-
1
-
-
0037027912
-
An overview of the pathogenesis of cystic fibrosis lung disease
-
Boucher RC. An overview of the pathogenesis of cystic fibrosis lung disease. Adv Drug Delivery Res 54: 1359-1371, 2002.
-
(2002)
Adv Drug Delivery Res
, vol.54
, pp. 1359-1371
-
-
Boucher, R.C.1
-
2
-
-
0037279811
-
Regulation of airway surface liquid volume by human airway epithelia
-
Boucher RC. Regulation of airway surface liquid volume by human airway epithelia. Pflügers Arch 445: 495-498, 2003.
-
(2003)
Pflügers Arch
, vol.445
, pp. 495-498
-
-
Boucher, R.C.1
-
3
-
-
0035199694
-
Examining basal chloride transport using the nasal potential difference response in a murine model
-
Brady KG, Kelley TJ, and Drumm ML. Examining basal chloride transport using the nasal potential difference response in a murine model. Am J Physiol Lung Cell Mol Physiol 281: L1173-L1179, 2001.
-
(2001)
Am J Physiol Lung Cell Mol Physiol
, vol.281
-
-
Brady, K.G.1
Kelley, T.J.2
Drumm, M.L.3
-
4
-
-
0034292343
-
Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo
-
Chroneos ZC, Wert SE, Livingston JL, Hassett DJ, and Whitsett JA. Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo. J Immunol 165: 3941-3950, 2000.
-
(2000)
J Immunol
, vol.165
, pp. 3941-3950
-
-
Chroneos, Z.C.1
Wert, S.E.2
Livingston, J.L.3
Hassett, D.J.4
Whitsett, J.A.5
-
5
-
-
0026640380
-
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis
-
Clarke LL, Grubb BR, Gabriel SE, Smithies O, Koller BH, and Boucher RC. Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257: 1125-1128, 1992.
-
(1992)
Science
, vol.257
, pp. 1125-1128
-
-
Clarke, L.L.1
Grubb, B.R.2
Gabriel, S.E.3
Smithies, O.4
Koller, B.H.5
Boucher, R.C.6
-
6
-
-
0032081281
-
Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells
-
DiMango E, Ratner AJ, Bryan R, Tabibi S, and Prince A. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest 101: 2598-2605, 1998.
-
(1998)
J Clin Invest
, vol.101
, pp. 2598-2605
-
-
DiMango, E.1
Ratner, A.J.2
Bryan, R.3
Tabibi, S.4
Prince, A.5
-
7
-
-
0028881698
-
Diverse Pseudomonas aeruginosa gene products stimulate respiratory epithelial cells to produce interleukin-8
-
DiMango E, Zar HJ, Bryan R, and Prince A. Diverse Pseudomonas aeruginosa gene products stimulate respiratory epithelial cells to produce interleukin-8. J Clin Invest 96: 2204-2210, 1995.
-
(1995)
J Clin Invest
, vol.96
, pp. 2204-2210
-
-
DiMango, E.1
Zar, H.J.2
Bryan, R.3
Prince, A.4
-
8
-
-
0037218939
-
Plasma membrane CFTR regulates RANTES expression via its C-terminal PDZ-interacting motif
-
Estell K, Braunstein G, Tucker T, Varga K, Collawn JF, and Schwiebert LM. Plasma membrane CFTR regulates RANTES expression via its C-terminal PDZ-interacting motif. Mol Cell Biol 23: 594-606, 2003.
-
(2003)
Mol Cell Biol
, vol.23
, pp. 594-606
-
-
Estell, K.1
Braunstein, G.2
Tucker, T.3
Varga, K.4
Collawn, J.F.5
Schwiebert, L.M.6
-
9
-
-
0031979299
-
Impaired ability of Cftr knockout mice to control lung infection with Pseudomonas aeruginosa
-
Gosselin D, Stevenson MM, Cowley EA, Griesenbach U, Eidelman DH, Boule M, Tam MF, Kent G, Skamene E, Tsui LC, and Radzioch D. Impaired ability of Cftr knockout mice to control lung infection with Pseudomonas aeruginosa. Am J Respir Crit Care Med 157: 1253-3262, 1998.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 1253-3262
-
-
Gosselin, D.1
Stevenson, M.M.2
Cowley, E.A.3
Griesenbach, U.4
Eidelman, D.H.5
Boule, M.6
Tam, M.F.7
Kent, G.8
Skamene, E.9
Tsui, L.C.10
Radzioch, D.11
-
10
-
-
0031452350
-
Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa
-
Heeckeren A, Walenga R, Konstan MW, Bonfield T, Davis PB, and Ferkol T. Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest 100: 2810-2815, 1997.
-
(1997)
J Clin Invest
, vol.100
, pp. 2810-2815
-
-
Heeckeren, A.1
Walenga, R.2
Konstan, M.W.3
Bonfield, T.4
Davis, P.B.5
Ferkol, T.6
-
11
-
-
0032576605
-
Aggresomes: A cellular response to misfolded proteins
-
Johnston JA, Ward CL, and Kopito RR. Aggresomes: a cellular response to misfolded proteins. J Cell Biol 143: 1883-1898, 1998.
-
(1998)
J Cell Biol
, vol.143
, pp. 1883-1898
-
-
Johnston, J.A.1
Ward, C.L.2
Kopito, R.R.3
-
12
-
-
0030900255
-
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF 508 in murine nasal epithelium
-
Kelley TJ, Thomas K, Milgram LJ, and Drumm ML. In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium. Proc Natl Acad Sci USA 94: 2604-2608, 1997.
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 2604-2608
-
-
Kelley, T.J.1
Thomas, K.2
Milgram, L.J.3
Drumm, M.L.4
-
13
-
-
0001278692
-
Infection and inflammation of the lung in cystic fibrosis
-
edited by Davis PB. New York: Dekker
-
Konstan MW and Berger M. Infection and inflammation of the lung in cystic fibrosis. In: Cystic Fibrosis, edited by Davis PB. New York: Dekker, 1993, p. 219-276.
-
(1993)
Cystic Fibrosis
, pp. 219-276
-
-
Konstan, M.W.1
Berger, M.2
-
14
-
-
0027682586
-
Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis
-
Konstan MW, Walenga RW, Hilliard KA, and Hilliard JB. Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis. Am Rev Respir Dis 148: 896-901, 1993.
-
(1993)
Am Rev Respir Dis
, vol.148
, pp. 896-901
-
-
Konstan, M.W.1
Walenga, R.W.2
Hilliard, K.A.3
Hilliard, J.B.4
-
17
-
-
0034822263
-
G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease
-
McMorran BJ, Palmer JS, Lunn DP, Oceandy D, Costelloe EO, Thomas GR, Hume DA, and Wainwright BJ. G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease. Am J Physiol Lung Cell Mol Physiol 281: L740-L747, 2001.
-
(2001)
Am J Physiol Lung Cell Mol Physiol
, vol.281
-
-
McMorran, B.J.1
Palmer, J.S.2
Lunn, D.P.3
Oceandy, D.4
Costelloe, E.O.5
Thomas, G.R.6
Hume, D.A.7
Wainwright, B.J.8
-
18
-
-
0037072890
-
Aggregation of misfolded proteins can be a selective process dependent upon peptide composition
-
Milewski MI, Mickle JE, Forrest JK, Stanton BA, and Cutting GR. Aggregation of misfolded proteins can be a selective process dependent upon peptide composition. J Biol Chem 277: 34462-34470, 2002.
-
(2002)
J Biol Chem
, vol.277
, pp. 34462-34470
-
-
Milewski, M.I.1
Mickle, J.E.2
Forrest, J.K.3
Stanton, B.A.4
Cutting, G.R.5
-
19
-
-
0032770489
-
Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients
-
Muhlebach MS, Stewart PW, Leigh MW, and Noah TL. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med 160: 186-191, 1999.
-
(1999)
Am J Respir Crit Care Med
, vol.160
, pp. 186-191
-
-
Muhlebach, M.S.1
Stewart, P.W.2
Leigh, M.W.3
Noah, T.L.4
-
20
-
-
0036565902
-
Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities
-
Oceandy D, McMorran BJ, Smith SN, Schreiber R, Kunzelmann K, Alton EW, Hume DA, and Wainwright BJ. Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities. Hum Mol Genet 11: 1059-1067, 2002.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 1059-1067
-
-
Oceandy, D.1
McMorran, B.J.2
Smith, S.N.3
Schreiber, R.4
Kunzelmann, K.5
Alton, E.W.6
Hume, D.A.7
Wainwright, B.J.8
-
21
-
-
0022204452
-
Estimation of volume of epithelial lining fluid recovered by lavage using urea as marker of dilution
-
Rennard SI, Basset G, Lecossier D, O'Donnell KM, Pinkston P, Martin PG, and Crystal RG. Estimation of volume of epithelial lining fluid recovered by lavage using urea as marker of dilution. J Appl Physiol 60: 532-538, 1986.
-
(1986)
J Appl Physiol
, vol.60
, pp. 532-538
-
-
Rennard, S.I.1
Basset, G.2
Lecossier, D.3
O'Donnell, K.M.4
Pinkston, P.5
Martin, P.G.6
Crystal, R.G.7
-
22
-
-
0035877054
-
Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract
-
Schroeder TH, Reiniger N, Meluleni G, Grout M, Coleman FT, and Pier GB. Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract. J Immunol 166: 7410-7418, 2001.
-
(2001)
J Immunol
, vol.166
, pp. 7410-7418
-
-
Schroeder, T.H.1
Reiniger, N.2
Meluleni, G.3
Grout, M.4
Coleman, F.T.5
Pier, G.B.6
-
23
-
-
0032955681
-
Chemokine expression in CF epithelia: Implications for the role of CFTR in RANTES expression
-
Schwiebert LM, Estell K, and Propst SM. Chemokine expression in CF epithelia: implications for the role of CFTR in RANTES expression. Am J Physiol Cell Physiol 276: C700-C710, 1999.
-
(1999)
Am J Physiol Cell Physiol
, vol.276
-
-
Schwiebert, L.M.1
Estell, K.2
Propst, S.M.3
-
24
-
-
0029870085
-
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
-
Smith JJ, Travis SM, Greenberg EP, and Welsh MJ. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 85: 229-236, 1996.
-
(1996)
Cell
, vol.85
, pp. 229-236
-
-
Smith, J.J.1
Travis, S.M.2
Greenberg, E.P.3
Welsh, M.J.4
-
25
-
-
0028856292
-
Defective protein folding as a basis of human disease
-
Thomas PJ, Qu BH, and Pedersen PL. Defective protein folding as a basis of human disease. Trends Blochem Sci 20: 456-459, 1995.
-
(1995)
Trends Blochem Sci
, vol.20
, pp. 456-459
-
-
Thomas, P.J.1
Qu, B.H.2
Pedersen, P.L.3
-
26
-
-
0031883334
-
Effects of bronchopulmonary inflammation induced by pseudomonas aeruginosa on adenovirus-mediated gene transfer to airway epithelial cells in mice
-
Van Heeckeren A, Ferkol T, and Tosi M. Effects of bronchopulmonary inflammation induced by pseudomonas aeruginosa on adenovirus-mediated gene transfer to airway epithelial cells in mice. Gene Ther 5: 345-351, 1998.
-
(1998)
Gene Ther
, vol.5
, pp. 345-351
-
-
Van Heeckeren, A.1
Ferkol, T.2
Tosi, M.3
-
27
-
-
1342339826
-
Delivery of CFTR by adenoviral vector to cystic fibrosis mouse lung in a model of chronic Pseudomonas aeruginosa lung infection
-
Van Heeckeren AM, Scaria A, Schluchter MD, Ferkol TW, Wadsworth S, and Davis PB. Delivery of CFTR by adenoviral vector to cystic fibrosis mouse lung in a model of chronic Pseudomonas aeruginosa lung infection. Am J Physiol Lung Cell Mol Physiol 286: L717-L726, 2004.
-
(2004)
Am J Physiol Lung Cell Mol Physiol
, vol.286
-
-
Van Heeckeren, A.M.1
Scaria, A.2
Schluchter, M.D.3
Ferkol, T.W.4
Wadsworth, S.5
Davis, P.B.6
-
28
-
-
1342323766
-
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice
-
Van Heeckeren AM, Schluchter M, Xue L, Alvarez J, Freedman S, St. George J, and Davis PB. Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice. Infect Immun 72: 1479-1486, 2004.
-
(2004)
Infect Immun
, vol.72
, pp. 1479-1486
-
-
Van Heeckeren, A.M.1
Schluchter, M.2
Xue, L.3
Alvarez, J.4
Freedman, S.5
St. George, J.6
Davis, P.B.7
-
29
-
-
0036066896
-
Murine models of chronic Pseudomonas aeruginosa lung infection
-
Van Heeckeren AM and Schluchter MD. Murine models of chronic Pseudomonas aeruginosa lung infection. Lab Anim 36: 291-312, 2002.
-
(2002)
Lab Anim
, vol.36
, pp. 291-312
-
-
Van Heeckeren, A.M.1
Schluchter, M.D.2
-
31
-
-
0028892299
-
A mouse model for the delta F508 allele of cystic fibrosis
-
Zeiher BG, Eichwald E, Zabner J, Smith JJ, Puga AP, McCray PB Jr, Capecchi MR, Welsh MJ, and Thomas KR. A mouse model for the delta F508 allele of cystic fibrosis. J Clin Invest 96: 2051-2064, 1995.
-
(1995)
J Clin Invest
, vol.96
, pp. 2051-2064
-
-
Zeiher, B.G.1
Eichwald, E.2
Zabner, J.3
Smith, J.J.4
Puga, A.P.5
McCray Jr., P.B.6
Capecchi, M.R.7
Welsh, M.J.8
Thomas, K.R.9
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