
Science
, vol.245
, Issue.4922
, pp. 1059-1065
-
-
Rommens, J.M.1
Iannuzzi, M.C.2
Kerem, B.3
-
16
-
-
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 245 (4922), 1066-1073 (1989).
-
(1989)
Science
, vol.245
, Issue.4922
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
-
17
-
-
33750010667
-
Gene therapy for cystic fibrosis lung disease: Current status and future perspectives
-
Rosenecker J, Huth S, Rudolph C. Gene therapy for cystic fibrosis lung disease: current status and future perspectives. Curr. Opin. Mol. Ther. 8 (5), 439-445 (2006).
-
(2006)
Curr. Opin. Mol. Ther.
, vol.8
, Issue.5
, pp. 439-445
-
-
Rosenecker, J.1
Huth, S.2
Rudolph, C.3
-
18
-
-
11144241287
-
Aerosols of compacted DNA nanoparticles retain structural integrity and biological activity
-
Oette S, Gedeon CR, Kowalczyk TH et al. Aerosols of compacted DNA nanoparticles retain structural integrity and biological activity. Mol. Ther. 9, S190-S190 (2004).
-
(2004)
Mol. Ther.
, vol.9
-
-
Oette, S.1
Gedeon, C.R.2
Kowalczyk, T.H.3
-
19
-
-
0033586375
-
Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: A double-blind placebo-controlled trial
-
Alton EW, Stern M, Farley R et al. Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial. Lancet 353 (9157), 947-954 (1999).
-
(1999)
Lancet
, vol.353
, Issue.9157
, pp. 947-954
-
-
Alton, E.W.1
Stern, M.2
Farley, R.3
-
20
-
-
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 358 (6389), 761-764 (1992).
-
(1992)
Nature
, vol.358
, Issue.6389
, 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
-
21
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the δ F508 cystic fibrosis mutation
-
Dalemans W, Barbry P, Champigny G et al. Altered chloride ion channel kinetics associated with the δ F508 cystic fibrosis mutation. Nature 354 (6354), 526-528 (1991).
-
(1991)
Nature
, vol.354
, Issue.6354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
-
22
-
-
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. 290 (6), L1117-L1130 (2006).
-
(2006)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.290
, Issue.6
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
-
23
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
• Overview of key in vitro data supporting VX-770
-
Van Goor F, Hadida S, Grootenhuis PD et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc. Natl Acad. Sci. USA 106 (44), 18825-18830 (2009). • Overview of key in vitro data supporting VX-770.
-
(2009)
Proc. Natl. Acad. Sci. USA
, vol.106
, Issue.44
, pp. 18825-18830
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
24
-
-
77952566610
-
Effect of VX-770, a CFTR potentiator, on spirometry and QOL assessment in subjects with CF and the G551D-CFTR mutation
-
Boyle M, Clancy JP, Rowe SM et al. Effect of VX-770, a CFTR potentiator, on spirometry and QOL assessment in subjects with CF and the G551D-CFTR mutation. Pediatr. Pulmonol. 44 (Suppl. 32), 287 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.32 SUPPL.
, pp. 287
-
-
Boyle, M.1
Clancy, J.P.2
Rowe, S.M.3
-
25
-
-
77952559992
-
Improvement in sweat chloride concentration by the CFTR potentiator VX-770 in subjects with cystic fibrosis and the G551D-CFTR mutation
-
Accurso F, Rowe SM, Durie PR et al. Improvement in sweat chloride concentration by the CFTR potentiator VX-770 in subjects with cystic fibrosis and the G551D-CFTR mutation. Pediatr. Pulmonol. 44 (Suppl. 32), 296 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.32 SUPPL.
, pp. 296
-
-
Accurso, F.1
Rowe, S.M.2
Durie, P.R.3
-
26
-
-
77952574720
-
NPD evaluation of ion transport in G551D CF patients treated with a CFTR potentiator
-
Clancy JP, Rowe SM, Durie P et al. NPD evaluation of ion transport in G551D CF patients treated with a CFTR potentiator. Pediatr. Pulmonol. 44 (Suppl. 32), 288-289 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.32 SUPPL.
, pp. 288-289
-
-
Clancy, J.P.1
Rowe, S.M.2
Durie, P.3
-
28
-
-
0038663174
-
Effect of genotype on phenotype and mortality in cystic fibrosis: A retrospective cohort study
-
McKone EF, Emerson SS, Edwards KL, Aitken ML. Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet 361 (9370), 1671-1676 (2003).
-
(2003)
Lancet
, vol.361
, Issue.9370
, pp. 1671-1676
-
-
McKone, E.F.1
Emerson, S.S.2
Edwards, K.L.3
Aitken, M.L.4
-
29
-
-
0033724681
-
Lung disease associated with the IVS8 5T allele of the CFTR gene
-
Noone PG, Pue CA, Zhou Z et al. Lung disease associated with the IVS8 5T allele of the CFTR gene. Am. J. Respir. Crit. Care Med. 162 (5), 1919-1924 (2000).
-
(2000)
Am. J. Respir. Crit. Care Med.
, vol.162
, Issue.5
, pp. 1919-1924
-
-
Noone, P.G.1
Pue, C.A.2
Zhou, Z.3
-
30
-
-
0035213536
-
Cystic fibrosis gene mutations and pancreatitis risk: Relation to epithelial ion transport and trypsin inhibitor gene mutations
-
Noone PG, Zhou Z, Silverman LM, Jowell PS, Knowles MR, Cohn JA. Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. Gastroenterology 121 (6), 1310-1309 (2001).
-
(2001)
Gastroenterology
, vol.121
, Issue.6
, pp. 1310-1309
-
-
Noone, P.G.1
Zhou, Z.2
Silverman, L.M.3
Jowell, P.S.4
Knowles, M.R.5
Cohn, J.A.6
-
31
-
-
70349778630
-
VX-809, a CFTR corrector, increases the cell surface density of functional F508del-CFTR in pre-clinical models of cystic fibrosis
-
Van Goor F, Hadida S, Grootenhuis PD et al. VX-809, a CFTR corrector, increases the cell surface density of functional F508del-CFTR in pre-clinical models of cystic fibrosis. Pediatr. Pulmonol 44 (Suppl. 32), 154 (2009)
-
(2009)
Pediatr. Pulmonol
, vol.44
, Issue.32 SUPPL.
, pp. 154
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
33
-
-
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. 163 (7), 1683-1692 (2001).
-
(2001)
Am. J. Respir. Crit. Care Med.
, vol.163
, Issue.7
, pp. 1683-1692
-
-
Clancy, J.P.1
Bebök, Z.2
Ruiz, F.3
-
34
-
-
50149098401
-
Effectiveness of PTC 124 treatment of cystic fibrosis caused by nonsense mutations: A prospective Phase II trial
-
Kerem E, Hirawat S, Armoni S et al. Effectiveness of PTC 124 treatment of cystic fibrosis caused by nonsense mutations: a prospective Phase II trial. Lancet 372 (9640), 719-727 (2008).
-
(2008)
Lancet
, vol.372
, Issue.9640
, pp. 719-727
-
-
Kerem, E.1
Hirawat, S.2
Armoni, S.3
-
35
-
-
54849413018
-
Introducing sense into nonsense in treatments of human genetic disease
-
Linde L, Kerem B. Introducing sense into nonsense in treatments of human genetic disease. Trends Genet. 24 (11), 552-563 (2008).
-
(2008)
Trends Genet.
, vol.24
, Issue.11
, pp. 552-563
-
-
Linde, L.1
Kerem, B.2
-
36
-
-
0024465128
-
Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C- and A-dependent mechanisms
-
Boucher RC, Cheng EH, Paradiso AM, Stutts MJ, Knowles MR, Earp HS. Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C- and A-dependent mechanisms. J. Clin. Invest. 84 (5), 1424-1431 (1989).
-
(1989)
J. Clin. Invest.
, vol.84
, Issue.5
, pp. 1424-1431
-
-
Boucher, R.C.1
Cheng, E.H.2
Paradiso, A.M.3
Stutts, M.J.4
Knowles, M.R.5
Earp, H.S.6
-
37
-
-
0025871551
-
Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia
-
Anderson MP, Welsh MJ. Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia. Proc. Natl Acad. Sci. USA 88 (14), 6003-6007 (1991).
-
(1991)
Proc. Natl. Acad. Sci. USA
, vol.88
, Issue.14
, pp. 6003-6007
-
-
Anderson, M.P.1
Welsh, M.J.2
-
39
-
-
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. 325 (8), 533-538 (1991).
-
(1991)
N. Engl. J. Med.
, vol.325
, Issue.8
, pp. 533-538
-
-
Knowles, M.R.1
Clarke, L.L.2
Boucher, R.C.3
-
40
-
-
0035165915
-
Polarized signaling via purinoceptors in normal and cystic fibrosis airway epithelia
-
Paradiso AM, Ribeiro CMP, Boucher RC. Polarized signaling via purinoceptors in normal and cystic fibrosis airway epithelia. J. Gen. Physiol. 117 (1), 53-67 (2001).
-
(2001)
J. Gen. Physiol.
, vol.117
, Issue.1
, pp. 53-67
-
-
Paradiso, A.M.1
Ribeiro, C.M.P.2
Boucher, R.C.3
-
41
-
-
0036720462
-
Pharmacology of INS37217 [P (1) - (uridine 5) -P (4) - (2'-deoxycytidine 5) tetraphosphate, tetrasodium salt], a nextgeneration P2Y (2) receptor agonist for the treatment of cystic fibrosis
-
Yerxa BR, Sabater JR, Davis CW et al. Pharmacology of INS37217 [P (1) - (uridine 5) -P (4) - (2'-deoxycytidine 5) tetraphosphate, tetrasodium salt], a nextgeneration P2Y (2) receptor agonist for the treatment of cystic fibrosis. J. Pharmacol Exp. Ther. 302 (3), 871-880 (2002).
-
(2002)
J. Pharmacol. Exp. Ther.
, vol.302
, Issue.3
, pp. 871-880
-
-
Yerxa, B.R.1
Sabater, J.R.2
Davis, C.W.3
-
42
-
-
18344392104
-
2 receptor agonist, in patients with mild to moderate cystic fibrosis: Results of a Phase 2 multi-center study
-
2 receptor agonist, in patients with mild to moderate cystic fibrosis: results of a Phase 2 multi-center study. Pediatr. Pulmonol. 38 (S27), 249 (2004).
-
(2004)
Pediatr. Pulmonol.
, vol.38
, Issue.S27
, pp. 249
-
-
Deterding, R.R.1
LaVange, L.2
Matthews, D.3
-
43
-
-
20144363443
-
2 receptor agonist: Results of a Phase 1/Phase 2 multicenter study in mild to moderate cystic fibrosis
-
2 receptor agonist: results of a Phase 1/Phase 2 multicenter study in mild to moderate cystic fibrosis. Pediatr. Pulmonol 39 (4), 339-348 (2005).
-
(2005)
Pediatr. Pulmonol
, vol.39
, Issue.4
, pp. 339-348
-
-
Deterding, R.1
Retsch-Bogart, G.2
Milgram, L.3
-
44
-
-
34547942924
-
Denufosol tetrasodium inhalation solution: Results from two Phase 2 trials in CF patients with mild to moderate lung disease
-
Smiley L, Rossi A, Matthews D et al. Denufosol tetrasodium inhalation solution: results from two Phase 2 trials in CF patients with mild to moderate lung disease. J. Cyst. Fibros. 5 (Suppl. 1), S11 (2006).
-
(2006)
J. Cyst. Fibros.
, vol.5
, Issue.1 SUPPL.
-
-
Smiley, L.1
Rossi, A.2
Matthews, D.3
-
45
-
-
66949114840
-
The TIGER-1 Phase 3 clinical trial of denufosol for cystic fibrosis
-
Accurso F. The TIGER-1 Phase 3 clinical trial of denufosol for cystic fibrosis. Pediatr. Pulmonol. 44 (S31), 191 (2008).
-
(2008)
Pediatr. Pulmonol.
, vol.44
, Issue.S31
, pp. 191
-
-
Accurso, F.1
-
47
-
-
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 322 (5901), 590-594 (2008).
-
(2008)
Science
, vol.322
, Issue.5901
, pp. 590-594
-
-
Caputo, A.1
Caci, E.2
Ferrera, L.3
-
48
-
-
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 134 (6), 1019-1029 (2008).
-
(2008)
Cell.
, vol.134
, Issue.6
, pp. 1019-1029
-
-
Schroeder, B.C.1
Cheng, T.2
Jan, Y.N.3
Jan, L.Y.4
-
49
-
-
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 455 (7217), 1210-1215 (2008).
-
(2008)
Nature
, vol.455
, Issue.7217
, pp. 1210-1215
-
-
Yang, Y.D.1
Cho, H.2
Koo, J.Y.3
-
50
-
-
70350367890
-
2+-dependent chloride transport
-
2+-dependent chloride transport. J. Biol. Chem. 284 (42), 28698-28703 (2009).
-
(2009)
J. Biol. Chem.
, vol.284
, Issue.42
, pp. 28698-28703
-
-
Ousingsawat, J.1
Martins, J.R.2
Schreiber, R.3
Rock, J.R.4
Harfe, B.D.5
Kunzelmann, K.6
-
51
-
-
49049118639
-
The transmembrane protein TMEM16A is required for normal development of the murine trachea
-
Rock JR, Futtner CR, Harfe BD. The transmembrane protein TMEM16A is required for normal development of the murine trachea. Dev. Biol. 321 (1), 141-149 (2008).
-
(2008)
Dev. Biol.
, vol.321
, Issue.1
, pp. 141-149
-
-
Rock, J.R.1
Futtner, C.R.2
Harfe, B.D.3
-
52
-
-
77952556946
-
Small-molecule activators and inhibitors of calcium-activated chloride channel TMEM16A
-
Namkung W, Carpenter RD, Verkman AS. Small-molecule activators and inhibitors of calcium-activated chloride channel TMEM16A. Pediatr. Pulmonol. 44 (S32), 288 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.S32
, pp. 288
-
-
Namkung, W.1
Carpenter, R.D.2
Verkman, A.S.3
-
53
-
-
34248597371
-
Inhalation of Moli1901 in patients with cystic fibrosis
-
Grasemann H, Stehling F, Brunar H. Inhalation of Moli1901 in patients with cystic fibrosis. Chest 131 (5), 1461-1466 (2007).
-
(2007)
Chest
, vol.131
, Issue.5
, pp. 1461-1466
-
-
Grasemann, H.1
Stehling, F.2
Brunar, H.3
-
54
-
-
77952568469
-
A randomized, double-blind, placebo-controlled, dose-finding study to evaluate the efficacy and safety of aerosolized lancovutide (moli1901) in adolescents (12 years of age or older) and adults with CF
-
Eber E, Trawinska-Bartnicka M, Sands DA et al. A randomized, double-blind, placebo-controlled, dose-finding study to evaluate the efficacy and safety of aerosolized lancovutide (moli1901) in adolescents (12 years of age or older) and adults with CF. Pediatr. Pulmonol. 44 (S32), 302 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.S32
, pp. 302
-
-
Eber, E.1
Trawinska-Bartnicka, M.2
Sands, D.A.3
-
56
-
-
73949143060
-
INO-4995 therapeutic efficacy is enhanced with repeat dosing in CF KO mice and human epithelia
-
Traynor-Kaplan AE, Moody M, Nur M et al. INO-4995 therapeutic efficacy is enhanced with repeat dosing in CF KO mice and human epithelia. Am. J. Respir. Cell Mol. Biol. 42 (1), 105-112 (2010).
-
(2010)
Am. J. Respir. Cell. Mol. Biol.
, vol.42
, Issue.1
, pp. 105-112
-
-
Traynor-Kaplan, A.E.1
Moody, M.2
Nur, M.3
-
57
-
-
0032518665
-
The heterotetrameric architecture of the epithelial sodium channel (ENaC)
-
Firsov D, Gautschi I, Merillat AM, Rossier BC, Schild L. The heterotetrameric architecture of the epithelial sodium channel (ENaC). EMBO J. 17 (2), 344-352 (1998).
-
(1998)
EMBO J.
, vol.17
, Issue.2
, pp. 344-352
-
-
Firsov, D.1
Gautschi, I.2
Merillat, A.M.3
Rossier, B.C.4
Schild, L.5
-
59
-
-
2442718786
-
+ absorption produces cystic fibrosis-like lung disease in mice
-
• β-ENaC overexpressing transgenic mouse with CF-like lung phenotype
-
+ absorption produces cystic fibrosis-like lung disease in mice. Nat. Med. 10 (5), 487-493 (2004). • β-ENaC overexpressing transgenic mouse with CF-like lung phenotype.
-
(2004)
Nat. Med.
, vol.10
, Issue.5
, pp. 487-493
-
-
Mall, M.1
Grubb, B.R.2
Harkema, J.R.3
O'Neal, W.K.4
Boucher, R.C.5
-
60
-
-
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. 178 (12), 1245-1256 (2008).
-
(2008)
Am. J. Respir. Crit. Care Med.
, vol.178
, Issue.12
, pp. 1245-1256
-
-
Zhou, Z.1
Treis, D.2
Schubert, S.C.3
-
61
-
-
0019490443
-
Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo
-
Knowles MR, Carson JL, Collier AM, Gatzy JT, Boucher RC. Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo. Am. Rev. Respir. Dis. 124 (4), 484-490 (1981).
-
(1981)
Am. Rev. Respir. Dis.
, vol.124
, Issue.4
, pp. 484-490
-
-
Knowles, M.R.1
Carson, J.L.2
Collier, A.M.3
Gatzy, J.T.4
Boucher, R.C.5
-
62
-
-
0028020058
-
Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium
-
Middleton PG, Geddes DM, Alton EW Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium. Eur. Respir. J. 7 (11), 2050-2056 (1994).
-
(1994)
Eur. Respir. J.
, vol.7
, Issue.11
, pp. 2050-2056
-
-
Middleton, P.G.1
Geddes, D.M.2
Alton, E.W.3
-
63
-
-
8544273222
-
Nasal airway ion transport is linked to the cystic fibrosis phenotype in adult patients
-
Fajac I, Hubert D, Guillemot D et al. Nasal airway ion transport is linked to the cystic fibrosis phenotype in adult patients. Thorax 59 (11), 971-976 (2004).
-
(2004)
Thorax
, vol.59
, Issue.11
, pp. 971-976
-
-
Fajac, I.1
Hubert, D.2
Guillemot, D.3
-
64
-
-
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. 141 (3), 605-612 (1990).
-
(1990)
Am. Rev. Respir. Dis.
, vol.141
, Issue.3
, pp. 605-612
-
-
App, E.M.1
King, M.2
Helfesrieder, R.3
Köhler, D.4
Matthys, H.5
-
65
-
-
0022988338
-
Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses
-
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 fibroses. Eur. J. Respir. Dis. Suppl. 146, 319-326 (1986).
-
(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
-
66
-
-
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. 322 (17), 1189-1194 (1990).
-
(1990)
N. Engl. J. Med.
, vol.322
, Issue.17
, pp. 1189-1194
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
-
67
-
-
0034047238
-
French multicenter randomized doubleblind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients
-
Amiloride-AFLM Collaborative Study Group
-
Pons G, Marchand MC, d'Athis P et al. French multicenter randomized doubleblind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group. Pediatr. Pulmonol. 30 (1), 25-31 (2000).
-
(2000)
Pediatr. Pulmonol.
, vol.30
, Issue.1
, pp. 25-31
-
-
Pons, G.1
Marchand, M.C.2
D'Athis, P.3
-
68
-
-
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 73 (5), 427-430 (1995).
-
(1995)
Arch. Dis. Child
, vol.73
, Issue.5
, pp. 427-430
-
-
Bowler, I.M.1
Kelman, B.2
Worthington, D.3
-
69
-
-
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. 6 (9), 1243-1248 (1993).
-
(1993)
Eur. Respir. J.
, vol.6
, Issue.9
, pp. 1243-1248
-
-
Graham, A.1
Hasani, A.2
Alton, E.W.3
-
70
-
-
0030663247
-
Airway deposition and clearance and systemic pharmacokinetics of amiloride following aerosolization with an ultrasonic nebulizer to normal airways
-
Noone PG, Regnis JA, Liu X et al. Airway deposition and clearance and systemic pharmacokinetics of amiloride following aerosolization with an ultrasonic nebulizer to normal airways. Chest 112 (5), 1283-1290 (1997).
-
(1997)
Chest
, vol.112
, Issue.5
, pp. 1283-1290
-
-
Noone, P.G.1
Regnis, J.A.2
Liu, X.3
-
72
-
-
0030879756
-
An epithelial serine protease activates the amiloridesensitive sodium channel
-
• Identification of a channel activating protease involved in regulating ENaC activity
-
Vallet V, Chraibi A, Gaeggeler HP, Horisberger JD, Rossier BC. An epithelial serine protease activates the amiloridesensitive sodium channel. Nature 389 (6651), 607-610 (1997). • Identification of a channel activating protease involved in regulating ENaC activity.
-
(1997)
Nature
, vol.389
, Issue.6651
, pp. 607-610
-
-
Vallet, V.1
Chraibi, A.2
Gaeggeler, H.P.3
Horisberger, J.D.4
Rossier, B.C.5
-
73
-
-
0037040995
-
Regulation of the epithelial sodium channel by serine proteases in human airways
-
Donaldson SH, Hirsh A, Li DC, Holloway G, Chao J, Boucher RC, Gabriel SE. Regulation of the epithelial sodium channel by serine proteases in human airways. J. Biol. Chem. 277 (10), 8338-8345 (2002).
-
(2002)
J. Biol. Chem.
, vol.277
, Issue.10
, pp. 8338-8345
-
-
Donaldson, S.H.1
Hirsh, A.2
Li, D.C.3
Holloway, G.4
Chao, J.5
Boucher, R.C.6
Gabriel, S.E.7
-
74
-
-
45849124564
-
Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis
-
Myerburg MM, McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, Pilewski JM. Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am. J. Physiol. Lung Cell Mol. Physiol. 294 (5), L932-L941 (2008).
-
(2008)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.294
, Issue.5
-
-
Myerburg, M.M.1
McKenna, E.E.2
Luke, C.J.3
Frizzell, R.A.4
Kleyman, T.R.5
Pilewski, J.M.6
-
75
-
-
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. 329 (2), 764-774 (2009).
-
(2009)
J. Pharmacol. Exp. Ther.
, vol.329
, Issue.2
, pp. 764-774
-
-
Coote, K.1
Atherton-Watson, H.C.2
Sugar, R.3
-
76
-
-
70349766602
-
Correction of sodium transport with nasal administration of the prostasin inhibitor QAU145 in CF subjects
-
Rowe SM, Reeves G, Young H et al. Correction of sodium transport with nasal administration of the prostasin inhibitor QAU145 in CF subjects. Pediatr. Pulmonol. 44 (S32), 295 (2009).
-
(2009)
Pediatr. Pulmonol.
, vol.44
, Issue.S32
, pp. 295
-
-
Rowe, S.M.1
Reeves, G.2
Young, H.3
-
77
-
-
33750842131
-
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis
-
Wang X, Venable J, LaPointe P et al. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 127 (4), 803-815 (2006).
-
(2006)
Cell.
, vol.127
, Issue.4
, pp. 803-815
-
-
Wang, X.1
Venable, J.2
LaPointe, P.3
-
78
-
-
77950428804
-
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis
-
Hutt DM, Herman D, Rodrigues AP et al. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat. Chem. Biol. 6 (1), 25-33 (2010).
-
(2010)
Nat. Chem. Biol.
, vol.6
, Issue.1
, pp. 25-33
-
-
Hutt, D.M.1
Herman, D.2
Rodrigues, A.P.3
|