-
1
-
-
0028586020
-
Effect of exercise on the nasal transmucosal potential difference in patients with cystic fibrosis and normal subjects
-
Alsuwaidan S., Li Wan Po A., Morrison G., Redmond A., Dodge J.A., McElnay J., Stewart E., and Stanford C.F. Effect of exercise on the nasal transmucosal potential difference in patients with cystic fibrosis and normal subjects. Thorax 49 (1994) 1249-1250
-
(1994)
Thorax
, vol.49
, pp. 1249-1250
-
-
Alsuwaidan, S.1
Li Wan Po, A.2
Morrison, G.3
Redmond, A.4
Dodge, J.A.5
McElnay, J.6
Stewart, E.7
Stanford, C.F.8
-
2
-
-
0026651508
-
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia
-
Anderson M.P., Sheppard D.N., Berger H.A., and Welsh M.J. Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am. J. Physiol. 263 (1992) L1-L14
-
(1992)
Am. J. Physiol.
, vol.263
-
-
Anderson, M.P.1
Sheppard, D.N.2
Berger, H.A.3
Welsh, M.J.4
-
4
-
-
1942421832
-
Liquid secretion properties of airway submucosal glands
-
Ballard S.T., and Inglis S.K. Liquid secretion properties of airway submucosal glands. J. Physiol. 556 (2004) 1-10
-
(2004)
J. Physiol.
, vol.556
, pp. 1-10
-
-
Ballard, S.T.1
Inglis, S.K.2
-
7
-
-
0028309683
-
Human airway ion transport. Part one
-
Boucher R.C. Human airway ion transport. Part one. Am. J. Respir. Crit. Care Med. 150 (1994) 271-281
-
(1994)
Am. J. Respir. Crit. Care Med.
, vol.150
, pp. 271-281
-
-
Boucher, R.C.1
-
8
-
-
0037279811
-
Regulation of airway surface liquid volume by human airway epithelia
-
Boucher R.C. Regulation of airway surface liquid volume by human airway epithelia. Pflugers Arch. 445 (2003) 495-498
-
(2003)
Pflugers Arch.
, vol.445
, pp. 495-498
-
-
Boucher, R.C.1
-
9
-
-
33845992179
-
Evidence for airway surface dehydration as the initiating event in CF airway disease
-
Boucher R.C. Evidence for airway surface dehydration as the initiating event in CF airway disease. J. Intern. Med. 261 (2007) 5-16
-
(2007)
J. Intern. Med.
, vol.261
, pp. 5-16
-
-
Boucher, R.C.1
-
10
-
-
0022973473
-
+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation
-
+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78 (1986) 1245-1252
-
(1986)
J. Clin. Invest.
, vol.78
, pp. 1245-1252
-
-
Boucher, R.C.1
Stutts, M.J.2
Knowles, M.R.3
Cantley, L.4
Gatzy, J.T.5
-
13
-
-
33747423674
-
Purinergic signalling-an overview
-
(discussion 48-57, 275-281)
-
Burnstock G. Purinergic signalling-an overview. Novartis Found Symp. 276 (2006) 26-48 (discussion 48-57, 275-281)
-
(2006)
Novartis Found Symp.
, vol.276
, pp. 26-48
-
-
Burnstock, G.1
-
15
-
-
1542267080
-
State of the art: why do the lungs of patients with cystic fibrosis become infected and why cannot they clear the infection?
-
Chmiel J.F., and Davis P.B. State of the art: why do the lungs of patients with cystic fibrosis become infected and why cannot they clear the infection?. Respir. Res. 4 (2003) 8
-
(2003)
Respir. Res.
, vol.4
, pp. 8
-
-
Chmiel, J.F.1
Davis, P.B.2
-
16
-
-
0031930649
-
Protease modulation of the activity of the epithelial sodium channel expressed in Xenopus oocytes
-
Chraibi A., Vallet V., Firsov D., Hess S.K., and Horisberger J.D. Protease modulation of the activity of the epithelial sodium channel expressed in Xenopus oocytes. J. Gen. Physiol. 111 (1998) 127-138
-
(1998)
J. Gen. Physiol.
, vol.111
, pp. 127-138
-
-
Chraibi, A.1
Vallet, V.2
Firsov, D.3
Hess, S.K.4
Horisberger, J.D.5
-
17
-
-
0028047286
-
Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice
-
Clarke L.L., Grubb B.R., Yankaskas J.R., Cotton C.U., McKenzie A., and Boucher R.C. Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice. Proc. Natl. Acad. Sci. U.S.A. 91 (1994) 479-483
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 479-483
-
-
Clarke, L.L.1
Grubb, B.R.2
Yankaskas, J.R.3
Cotton, C.U.4
McKenzie, A.5
Boucher, R.C.6
-
18
-
-
0346103648
-
Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
-
Coakley R.D., Grubb B.R., Paradiso A.M., Gatzy J.T., Johnson L.G., Kreda S.M., O'Neal W.K., and Boucher R.C. Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc. Natl. Acad. Sci. U.S.A. 100 (2003) 16083-16088
-
(2003)
Proc. Natl. Acad. Sci. U.S.A.
, 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
-
22
-
-
0034326719
-
Basal nucleotide levels, release, and metabolism in normal and cystic fibrosis airways
-
Donaldson S.H., Lazarowski E.R., Picher M., Knowles M.R., Stutts M.J., and Boucher R.C. Basal nucleotide levels, release, and metabolism in normal and cystic fibrosis airways. Mol. Med. 6 (2000) 969-982
-
(2000)
Mol. Med.
, vol.6
, pp. 969-982
-
-
Donaldson, S.H.1
Lazarowski, E.R.2
Picher, M.3
Knowles, M.R.4
Stutts, M.J.5
Boucher, R.C.6
-
23
-
-
0037040995
-
Regulation of the epithelial sodium channel by serine proteases in human airways
-
Donaldson S.H., Hirsh A., Li D.C., Holloway G., Chao J., Boucher R.C., and Gabriel S.E. Regulation of the epithelial sodium channel by serine proteases in human airways. J. Biol. Chem. 277 (2002) 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
-
24
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson S.H., Bennett W.D., Zeman K.L., Knowles M.R., Tarran R., and Boucher R.C. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N. Engl. J. Med. 354 (2006) 241-250
-
(2006)
N. Engl. J. Med.
, vol.354
, pp. 241-250
-
-
Donaldson, S.H.1
Bennett, W.D.2
Zeman, K.L.3
Knowles, M.R.4
Tarran, R.5
Boucher, R.C.6
-
25
-
-
33750372715
-
Inhaled hypertonic saline as a therapy for cystic fibrosis
-
Elkins M.R., and Bye P.T. Inhaled hypertonic saline as a therapy for cystic fibrosis. Curr. Opin. Pulm. Med. 12 (2006) 445-452
-
(2006)
Curr. Opin. Pulm. Med.
, vol.12
, pp. 445-452
-
-
Elkins, M.R.1
Bye, P.T.2
-
26
-
-
34447553334
-
-
Fischer, H.B., Illek, B., Finkbeiner, W.E., Widdicombe, J.H., 2007. Basolateral Cl channels in primary airway epithelial cultures. Am. J. Physiol. Lung Cell. Mol. Physiol.
-
-
-
-
27
-
-
0026058195
-
Microbiology of airway disease in patients with cystic fibrosis
-
Gilligan P.H. Microbiology of airway disease in patients with cystic fibrosis. Clin. Microbiol. Rev. 4 (1991) 35-51
-
(1991)
Clin. Microbiol. Rev.
, vol.4
, pp. 35-51
-
-
Gilligan, P.H.1
-
28
-
-
0027526366
-
cAMP-regulated whole cell chloride currents in pancreatic duct cells
-
Gray M.A., Plant S., and Argent B.E. cAMP-regulated whole cell chloride currents in pancreatic duct cells. Am. J. Physiol. 264 (1993) C591-C602
-
(1993)
Am. J. Physiol.
, vol.264
-
-
Gray, M.A.1
Plant, S.2
Argent, B.E.3
-
29
-
-
0028111842
-
Anomalies in ion transport in CF mouse tracheal epithelium
-
Grubb B.R., Paradiso A.M., and Boucher R.C. Anomalies in ion transport in CF mouse tracheal epithelium. Am. J. Physiol. 267 (1994) C293-C300
-
(1994)
Am. J. Physiol.
, vol.267
-
-
Grubb, B.R.1
Paradiso, A.M.2
Boucher, R.C.3
-
30
-
-
0037112390
-
ATP release from human airway epithelial cells studied using a capillary cell culture system
-
Guyot A., and Hanrahan J.W. ATP release from human airway epithelial cells studied using a capillary cell culture system. J. Physiol. 545 (2002) 199-206
-
(2002)
J. Physiol.
, vol.545
, pp. 199-206
-
-
Guyot, A.1
Hanrahan, J.W.2
-
31
-
-
0035421664
-
Exercise inhibits epithelial sodium channels in patients with cystic fibrosis
-
Hebestreit A., Kersting U., Basler B., Jeschke R., and Hebestreit H. Exercise inhibits epithelial sodium channels in patients with cystic fibrosis. Am. J. Respir. Crit. Care Med. 164 (2001) 443-446
-
(2001)
Am. J. Respir. Crit. Care Med.
, vol.164
, pp. 443-446
-
-
Hebestreit, A.1
Kersting, U.2
Basler, B.3
Jeschke, R.4
Hebestreit, H.5
-
32
-
-
0035923676
-
Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells
-
Huang P., Lazarowski E.R., Tarran R., Milgram S.L., Boucher R.C., and Stutts M.J. Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells. Proc. Natl. Acad. Sci. U.S.A. 98 (2001) 14120-14125
-
(2001)
Proc. Natl. Acad. Sci. U.S.A.
, vol.98
, pp. 14120-14125
-
-
Huang, P.1
Lazarowski, E.R.2
Tarran, R.3
Milgram, S.L.4
Boucher, R.C.5
Stutts, M.J.6
-
33
-
-
24944495525
-
Local regulation of cystic fibrosis transmembrane regulator and epithelial sodium channel in airway epithelium
-
Huang P., Gilmore E., Kultgen P., Barnes P., Milgram S., and Stutts M.J. Local regulation of cystic fibrosis transmembrane regulator and epithelial sodium channel in airway epithelium. Proc. Am. Thorac. Soc. 1 (2004) 33-37
-
(2004)
Proc. Am. Thorac. Soc.
, vol.1
, pp. 33-37
-
-
Huang, P.1
Gilmore, E.2
Kultgen, P.3
Barnes, P.4
Milgram, S.5
Stutts, M.J.6
-
34
-
-
26444432400
-
Cystic fibrosis and airway submucosal glands
-
Inglis S.K., and Wilson S.M. Cystic fibrosis and airway submucosal glands. Pediatr. Pulmonol. 40 (2005) 279-284
-
(2005)
Pediatr. Pulmonol.
, vol.40
, pp. 279-284
-
-
Inglis, S.K.1
Wilson, S.M.2
-
35
-
-
0030912066
-
Role of actin in regulation of epithelial sodium channels by CFTR
-
Ismailov I.I., Berdiev B.K., Shlyonsky V.G., Fuller C.M., Prat A.G., Jovov B., Cantiello H.F., Ausiello D.A., and Benos D.J. Role of actin in regulation of epithelial sodium channels by CFTR. Am. J. Physiol. 272 (1997) C1077-C1086
-
(1997)
Am. J. Physiol.
, vol.272
-
-
Ismailov, I.I.1
Berdiev, B.K.2
Shlyonsky, V.G.3
Fuller, C.M.4
Prat, A.G.5
Jovov, B.6
Cantiello, H.F.7
Ausiello, D.A.8
Benos, D.J.9
-
36
-
-
0020544649
-
Morphologic features of airway surface epithelial cells and glands
-
Jeffery P.K. Morphologic features of airway surface epithelial cells and glands. Am. Rev. Respir. Dis. 128 (1983) S14-S20
-
(1983)
Am. Rev. Respir. Dis.
, vol.128
-
-
Jeffery, P.K.1
-
37
-
-
0034623128
-
The cytosolic termini of the beta- and gamma-ENaC subunits are involved in the functional interactions between cystic fibrosis transmembrane conductance regulator and epithelial sodium channel
-
Ji H.L., Chalfant M.L., Jovov B., Lockhart J.P., Parker S.B., Fuller C.M., Stanton B.A., and Benos D.J. The cytosolic termini of the beta- and gamma-ENaC subunits are involved in the functional interactions between cystic fibrosis transmembrane conductance regulator and epithelial sodium channel. J. Biol. Chem. 275 (2000) 27947-27956
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 27947-27956
-
-
Ji, H.L.1
Chalfant, M.L.2
Jovov, B.3
Lockhart, J.P.4
Parker, S.B.5
Fuller, C.M.6
Stanton, B.A.7
Benos, D.J.8
-
38
-
-
0031046796
-
Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A
-
Jia Y., Mathews C.J., and Hanrahan J.W. Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J. Biol. Chem. 272 (1997) 4978-4984
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 4978-4984
-
-
Jia, Y.1
Mathews, C.J.2
Hanrahan, J.W.3
-
39
-
-
4644303141
-
Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells
-
Joo N.S., Lee D.J., Winges K.M., Rustagi A., and Wine J.J. Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells. J. Biol. Chem. 279 (2004) 38854-38860
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 38854-38860
-
-
Joo, N.S.1
Lee, D.J.2
Winges, K.M.3
Rustagi, A.4
Wine, J.J.5
-
40
-
-
0024423668
-
Identification of the cystic fibrosis gene: genetic analysis
-
Kerem B., Rommens J.M., Buchanan J.A., Markiewicz D., Cox T.K., Chakravarti A., Buchwald M., and Tsui L.C. Identification of the cystic fibrosis gene: genetic analysis. Science 245 (1989) 1073-1080
-
(1989)
Science
, vol.245
, pp. 1073-1080
-
-
Kerem, B.1
Rommens, J.M.2
Buchanan, J.A.3
Markiewicz, D.4
Cox, T.K.5
Chakravarti, A.6
Buchwald, M.7
Tsui, L.C.8
-
41
-
-
0036194724
-
Mucus clearance as a primary innate defense mechanism for mammalian airways
-
Knowles M.R., and Boucher R.C. Mucus clearance as a primary innate defense mechanism for mammalian airways. J. Clin. Invest. 109 (2002) 571-577
-
(2002)
J. Clin. Invest.
, vol.109
, pp. 571-577
-
-
Knowles, M.R.1
Boucher, R.C.2
-
42
-
-
0020596438
-
Relative ion permeability of normal and cystic fibrosis nasal epithelium
-
Knowles M., Gatzy J., and Boucher R. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J. Clin. Invest. 71 (1983) 1410-1417
-
(1983)
J. Clin. Invest.
, vol.71
, pp. 1410-1417
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
43
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
Knowles M.R., Stutts M.J., Spock A., Fischer N., Gatzy J.T., and Boucher R.C. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221 (1983) 1067-1070
-
(1983)
Science
, vol.221
, pp. 1067-1070
-
-
Knowles, M.R.1
Stutts, M.J.2
Spock, A.3
Fischer, N.4
Gatzy, J.T.5
Boucher, R.C.6
-
44
-
-
0031024767
-
+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator
-
+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 400 (1997) 341-344
-
(1997)
FEBS Lett.
, vol.400
, pp. 341-344
-
-
Kunzelmann, K.1
Kiser, G.L.2
Schreiber, R.3
Riordan, J.R.4
-
46
-
-
4344619319
-
Nucleotide release provides a mechanism for airway surface liquid homeostasis
-
Lazarowski E.R., Tarran R., Grubb B.R., van Heusden C.A., Okada S., and Boucher R.C. Nucleotide release provides a mechanism for airway surface liquid homeostasis. J. Biol. Chem. 279 (2004) 36855-36864
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 36855-36864
-
-
Lazarowski, E.R.1
Tarran, R.2
Grubb, B.R.3
van Heusden, C.A.4
Okada, S.5
Boucher, R.C.6
-
48
-
-
0036896008
-
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion
-
Ma T., Thiagarajah J.R., Yang H., Sonawane N.D., Folli C., Galietta L.J., and Verkman A.S. Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J. Clin. Invest. 110 (2002) 1651-1658
-
(2002)
J. Clin. Invest.
, vol.110
, pp. 1651-1658
-
-
Ma, T.1
Thiagarajah, J.R.2
Yang, H.3
Sonawane, N.D.4
Folli, C.5
Galietta, L.J.6
Verkman, A.S.7
-
49
-
-
0034537007
-
Inhibition of amiloride-sensitive epithelial Na(+) absorption by extracellular nucleotides in human normal and cystic fibrosis airways
-
Mall M., Wissner A., Gonska T., Calenborn D., Kuehr J., Brandis M., and Kunzelmann K. Inhibition of amiloride-sensitive epithelial Na(+) absorption by extracellular nucleotides in human normal and cystic fibrosis airways. Am. J. Respir. Cell. Mol. Biol. 23 (2000) 755-761
-
(2000)
Am. J. Respir. Cell. Mol. Biol.
, vol.23
, pp. 755-761
-
-
Mall, M.1
Wissner, A.2
Gonska, T.3
Calenborn, D.4
Kuehr, J.5
Brandis, M.6
Kunzelmann, K.7
-
51
-
-
0025845558
-
Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium
-
Mason S.J., Paradiso A.M., and Boucher R.C. Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium. Br. J. Pharmacol. 103 (1991) 1649-1656
-
(1991)
Br. J. Pharmacol.
, vol.103
, pp. 1649-1656
-
-
Mason, S.J.1
Paradiso, A.M.2
Boucher, R.C.3
-
52
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
Matsui H., Grubb B.R., Tarran R., Randell S.H., Gatzy J.T., Davis C.W., and Boucher R.C. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95 (1998) 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
-
53
-
-
0034125685
-
Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia
-
Matsui H., Davis C.W., Tarran R., and Boucher R.C. Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia. J. Clin. Invest. 105 (2000) 1419-1427
-
(2000)
J. Clin. Invest.
, vol.105
, pp. 1419-1427
-
-
Matsui, H.1
Davis, C.W.2
Tarran, R.3
Boucher, R.C.4
-
54
-
-
14544294477
-
ClC-5 chloride channel alters expression of the epithelial sodium channel (ENaC)
-
Mo L., and Wills N.K. ClC-5 chloride channel alters expression of the epithelial sodium channel (ENaC). J. Membr. Biol. 202 (2004) 21-37
-
(2004)
J. Membr. Biol.
, vol.202
, pp. 21-37
-
-
Mo, L.1
Wills, N.K.2
-
55
-
-
23644462552
-
Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment
-
Moskowitz S.M., Gibson R.L., and Effmann E.L. Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment. Pediatr. Radiol. 35 (2005) 739-757
-
(2005)
Pediatr. Radiol.
, vol.35
, pp. 739-757
-
-
Moskowitz, S.M.1
Gibson, R.L.2
Effmann, E.L.3
-
56
-
-
33748778207
-
-
Myerburg, M.M., Butterworth, M.B., McKenna, E.E., Peters, K.W., Frizzell, R.A., Kleyman, T.R., Pilewski, J.M., 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.
-
-
-
-
57
-
-
33947314619
-
Cell surface dynamics of CFTR: the ins and outs
-
Okiyoneda T., and Lukacs G.L. Cell surface dynamics of CFTR: the ins and outs. Biochim. Biophys. Acta 1773 (2007) 476-479
-
(2007)
Biochim. Biophys. Acta
, vol.1773
, pp. 476-479
-
-
Okiyoneda, T.1
Lukacs, G.L.2
-
58
-
-
0038514195
-
Human airway ecto-adenylate kinase. A mechanism to propagate ATP signaling on airway surfaces
-
Picher M., and Boucher R.C. Human airway ecto-adenylate kinase. A mechanism to propagate ATP signaling on airway surfaces. J. Biol. Chem. 278 (2003) 11256-11264
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 11256-11264
-
-
Picher, M.1
Boucher, R.C.2
-
59
-
-
2442427376
-
Metabolism of P2 receptor agonists in human airways: implications for mucociliary clearance and cystic fibrosis
-
Picher M., Burch L.H., and Boucher R.C. Metabolism of P2 receptor agonists in human airways: implications for mucociliary clearance and cystic fibrosis. J. Biol. Chem. 279 (2004) 20234-20241
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 20234-20241
-
-
Picher, M.1
Burch, L.H.2
Boucher, R.C.3
-
61
-
-
0024424270
-
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
-
Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., Chou J.L., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245 (1989) 1066-1073
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
62
-
-
10344236448
-
The epithelial sodium channel: activation by membrane-bound serine proteases
-
Rossier B.C. The epithelial sodium channel: activation by membrane-bound serine proteases. Proc. Am. Thorac. Soc. 1 (2004) 4-9
-
(2004)
Proc. Am. Thorac. Soc.
, vol.1
, pp. 4-9
-
-
Rossier, B.C.1
-
63
-
-
0033913469
-
A randomized controlled trial of a 3-year home exercise program in cystic fibrosis
-
Schneiderman-Walker J., Pollock S.L., Corey M., Wilkes D.D., Canny G.J., Pedder L., and Reisman J.J. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J. Pediatr. 136 (2000) 304-310
-
(2000)
J. Pediatr.
, vol.136
, pp. 304-310
-
-
Schneiderman-Walker, J.1
Pollock, S.L.2
Corey, M.3
Wilkes, D.D.4
Canny, G.J.5
Pedder, L.6
Reisman, J.J.7
-
64
-
-
33749268442
-
Heterogeneity of the composition and thickness of tracheal mucus in rats
-
Sims D.E., and Horne M.M. Heterogeneity of the composition and thickness of tracheal mucus in rats. Am. J. Physiol. 273 (1997) L1036-L1041
-
(1997)
Am. J. Physiol.
, vol.273
-
-
Sims, D.E.1
Horne, M.M.2
-
65
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts M.J., Canessa C.M., Olsen J.C., Hamrick M., Cohn J.A., Rossier B.C., and Boucher R.C. CFTR as a cAMP-dependent regulator of sodium channels. Science 269 (1995) 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
-
66
-
-
34247330452
-
Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC
-
Suaud L., Yan W., Carattino M.D., Robay A., Kleyman T.R., and Rubenstein R.C. Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC. Am. J. Physiol. Cell. Physiol. 292 (2007) C1553-C1561
-
(2007)
Am. J. Physiol. Cell. Physiol.
, vol.292
-
-
Suaud, L.1
Yan, W.2
Carattino, M.D.3
Robay, A.4
Kleyman, T.R.5
Rubenstein, R.C.6
-
67
-
-
0035661677
-
Regulation of K(+) current in human airway epithelial cells by exogenous and autocrine adenosine
-
Szkotak A.J., Ng A.M., Sawicka J., Baldwin S.A., Man S.F., Cass C.E., Young J.D., and Duszyk M. Regulation of K(+) current in human airway epithelial cells by exogenous and autocrine adenosine. Am. J. Physiol. Cell. Physiol. 281 (2001) C1991-C2002
-
(2001)
Am. J. Physiol. Cell. Physiol.
, vol.281
-
-
Szkotak, A.J.1
Ng, A.M.2
Sawicka, J.3
Baldwin, S.A.4
Man, S.F.5
Cass, C.E.6
Young, J.D.7
Duszyk, M.8
-
68
-
-
0036012529
-
Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro
-
Tarran R., and Boucher R.C. Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro. Methods Mol. Med. 70 (2002) 479-492
-
(2002)
Methods Mol. Med.
, vol.70
, pp. 479-492
-
-
Tarran, R.1
Boucher, R.C.2
-
69
-
-
0034885192
-
The relative roles of passive surface forces and active ion transport in the modulation of airway surface liquid volume and composition
-
Tarran R., Grubb B.R., Gatzy J.T., Davis C.W., and Boucher R.C. The relative roles of passive surface forces and active ion transport in the modulation of airway surface liquid volume and composition. J. Gen. Physiol. 118 (2001) 223-236
-
(2001)
J. Gen. Physiol.
, vol.118
, pp. 223-236
-
-
Tarran, R.1
Grubb, B.R.2
Gatzy, J.T.3
Davis, C.W.4
Boucher, R.C.5
-
70
-
-
0034885663
-
The CF salt controversy: in vivo observations and therapeutic approaches
-
Tarran R., Grubb B.R., Parsons D., Picher M., Hirsh A.J., Davis C.W., and Boucher R.C. The CF salt controversy: in vivo observations and therapeutic approaches. Mol. Cell. 8 (2001) 149-158
-
(2001)
Mol. Cell.
, vol.8
, pp. 149-158
-
-
Tarran, R.1
Grubb, B.R.2
Parsons, D.3
Picher, M.4
Hirsh, A.J.5
Davis, C.W.6
Boucher, R.C.7
-
71
-
-
0036712439
-
- conductances
-
- conductances. J. Gen. Physiol. 120 (2002) 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
-
72
-
-
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 A.M., Ribeiro C.M., Lazarowski E.R., Zhang L., Collins P.L., Pickles R.J., Fredberg J.J., and Boucher R.C. Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections. J. Biol. Chem. 280 (2005) 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
Boucher, R.C.11
-
73
-
-
33645964965
-
Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress
-
Tarran R., Button B., and Boucher R.C. Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress. Annu. Rev. Physiol. 68 (2006) 543-561
-
(2006)
Annu. Rev. Physiol.
, vol.68
, pp. 543-561
-
-
Tarran, R.1
Button, B.2
Boucher, R.C.3
-
74
-
-
33646138489
-
Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia
-
Tarran R., Trout L., Donaldson S.H., and Boucher R.C. Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia. J. Gen. Physiol. 127 (2006) 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
-
75
-
-
5444262302
-
Prostasin, a membrane-anchored serine peptidase, regulates sodium currents in JME/CF15 cells, a cystic fibrosis airway epithelial cell line
-
Tong Z., Illek B., Bhagwandin V.J., Verghese G.M., and Caughey G.H. Prostasin, a membrane-anchored serine peptidase, regulates sodium currents in JME/CF15 cells, a cystic fibrosis airway epithelial cell line. Am. J. Physiol. Lung Cell. Mol. Physiol. 287 (2004) L928-L935
-
(2004)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.287
-
-
Tong, Z.1
Illek, B.2
Bhagwandin, V.J.3
Verghese, G.M.4
Caughey, G.H.5
-
76
-
-
0038718857
-
Disruptive effects of anion secretion inhibitors on airway mucus morphology in isolated perfused pig lung
-
Trout L., Townsley M.I., Bowden A.L., and Ballard S.T. Disruptive effects of anion secretion inhibitors on airway mucus morphology in isolated perfused pig lung. J. Physiol. 549 (2003) 845-853
-
(2003)
J. Physiol.
, vol.549
, pp. 845-853
-
-
Trout, L.1
Townsley, M.I.2
Bowden, A.L.3
Ballard, S.T.4
-
77
-
-
0030879756
-
An epithelial serine protease activates the amiloride-sensitive sodium channel
-
Vallet V., Chraibi A., Gaeggeler H.P., Horisberger J.D., and Rossier B.C. An epithelial serine protease activates the amiloride-sensitive sodium channel. Nature 389 (1997) 607-610
-
(1997)
Nature
, vol.389
, pp. 607-610
-
-
Vallet, V.1
Chraibi, A.2
Gaeggeler, H.P.3
Horisberger, J.D.4
Rossier, B.C.5
-
78
-
-
33845308239
-
Prostasin regulates epithelial monolayer function: cell-specific Gpld1-mediated secretion and functional role for GPI anchor
-
Verghese G.M., Gutknecht M.F., and Caughey G.H. Prostasin regulates epithelial monolayer function: cell-specific Gpld1-mediated secretion and functional role for GPI anchor. Am. J. Physiol. Cell. Physiol. 291 (2006) C1258-C1270
-
(2006)
Am. J. Physiol. Cell. Physiol.
, vol.291
-
-
Verghese, G.M.1
Gutknecht, M.F.2
Caughey, G.H.3
-
79
-
-
17544386000
-
Activation of the amiloride-sensitive epithelial sodium channel by the serine protease mCAP1 expressed in a mouse cortical collecting duct cell line
-
Vuagniaux G., Vallet V., Jaeger N.F., Pfister C., Bens M., Farman N., Courtois-Coutry N., Vandewalle A., Rossier B.C., and Hummler E. Activation of the amiloride-sensitive epithelial sodium channel by the serine protease mCAP1 expressed in a mouse cortical collecting duct cell line. J. Am. Soc. Nephrol. 11 (2000) 828-834
-
(2000)
J. Am. Soc. Nephrol.
, vol.11
, pp. 828-834
-
-
Vuagniaux, G.1
Vallet, V.2
Jaeger, N.F.3
Pfister, C.4
Bens, M.5
Farman, N.6
Courtois-Coutry, N.7
Vandewalle, A.8
Rossier, B.C.9
Hummler, E.10
-
80
-
-
0042024971
-
Respiratory virus infections in cystic fibrosis
-
Wat D., and Doull I. Respiratory virus infections in cystic fibrosis. Paediatr. Respir. Rev. 4 (2003) 172-177
-
(2003)
Paediatr. Respir. Rev.
, vol.4
, pp. 172-177
-
-
Wat, D.1
Doull, I.2
-
81
-
-
0032577474
-
Cystic fibrosis transmembrane regulator-independent release of ATP. Its implications for the regulation of P2Y2 receptors in airway epithelia
-
Watt W.C., Lazarowski E.R., and Boucher R.C. Cystic fibrosis transmembrane regulator-independent release of ATP. Its implications for the regulation of P2Y2 receptors in airway epithelia. J. Biol. Chem. 273 (1998) 14053-14058
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 14053-14058
-
-
Watt, W.C.1
Lazarowski, E.R.2
Boucher, R.C.3
-
82
-
-
0024547744
-
Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia
-
Willumsen N.J., and Boucher R.C. Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia. Am. J. Physiol. 256 (1989) C1054-C1063
-
(1989)
Am. J. Physiol.
, vol.256
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
83
-
-
0025997759
-
Sodium transport and intracellular sodium activity in cultured human nasal epithelium
-
Willumsen N.J., and Boucher R.C. Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am. J. Physiol. 261 (1991) C319-C331
-
(1991)
Am. J. Physiol.
, vol.261
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
84
-
-
0025995248
-
Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium
-
Willumsen N.J., and Boucher R.C. Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am. J. Physiol. 261 (1991) C332-C341
-
(1991)
Am. J. Physiol.
, vol.261
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
87
-
-
4544353992
-
Submucosal glands and airway defense
-
Wine J.J., and Joo N.S. Submucosal glands and airway defense. Proc. Am. Thorac. Soc. 1 (2004) 47-53
-
(2004)
Proc. Am. Thorac. Soc.
, vol.1
, pp. 47-53
-
-
Wine, J.J.1
Joo, N.S.2
-
88
-
-
0028937158
-
Protein kinase C regulates the magnitude and stability of CFTR currents in pancreatic duct cells
-
Winpenny J.P., McAlroy H.L., Gray M.A., and Argent B.E. Protein kinase C regulates the magnitude and stability of CFTR currents in pancreatic duct cells. Am. J. Physiol. 268 (1995) C823-C828
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Winpenny, J.P.1
McAlroy, H.L.2
Gray, M.A.3
Argent, B.E.4
-
89
-
-
0031953795
-
Regulation of the depth of surface liquid in bovine trachea
-
Wu D.X., Lee C.Y., Uyekubo S.N., Choi H.K., Bastacky S.J., and Widdicombe J.H. Regulation of the depth of surface liquid in bovine trachea. Am. J. Physiol. 274 (1998) L388-L395
-
(1998)
Am. J. Physiol.
, vol.274
-
-
Wu, D.X.1
Lee, C.Y.2
Uyekubo, S.N.3
Choi, H.K.4
Bastacky, S.J.5
Widdicombe, J.H.6
-
90
-
-
0036720462
-
Pharmacology of INS37217 [P(1)-(uridine 5′)-P(4)-(2′-deoxycytidine-5′)tetraphosphate, tetrasodium salt], a next-generation P2Y(2) receptor agonist for the treatment of cystic fibrosis
-
Yerxa B.R., Sabater J.R., Davis C.W., Stutts M.J., Lang-Furr M., Picher M., Jones A.C., Cowlen M., Dougherty R., Boyer J., Abraham W.M., and Boucher R.C. Pharmacology of INS37217 [P(1)-(uridine 5′)-P(4)-(2′-deoxycytidine-5′)tetraphosphate, tetrasodium salt], a next-generation P2Y(2) receptor agonist for the treatment of cystic fibrosis. J. Pharmacol. Exp. Ther. 302 (2002) 871-880
-
(2002)
J. Pharmacol. Exp. Ther.
, vol.302
, pp. 871-880
-
-
Yerxa, B.R.1
Sabater, J.R.2
Davis, C.W.3
Stutts, M.J.4
Lang-Furr, M.5
Picher, M.6
Jones, A.C.7
Cowlen, M.8
Dougherty, R.9
Boyer, J.10
Abraham, W.M.11
Boucher, R.C.12
|