-
1
-
-
78549279173
-
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation
-
Accurso F.J., Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie P.R., Sagel SD, Hornick DB, Konstan MW, Donaldson S.H., et al. 2010. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 363: 1991-2003.
-
(2010)
N Engl J Med
, vol.363
, pp. 1991-2003
-
-
Accurso, F.J.1
Rowe, S.M.2
Clancy, J.P.3
Boyle, M.P.4
Dunitz, J.M.5
Durie, P.R.6
Sagel, S.D.7
Hornick, D.B.8
Konstan, M.W.9
Donaldson, S.H.10
-
2
-
-
79952211206
-
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function
-
TIGER-1 Investigator Study Group
-
Accurso F.J., Moss RB, Wilmott RW, Anbar RD, Schaberg AE, Durham T.A., Ramsey BW, TIGER-1 Investigator Study Group. 2011. Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function. Am J Respir Crit Care Med 183: 627-634.
-
(2011)
Am J Respir Crit Care Med
, vol.183
, pp. 627-634
-
-
Accurso, F.J.1
Moss, R.B.2
Wilmott, R.W.3
Anbar, R.D.4
Schaberg, A.E.5
Durham, T.A.6
Ramsey, B.W.7
-
3
-
-
0025871551
-
Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia
-
Anderson M.P., Welsh MJ. 1991. Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia. Proc Natl Acad Sci 88: 6003-6007.
-
(1991)
Proc Natl Acad Sci
, vol.88
, pp. 6003-6007
-
-
Anderson, M.P.1
Welsh, M.J.2
-
4
-
-
0029887172
-
Effect of uridine 50-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis
-
Bennett W.D., Olivier KN, Zeman KL, Hohneker KW, Boucher R.C., Knowles MR. 1996. Effect of uridine 50-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. Am J Respir Crit Care Med 153: 1796-1801.
-
(1996)
Am J Respir Crit Care Med
, vol.153
, pp. 1796-1801
-
-
Bennett, W.D.1
Olivier, K.N.2
Zeman, K.L.3
Hohneker, K.W.4
Boucher, R.C.5
Knowles, M.R.6
-
5
-
-
77952558917
-
Randomized, double blind, placebo-controlled phase iii study of inhaled dry powder mannitol (Bronchitol) in CF
-
BiltonD, Robinson P, Cooper P, Charlton B. 2009. Randomized, double blind, placebo-controlled phase iii study of inhaled dry powder mannitol (Bronchitol) in CF. Pediatr Pulmonol 44 (Suppl): A216.
-
(2009)
Pediatr Pulmonol
, vol.44
, Issue.SUPPL.
-
-
Bilton, D.1
Robinson, P.2
Cooper, P.3
Charlton, B.4
-
8
-
-
54949112835
-
TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity
-
Caputo A., Caci E, Ferrera L, Pedemonte N, Barsanti C, Sondo E., Pfeffer U, Ravazzolo R, Zegarra-Moran O, Galietta LJ. 2008. TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity. Science 322: 590-594.
-
(2008)
Science
, vol.322
, pp. 590-594
-
-
Caputo, A.1
Caci, E.2
Ferrera, L.3
Pedemonte, N.4
Barsanti, C.5
Sondo, E.6
Pfeffer, U.7
Ravazzolo, R.8
Zegarra-Moran, O.9
Galietta, L.J.10
-
9
-
-
0033111840
-
Desensitization of P2Y2 receptoractivated transepithelial anion secretion
-
Clarke L.L., Harline MC, Otero MA, Glover GG, Garrad RC, Krugh B, Walker NM, González FA, Turner JT, Weisman GA. 1999. Desensitization of P2Y2 receptoractivated transepithelial anion secretion. Am J Physiol 276: C777-C787.
-
(1999)
Am J Physiol
, vol.276
-
-
Clarke, L.L.1
Harline, M.C.2
Otero, M.A.3
Glover, G.G.4
Garrad, R.C.5
Krugh, B.6
Walker, N.M.7
González, F.A.8
Turner, J.T.9
Weisman, G.A.10
-
10
-
-
0346103648
-
Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
-
CoakleyRD, Grubb BR, Paradiso AM, Gatzy JT, Johnson LG, Kreda S.M., O'Neal WK, Boucher RC. 2003. Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc Natl Acad Sci 100: 16083-16088.
-
(2003)
Proc Natl Acad Sci
, vol.100
, pp. 16083-16088
-
-
Coakley, R.D.1
Grubb, B.R.2
Paradiso, A.M.3
Gatzy, J.T.4
Johnson, L.G.5
Kreda, S.M.6
O'Neal, W.K.7
Boucher, R.C.8
-
11
-
-
33846211109
-
The lactoperoxidase system links anion transport to host defense in cystic fibrosis
-
Conner G.E., Wijkstrom-Frei C, Randell SH, Fernandez VE, Salathe M. 2007. The lactoperoxidase system links anion transport to host defense in cystic fibrosis. FEBS Lett 581: 271-278.
-
(2007)
FEBS Lett
, vol.581
, pp. 271-278
-
-
Conner, G.E.1
Wijkstrom-Frei, C.2
Randell, S.H.3
Fernandez, V.E.4
Salathe, M.5
-
12
-
-
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, Young A, MacKenzie-Beevor A., Gosling M, Bhalay G, Bloomfield G, Dunstan A., Bridges RJ, et al. 2009. Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease. J Pharmacol Exp Ther 329: 764-774.
-
(2009)
J Pharmacol Exp Ther
, vol.329
, pp. 764-774
-
-
Coote, K.1
Atherton-Watson, H.C.2
Sugar, R.3
Young, A.4
McKenzie-Beevor, A.5
Gosling, M.6
Bhalay, G.7
Bloomfield, G.8
Dunstan, A.9
Bridges, R.J.10
-
13
-
-
0031444391
-
Inhalation of dry-powder mannitol increases mucociliary clearance
-
Daviskas E., Anderson SD, Brannan JD, Chan HK, Eberl S, Bautovich G. 1997. Inhalation of dry-powder mannitol increases mucociliary clearance. Eur Respir J 10: 2449-2454.
-
(1997)
Eur Respir J
, vol.10
, pp. 2449-2454
-
-
Daviskas, E.1
Anderson, S.D.2
Brannan, J.D.3
Chan, H.K.4
Eberl, S.5
Bautovich, G.6
-
14
-
-
0033039551
-
Inhalation of dry powder mannitol improves clearance of mucus in patients with bronchiectasis
-
Daviskas E., Anderson SD, Eberl S, Chan HK, Bautovich G. 1999. Inhalation of dry powder mannitol improves clearance of mucus in patients with bronchiectasis. Am J Respir Crit Care Med 159: 1843-1848.
-
(1999)
Am J Respir Crit Care Med
, vol.159
, pp. 1843-1848
-
-
Daviskas, E.1
Anderson, S.D.2
Eberl, S.3
Chan, H.K.4
Bautovich, G.5
-
15
-
-
0035110224
-
The 24-h effect of mannitol on the clearance of mucus in patients with bronchiectasis
-
Daviskas E., Anderson SD, Eberl S, Chan HK, Young IH. 2001. The 24-h effect of mannitol on the clearance of mucus in patients with bronchiectasis. Chest 119: 414-421.
-
(2001)
Chest
, vol.119
, pp. 414-421
-
-
Daviskas, E.1
Anderson, S.D.2
Eberl, S.3
Chan, H.K.4
Young, I.H.5
-
16
-
-
42949178050
-
Effect of increasing doses of mannitol on mucus clearance in patients with bronchiectasis
-
Daviskas E., Anderson SD, Eberl S, Young IH. 2008. Effect of increasing doses of mannitol on mucus clearance in patients with bronchiectasis. Eur Respir J 31: 765-772.
-
(2008)
Eur Respir J
, vol.31
, pp. 765-772
-
-
Daviskas, E.1
Anderson, S.D.2
Eberl, S.3
Young, I.H.4
-
17
-
-
77950787429
-
Inhaled mannitol improves the hydration and surface properties of sputum in patientswith cystic fibrosis
-
Daviskas E., Anderson SD, Jaques A, Charlton B. 2010. Inhaled mannitol improves the hydration and surface properties of sputum in patientswith cystic fibrosis. Chest 137: 861-868.
-
(2010)
Chest
, vol.137
, pp. 861-868
-
-
Daviskas, E.1
Anderson, S.D.2
Jaques, A.3
Charlton, B.4
-
18
-
-
20144363443
-
Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: Results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis
-
Deterding R., Retsch-Bogart G, Milgram L, Gibson R, Daines C., Zeitlin PL, Milla C, Marshall B, Lavange L, Engels J., et al. 2005. Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: Results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis. Pediatr Pulmonol 39: 339-348.
-
(2005)
Pediatr Pulmonol
, vol.39
, pp. 339-348
-
-
Deterding, R.1
Retsch-Bogart, G.2
Milgram, L.3
Gibson, R.4
Daines, C.5
Zeitlin, P.L.6
Milla, C.7
Marshall, B.8
Lavange, L.9
Engels, J.10
-
19
-
-
34547949974
-
Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis
-
Cystic Fibrosis Therapeutics DevelopmentNetwork Inspire 08-103 Working Group
-
Deterding R.R., Lavange LM, Engels JM, Mathews DW, Coquillette S.J., Brody AS, Millard SP, Ramsey BW, Cystic Fibrosis Therapeutics DevelopmentNetwork Inspire 08-103 Working Group. 2007. Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis. Am J Respir Crit Care Med 176: 362-369.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, pp. 362-369
-
-
Deterding, R.R.1
Lavange, L.M.2
Engels, J.M.3
Mathews, D.W.4
Coquillette, S.J.5
Brody, A.S.6
Millard, S.P.7
Ramsey, B.W.8
-
20
-
-
0037040995
-
Regulation of the epithelial sodium channel by serine proteases in human airways
-
Donaldson S.H., Hirsh A, Li DC, Holloway G, Chao J, Boucher R.C., Gabriel SE. 2002. Regulation of the epithelial sodium channel by serine proteases in human airways. J Biol Chem 277: 8338-8345.
-
(2002)
J Biol Chem
, vol.277
, 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
-
21
-
-
34548266959
-
Safety, pharmacokinetics and effects on mucus clearance following administration of 552-02 to normal healthy volunteers
-
Donaldson S., Smith R, Doran J, DiMassimo B, Zeman K, Bennett B., Hurd H, Hopkins S. 2005. Safety, pharmacokinetics and effects on mucus clearance following administration of 552-02 to normal healthy volunteers. Pediatr Pulmonol 28 (Suppl): 218.
-
(2005)
Pediatr Pulmonol
, vol.28
, Issue.SUPPL.
, pp. 218
-
-
Donaldson, S.1
Smith, R.2
Doran, J.3
DiMassimo, B.4
Zeman, K.5
Bennett, B.6
Hurd, H.7
Hopkins, S.8
-
22
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson S.H., Bennett WD, Zeman KL, Knowles MR, Tarran R., Boucher RC. 2006. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. New Engl J Med 354: 241-250.
-
(2006)
New Engl J Med
, vol.354
, pp. 241-250
-
-
Donaldson, S.H.1
Bennett, W.D.2
Zeman, K.L.3
Knowles, M.R.4
Tarran, R.5
Boucher, R.C.6
-
23
-
-
30944466084
-
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
-
National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group
-
Elkins M.R., Robinson M, Rose BR, Harbour C, Moriarty CP, Marks G.B., Belousova EG, Xuan W, Bye PT, National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. 2006. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354: 229-240.
-
(2006)
N Engl J Med
, vol.354
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
Harbour, C.4
Moriarty, C.P.5
Marks, G.B.6
Belousova, E.G.7
Xuan, W.8
Bye, P.T.9
-
24
-
-
9244227025
-
Transcellular thiocyanate transport by human airway epithelia
-
Fragoso M.A., FernandezV, Forteza R, Randell SH, Salathe M, Conner GE. 2004. Transcellular thiocyanate transport by human airway epithelia. J Physiol 561: 183-194.
-
(2004)
J Physiol
, vol.561
, pp. 183-194
-
-
Fragoso, M.A.1
Fernandez, V.2
Forteza, R.3
Randell, S.H.4
Salathe, M.5
Conner, G.E.6
-
25
-
-
73449138427
-
The TMEM16 protein family: A new class of chloride channels?
-
Galietta LJ. 2009. The TMEM16 protein family: A new class of chloride channels? Biophys J 97: 3047-3053.
-
(2009)
Biophys J
, vol.97
, pp. 3047-3053
-
-
Galietta, L.J.1
-
26
-
-
0037080350
-
IL-4 is a potent modulator of ion transport in the human bronchial epithelium in vitro
-
Galietta L.J., Pagesy P, Folli C, Caci E, Romio L, Costes B, Nicolis E., Cabrini G, Goossens M, Ravazzolo R, et al. 2002. IL-4 is a potent modulator of ion transport in the human bronchial epithelium in vitro. J Immunol 168: 839-845.
-
(2002)
J Immunol
, vol.168
, pp. 839-845
-
-
Galietta, L.J.1
Pagesy, P.2
Folli, C.3
Caci, E.4
Romio, L.5
Costes, B.6
Nicolis, E.7
Cabrini, G.8
Goossens, M.9
Ravazzolo, R.10
-
27
-
-
70349223856
-
Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion
-
Garcia M.A., Yang N, Quinton PM. 2009. Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion. J Clin Invest 119: 2613-2622.
-
(2009)
J Clin Invest
, vol.119
, pp. 2613-2622
-
-
Garcia, M.A.1
Yang, N.2
Quinton, P.M.3
-
29
-
-
67650488822
-
SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage
-
Garcia-Caballero A, Rasmussen JE, Gaillard E, Watson MJ, Olsen J.C., Donaldson SH, Stutts MJ, Tarran R. 2009. SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage. Proc Natl Acad Sci 106: 11412-11417.
-
(2009)
Proc Natl Acad Sci
, vol.106
, pp. 11412-11417
-
-
Garcia-Caballero, A.1
Rasmussen, J.E.2
Gaillard, E.3
Watson, M.J.4
Olsen, J.C.5
Donaldson, S.H.6
Stutts, M.J.7
Tarran, R.8
-
31
-
-
0034095739
-
The lactoperoxidase system functions in bacterial clearance of airways
-
Gerson C., Sabater J, Scuri M, Torbati A, Coffey R, Abraham JW, Lauredo I, Forteza R, Wanner A, Salathe M, et al. 2000. The lactoperoxidase system functions in bacterial clearance of airways. Am J Respir Cell Mol Biol 22: 665-671.
-
(2000)
Am J Respir Cell Mol Biol
, vol.22
, pp. 665-671
-
-
Gerson, C.1
Sabater, J.2
Scuri, M.3
Torbati, A.4
Coffey, R.5
Abraham, J.W.6
Lauredo, I.7
Forteza, R.8
Wanner, A.9
Salathe, M.10
-
32
-
-
0027486593
-
No added benefit from nebulized amiloride in patients with cystic fibrosis
-
Graham A., Hasani A, Alton EW, Martin GP, Marriott C, Hodson M.E., Clarke SW, Geddes DM. 1993. No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur Respir J 6: 1243-1248.
-
(1993)
Eur Respir J
, vol.6
, pp. 1243-1248
-
-
Graham, A.1
Hasani, A.2
Alton, E.W.3
Martin, G.P.4
Marriott, C.5
Hodson, M.E.6
Clarke, S.W.7
Geddes, D.M.8
-
33
-
-
34248597371
-
Inhalation of Moli1901 in patients with cystic fibrosis
-
Grasemann H., Stehling F, Brunar H, Widmann R, Laliberte TW, Molina L, Döring G, Ratjen F. 2007. Inhalation of Moli1901 in patients with cystic fibrosis. Chest 131: 1461-1466.
-
(2007)
Chest
, vol.131
, pp. 1461-1466
-
-
Grasemann, H.1
Stehling, F.2
Brunar, H.3
Widmann, R.4
Laliberte, T.W.5
Molina, L.6
Döring, G.7
Ratjen, F.8
-
35
-
-
41149164824
-
Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N0-4-[4-(2,3-dihydroxypropoxy) phenyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease
-
Hirsh A.J., Zhang J, Zamurs A, Fleegle J, Thelin WR, Caldwell RA, Sabater JR, Abraham WM, Donowitz M, Cha B, et al. 2008. Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N0-4-[4-(2,3-dihydroxypropoxy) phenyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease. J Pharmacol Exp Ther 325: 77-88.
-
(2008)
J Pharmacol Exp Ther
, vol.325
, pp. 77-88
-
-
Hirsh, A.J.1
Zhang, J.2
Zamurs, A.3
Fleegle, J.4
Thelin, W.R.5
Caldwell, R.A.6
Sabater, J.R.7
Abraham, W.M.8
Donowitz, M.9
Cha, B.10
-
36
-
-
0030956487
-
cAMPand genistein stimulate HCO3-conductance through CFTR in human airway epithelia
-
Illek B., Yankaskas JR, Machen TE. 1997. cAMPand genistein stimulate HCO3-conductance through CFTR in human airway epithelia. Am J Physiol 272: L752-L761.
-
(1997)
Am J Physiol
, vol.272
-
-
Illek, B.1
Yankaskas, J.R.2
McHen, T.E.3
-
37
-
-
44649166466
-
Inhaled mannitol improves lung function in cystic fibrosis
-
Jaques A., Daviskas E, Turton JA, McKay K, Cooper P, Stirling RG, Robertson CF, Bye PT, Lesouëf PN, Shadbolt B, et al. 2008. Inhaled mannitol improves lung function in cystic fibrosis. Chest 133: 1388-1396.
-
(2008)
Chest
, vol.133
, pp. 1388-1396
-
-
Jaques, A.1
Daviskas, E.2
Turton, J.A.3
McKay, K.4
Cooper, P.5
Stirling, R.G.6
Robertson, C.F.7
Bye, P.T.8
Lesouëf, P.N.9
Shadbolt, B.10
-
38
-
-
79952764514
-
Deletion of the ubiquitin ligase Nedd4 L in lung epithelia causes cystic fibrosis-like disease
-
Kimura T., Kawabe H, Jiang C, Zhang W, Xiang YY, Lu C, Salter M.W., Brose N, Lu WY, Rotin D. 2011. Deletion of the ubiquitin ligase Nedd4 L in lung epithelia causes cystic fibrosis-like disease. Proc Natl Acad Sci 108: 3216-3221.
-
(2011)
Proc Natl Acad Sci
, vol.108
, pp. 3216-3221
-
-
Kimura, T.1
Kawabe, H.2
Jiang, C.3
Zhang, W.4
Xiang, Y.Y.5
Lu, C.6
Salter, M.W.7
Brose, N.8
Lu, W.Y.9
Rotin, D.10
-
39
-
-
0019809960
-
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
-
Knowles M., Gatzy J, Boucher R. 1981. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 305: 1489-1495.
-
(1981)
N Engl J Med
, vol.305
, pp. 1489-1495
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
40
-
-
0025236867
-
A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
-
Knowles M.R., Church NL, Waltner WE, Yankaskas JR, Gilligan P., King M, Edwards LJ, Helms RW, Boucher RC. 1990. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 322: 1189-1194.
-
(1990)
N Engl J Med
, vol.322
, pp. 1189-1194
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
Yankaskas, J.R.4
Gilligan, P.5
King, M.6
Edwards, L.J.7
Helms, R.W.8
Boucher, R.C.9
-
41
-
-
0025874599
-
Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis [see comments]
-
Knowles M.R., Clarke LL, Boucher RC. 1991. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis [see comments]. N Engl J Med 325: 533-538.
-
(1991)
N Engl J Med
, vol.325
, pp. 533-538
-
-
Knowles, M.R.1
Clarke, L.L.2
Boucher, R.C.3
-
42
-
-
0000930452
-
Amiloride in CF: Safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease
-
In (ed. Cragoe EJ, et al.), VCH, Chichester, U. K
-
Knowles M.R., Church NL, Waltner WE, et al. 1992. Amiloride in CF: Safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease. In Amiloride and its analogs: Unique cation transport inhibitors (ed. Cragoe EJ, et al.), pp. 301-316. VCH, Chichester, U.K.
-
(1992)
Amiloride and its analogs: Unique cation transport inhibitors
, pp. 301-316
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
-
43
-
-
0022988338
-
Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses
-
Kohler D., App E, Schmitz-Schumann M, Wurtemberger G, Matthys H. 1986. Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses. Eur Respir J Dis Suppl 146: 319-326.
-
(1986)
Eur Respir J Dis Suppl
, vol.146
, pp. 319-326
-
-
Kohler, D.1
App, E.2
Schmitz-Schumann, M.3
Wurtemberger, G.4
Matthys, H.5
-
45
-
-
0025845558
-
Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium
-
Mason S.J., Paradiso AM, Boucher RC. 1991. Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium. Br J Pharmacol 103: 1649-1656.
-
(1991)
Br J Pharmacol
, vol.103
, pp. 1649-1656
-
-
Mason, S.J.1
Paradiso, A.M.2
Boucher, R.C.3
-
46
-
-
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, Randell SH, Gatzy JT, Davis C.W., Boucher RC. 1998. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95: 1005-1015.
-
(1998)
Cell
, vol.95
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
Randell, S.H.4
Gatzy, J.T.5
Davis, C.W.6
Boucher, R.C.7
-
47
-
-
73449118258
-
Comparison of inhaled mannitol, daily rhDNase and a combination of both in childrenwith cystic fibrosis: A randomised trial
-
Minasian C., Wallis C, Metcalfe C, Bush A. 2010. Comparison of inhaled mannitol, daily rhDNase and a combination of both in childrenwith cystic fibrosis: A randomised trial. Thorax 65: 51-56.
-
(2010)
Thorax
, vol.65
, pp. 51-56
-
-
Minasian, C.1
Wallis, C.2
Metcalfe, C.3
Bush, A.4
-
48
-
-
33846314338
-
A novel host defense system of airways is defective in cystic fibrosis
-
Moskwa P., Lorentzen D, Excoffon KJ, Zabner J, McCray PB Jr, Nauseef WM, Dupuy C, Bánfi B. 2007. A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med 175: 174-183.
-
(2007)
Am J Respir Crit Care Med
, vol.175
, pp. 174-183
-
-
Moskwa, P.1
Lorentzen, D.2
Excoffon, K.J.3
Zabner, J.4
McCray Jr., P.B.5
Nauseef, W.M.6
Dupuy, C.7
Bánfi, B.8
-
49
-
-
33748778207
-
Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: A mechanism for sodium hyperabsorption in cystic fibrosis
-
Myerburg M.M., Butterworth MB, McKenna EE, Peters KW, Frizzell R.A., Kleyman TR, Pilewski JM. 2006. Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: A mechanism for sodium hyperabsorption in cystic fibrosis. J Biol Chem 281: 27942-27949.
-
(2006)
J Biol Chem
, vol.281
, pp. 27942-27949
-
-
Myerburg, M.M.1
Butterworth, M.B.2
McKenna, E.E.3
Peters, K.W.4
Frizzell, R.A.5
Kleyman, T.R.6
Pilewski, J.M.7
-
50
-
-
45849124564
-
Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis
-
Myerburg M.M., McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, Pilewski JM. 2008. Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 294: L932-L941.
-
(2008)
Am J Physiol Lung Cell Mol Physiol
, vol.294
-
-
Myerburg, M.M.1
McKenna, E.E.2
Luke, C.J.3
Frizzell, R.A.4
Kleyman, T.R.5
Pilewski, J.M.6
-
51
-
-
78649743537
-
Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking
-
Myerburg M.M., Harvey PR, Heidrich EM, Pilewski JM, Butterworth MB. 2010. Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking. Am J Res Cell Mol Biol 43: 712-719.
-
(2010)
Am J Res Cell Mol Biol
, vol.43
, pp. 712-719
-
-
Myerburg, M.M.1
Harvey, P.R.2
Heidrich, E.M.3
Pilewski, J.M.4
Butterworth, M.B.5
-
52
-
-
78751478642
-
TMEM16A inhibitors reveal TMEM16A as a minor component of calcium-activated chloride channel conductance in airway and intestinal epithelial cells
-
Namkung W., Phuan PW, Verkman AS. 2011. TMEM16A inhibitors reveal TMEM16A as a minor component of calcium-activated chloride channel conductance in airway and intestinal epithelial cells. J Biol Chem 286: 2365-2374.
-
(2011)
J Biol Chem
, vol.286
, pp. 2365-2374
-
-
Namkung, W.1
Phuan, P.W.2
Verkman, A.S.3
-
53
-
-
0032811712
-
Effect of aerosolized uridine-50-triphosphate on airway clearance with cough in patients with primary ciliary dyskinesia
-
Noone P.G., Bennett WD, Regnis JA, Zeman KL, Carson JL, King M., Boucher RC, Knowles MR. 1999. Effect of aerosolized uridine-50-triphosphate on airway clearance with cough in patients with primary ciliary dyskinesia. Am J Respir Crit Care Med 160: 144-149.
-
(1999)
Am J Respir Crit Care Med
, vol.160
, pp. 144-149
-
-
Noone, P.G.1
Bennett, W.D.2
Regnis, J.A.3
Zeman, K.L.4
Carson, J.L.5
King, M.6
Boucher, R.C.7
Knowles, M.R.8
-
54
-
-
0030056228
-
Acute safety and effects on mucociliary clearance of aerosolized uridine 50-triphosphate +/2 amiloride in normal human adults
-
Olivier K.N., Bennett WD, Hohneker KW, Zeman KL, Edwards L.J., Boucher RC, Knowles MR. 1996. Acute safety and effects on mucociliary clearance of aerosolized uridine 50-triphosphate +/2 amiloride in normal human adults. Am J Respir Crit Care Med 154: 217-223.
-
(1996)
Am J Respir Crit Care Med
, vol.154
, pp. 217-223
-
-
Olivier, K.N.1
Bennett, W.D.2
Hohneker, K.W.3
Zeman, K.L.4
Edwards, L.J.5
Boucher, R.C.6
Knowles, M.R.7
-
56
-
-
2442427376
-
Metabolism of P2 receptor agonists in human airways: Implications for mucociliary clearance and cystic fibrosis
-
Picher M., Burch LH, Boucher RC. 2004. Metabolism of P2 receptor agonists in human airways: Implications for mucociliary clearance and cystic fibrosis. J Biol Chem 279: 20234-20241.
-
(2004)
J Biol Chem
, vol.279
, pp. 20234-20241
-
-
Picher, M.1
Burch, L.H.2
Boucher, R.C.3
-
57
-
-
0032834277
-
The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: A pilot study
-
Robinson M., Daviskas E, Eberl S, Baker J, Chan HK, Anderson S.D., Bye PT. 1999. The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: A pilot study. Eur Respir J 14: 678-685.
-
(1999)
Eur Respir J
, vol.14
, pp. 678-685
-
-
Robinson, M.1
Daviskas, E.2
Eberl, S.3
Baker, J.4
Chan, H.K.5
Anderson, S.D.6
Bye, P.T.7
-
59
-
-
77952891046
-
SPLUNC1 expression reduces surface levels of the epithelial sodium channel (ENaC) in Xenopus laevis oocytes
-
Rollins B.M., Garcia-Caballero A, Stutts MJ, Tarran R. 2010. SPLUNC1 expression reduces surface levels of the epithelial sodium channel (ENaC) in Xenopus laevis oocytes. Channels (Austin) 4: 255-259.
-
(2010)
Channels (Austin)
, vol.4
, pp. 255-259
-
-
Rollins, B.M.1
Garcia-Caballero, A.2
Stutts, M.J.3
Tarran, R.4
-
60
-
-
51549120559
-
Expression cloning of TMEM16A as a calcium-activated chloride channel subunit
-
Schroeder B.C., Cheng T, Jan YN, Jan LY. 2008. Expression cloning of TMEM16A as a calcium-activated chloride channel subunit. Cell 134: 1019-1029.
-
(2008)
Cell
, vol.134
, pp. 1019-1029
-
-
Schroeder, B.C.1
Cheng, T.2
Jan, Y.N.3
Jan, L.Y.4
-
61
-
-
0026645366
-
cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia
-
Smith J.J., Welsh MJ. 1992. cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia. J Clin Invest 89: 1148-1153.
-
(1992)
J Clin Invest
, vol.89
, pp. 1148-1153
-
-
Smith, J.J.1
Welsh, M.J.2
-
62
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels [see comments]
-
Stutts M.J., Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier B.C., Boucher RC. 1995. CFTR as a cAMP-dependent regulator of sodium channels [see comments]. Science 269: 847-850.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
Hamrick, M.4
Cohn, J.A.5
Rossier, B.C.6
Boucher, R.C.7
-
63
-
-
0036712439
-
2+activated Cl2 conductances
-
2+activated Cl2 conductances. J Gen Physiol 120: 407-418.
-
(2002)
J Gen Physiol
, vol.120
, pp. 407-418
-
-
Tarran, R.1
Loewen, M.E.2
Paradiso, A.M.3
Olsen, J.C.4
Gray, M.A.5
Argent, B.E.6
Boucher, R.C.7
Gabriel, S.E.8
-
64
-
-
27444444705
-
Normal and cystic fibrosis airway surface liquid homeostasis: The effects of phasic shear stress and viral infections
-
Tarran R., Button B, Picher M, Paradiso AM, Ribeiro CM, Lazarowski E.R., Zhang L, Collins PL, Pickles RJ, Fredberg JJ, et al. 2005. Normal and cystic fibrosis airway surface liquid homeostasis: The effects of phasic shear stress and viral infections. J Biol Chem 280: 35751-35759.
-
(2005)
J Biol Chem
, vol.280
, pp. 35751-35759
-
-
Tarran, R.1
Button, B.2
Picher, M.3
Paradiso, A.M.4
Ribeiro, C.M.5
Lazarowski, E.R.6
Zhang, L.7
Collins, P.L.8
Pickles, R.J.9
Fredberg, J.J.10
-
65
-
-
33646138489
-
Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia
-
Tarran R., Trout L, Donaldson SH, Boucher RC. 2006. Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia. J Gen Physiol 127: 591-604.
-
(2006)
J Gen Physiol
, vol.127
, pp. 591-604
-
-
Tarran, R.1
Trout, L.2
Donaldson, S.H.3
Boucher, R.C.4
-
66
-
-
34250887562
-
Rationale for hypertonic saline therapy for cystic fibrosis lung disease
-
Tarran R., Donaldson S, Boucher RC. 2007. Rationale for hypertonic saline therapy for cystic fibrosis lung disease. Semin Respir Crit Care Med 28: 295-302.
-
(2007)
Semin Respir Crit Care Med
, vol.28
, pp. 295-302
-
-
Tarran, R.1
Donaldson, S.2
Boucher, R.C.3
-
67
-
-
78651452280
-
Inhaled mannitol in patients with cystic fibrosis: A randomised open-label dose response trial
-
Teper A., Jaques A, Charlton B. 2011. Inhaled mannitol in patients with cystic fibrosis: A randomised open-label dose response trial. J Cystic Fibrosis 10: 1-8.
-
(2011)
J Cystic Fibrosis
, vol.10
, pp. 1-8
-
-
Teper, A.1
Jaques, A.2
Charlton, B.3
-
68
-
-
79953863174
-
Calmodulin-dependent activation of the epithelial calcium-dependent chloride channel TMEM16A
-
Tian Y., Kongsuphol P, Hug M, Ousingsawat J, Witzgall R, Schreiber R., Kunzelmann K. 2011. Calmodulin-dependent activation of the epithelial calcium-dependent chloride channel TMEM16A. FASEB J 25: 1058-1068.
-
(2011)
FASEB J
, vol.25
, pp. 1058-1068
-
-
Tian, Y.1
Kongsuphol, P.2
Hug, M.3
Ousingsawat, J.4
Witzgall, R.5
Schreiber, R.6
Kunzelmann, K.7
-
70
-
-
0036023427
-
Synergistic activation of ENaC by three membranebound channel-activating serine proteases (mCAP1, mCAP2, and mCAP3) and serum-and glucocorticoidregulated kinase (Sgk1) in Xenopus Oocytes
-
Vuagniaux G., Vallet V, Jaeger NF, Hummler E, Rossier BC. 2002. Synergistic activation of ENaC by three membranebound channel-activating serine proteases (mCAP1, mCAP2, and mCAP3) and serum-and glucocorticoidregulated kinase (Sgk1) in Xenopus Oocytes. J Gen Physiol 120: 191-201.
-
(2002)
J Gen Physiol
, vol.120
, pp. 191-201
-
-
Vuagniaux, G.1
Vallet, V.2
Jaeger, N.F.3
Hummler, E.4
Rossier, B.C.5
-
71
-
-
0026080664
-
Activation of chloride channels in normal and cystic fibrosis airway epithelial cells by multifunctional calcium/calmodulin-dependent protein kinase
-
Wagner J.A., Cozens AL, Schulman H, Gruenert DC, Stryer L, Gardner P. 1991. Activation of chloride channels in normal and cystic fibrosis airway epithelial cells by multifunctional calcium/calmodulin-dependent protein kinase. Nature 349: 793-796.
-
(1991)
Nature
, vol.349
, pp. 793-796
-
-
Wagner, J.A.1
Cozens, A.L.2
Schulman, H.3
Gruenert, D.C.4
Stryer, L.5
Gardner, P.6
-
72
-
-
13144268551
-
Inactivation of calcium-activated chloride channels in smooth muscle by calcium/ calmodulin-dependent protein kinase
-
Wang Y.X., Kotlikoff MI. 1997. Inactivation of calcium-activated chloride channels in smooth muscle by calcium/ calmodulin-dependent protein kinase. ProcNatl Acad Sci 94: 14918-14923.
-
(1997)
ProcNatl Acad Sci
, vol.94
, pp. 14918-14923
-
-
Wang, Y.X.1
Kotlikoff, M.I.2
-
73
-
-
0025995248
-
Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium
-
Willumsen N.J., Boucher RC. 1991. Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am J Physiol 261: C332-C341.
-
(1991)
Am J Physiol
, vol.261
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
74
-
-
0029938944
-
Inositol 3,4,5,6-tetrakisphosphate inhibits the calmodulin-dependent protein kinase II-activated chloride conductance in T84 colonic epithelial cells
-
Xie W., Kaetzel MA, Bruzik KS, Dedman JR, Shears SB, Nelson DJ. 1996. Inositol 3,4,5,6-tetrakisphosphate inhibits the calmodulin-dependent protein kinase II-activated chloride conductance in T84 colonic epithelial cells. J Biol Chem 271: 14092-14097.
-
(1996)
J Biol Chem
, vol.271
, pp. 14092-14097
-
-
Xie, W.1
Kaetzel, M.A.2
Bruzik, K.S.3
Dedman, J.R.4
Shears, S.B.5
Nelson, D.J.6
-
75
-
-
55249091085
-
TMEM16A confers receptor-activated calcium-dependent chloride conductance
-
Yang Y.D., Cho H, Koo JY, Tak MH, Cho Y, Shim WS, Park SP, Lee J., Lee B, Kim BM, et al. 2008. TMEM16A confers receptor-activated calcium-dependent chloride conductance. Nature 455: 1210-1215.
-
(2008)
Nature
, vol.455
, pp. 1210-1215
-
-
Yang, Y.D.1
Cho, H.2
Koo, J.Y.3
Tak, M.H.4
Cho, Y.5
Shim, W.S.6
Park, S.P.7
Lee, J.8
Lee, B.9
Kim, B.M.10
-
76
-
-
1642493889
-
A phase I trial of intranasalMoli1901 for cystic fibrosis
-
Zeitlin P.L., Boyle MP, GugginoWB, Molina L. 2004. A phase I trial of intranasalMoli1901 for cystic fibrosis. Chest 125: 143-149.
-
(2004)
Chest
, vol.125
, pp. 143-149
-
-
Zeitlin, P.L.1
Boyle, M.P.2
Guggino, W.B.3
Molina, L.4
|