-
1
-
-
0027162649
-
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
-
Welsh, M. J., and A. E. Smith. 1993. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73:1251-1254.
-
(1993)
Cell
, vol.73
, pp. 1251-1254
-
-
Welsh, M.J.1
Smith, A.E.2
-
2
-
-
0027141851
-
Elemental composition of human airway surface fluid in healthy and diseased airways
-
Joris, L., I. Dab, and P. M. Quinton. 1993. Elemental composition of human airway surface fluid in healthy and diseased airways. Am. Rev. Respir. Dis. 148:1633-1637.
-
(1993)
Am. Rev. Respir. Dis.
, vol.148
, pp. 1633-1637
-
-
Joris, L.1
Dab, I.2
Quinton, P.M.3
-
3
-
-
0029870085
-
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
-
Smith, J. J., S. M. Travis, E. P. Greenberg, and M. J. Welsh. 1996. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 85:229-236.
-
(1996)
Cell
, vol.85
, pp. 229-236
-
-
Smith, J.J.1
Travis, S.M.2
Greenberg, E.P.3
Welsh, M.J.4
-
4
-
-
0031156473
-
Cftr gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium
-
Davies, J. C., M. Stern, A. Dewar, N. J. Caplen, F. M. Munkonge, T. Pitt, F. Sorgi, L. Huang, A. Bush, D. M. Geddes, and E. W. F. W. Alton. 1997. Cftr gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium. Am. J. Respir. Cell Mol. Biol. 16:657-663.
-
(1997)
Am. J. Respir. Cell Mol. Biol.
, vol.16
, pp. 657-663
-
-
Davies, J.C.1
Stern, M.2
Dewar, A.3
Caplen, N.J.4
Munkonge, F.M.5
Pitt, T.6
Sorgi, F.7
Huang, L.8
Bush, A.9
Geddes, D.M.10
Alton, E.W.F.W.11
-
5
-
-
0029874138
-
The NF-kappa B and I kappa B proteins: New discoveries and insights
-
Baldwin, A. S., Jr. 1996. The NF-kappa B and I kappa B proteins: new discoveries and insights. Annu. Rev. Immunol. 14:649-683.
-
(1996)
Annu. Rev. Immunol.
, vol.14
, pp. 649-683
-
-
Baldwin A.S., Jr.1
-
6
-
-
0030909647
-
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice
-
Zahm, J. M., D. Gaillard, F. Dupuit, J. Hinnrasky, D. Porteous, J. R. Dorin, and E. Puchelle. 1997. Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am. J. Physiol. 272: C853-C859.
-
(1997)
Am. J. Physiol.
, vol.272
-
-
Zahm, J.M.1
Gaillard, D.2
Dupuit, F.3
Hinnrasky, J.4
Porteous, D.5
Dorin, J.R.6
Puchelle, E.7
-
7
-
-
0031047239
-
Gene therapy for cystic fibrosis: Steady progress, should do well
-
Alton, E. W. F. W., and D. M. Geddes. 1997. Gene therapy for cystic fibrosis: steady progress, should do well. Eur. Respir. J. 10:257-259.
-
(1997)
Eur. Respir. J.
, vol.10
, pp. 257-259
-
-
Alton, E.W.F.W.1
Geddes, D.M.2
-
8
-
-
0038923210
-
Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis
-
Knowles, M. R., K. N. Olivier, K. W. Hohneker, J. Robinson, W. D. Bennett, and R. C. Boucher. 1995. Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis. Adv. Exp. Med. Biol. 290:119-128.
-
(1995)
Adv. Exp. Med. Biol.
, vol.290
, pp. 119-128
-
-
Knowles, M.R.1
Olivier, K.N.2
Hohneker, K.W.3
Robinson, J.4
Bennett, W.D.5
Boucher, R.C.6
-
9
-
-
0029813969
-
A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction
-
Dorin, J. R., R. Farley, S. Webb, S. N. Smith, E. Farini, S. J. Delaney, B. J. Wainwright, E. W. F. W. Alton, and D. J. Porteous. 1996. A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction. Gene Ther. 3:797-801.
-
(1996)
Gene Ther.
, vol.3
, pp. 797-801
-
-
Dorin, J.R.1
Farley, R.2
Webb, S.3
Smith, S.N.4
Farini, E.5
Delaney, S.J.6
Wainwright, B.J.7
Alton, E.W.F.W.8
Porteous, D.J.9
-
10
-
-
0028802726
-
Cyclic nucleotide phosphodiesterases: Functional implications of multiple isoforms
-
Beavo, J. A. 1995. Cyclic nucleotide phosphodiesterases: functional implications of multiple isoforms. Physiol. Rev. 75:725-748.
-
(1995)
Physiol. Rev.
, vol.75
, pp. 725-748
-
-
Beavo, J.A.1
-
11
-
-
0028577602
-
Phosphatase inhibitors activate normal and defective CFTR chloride channels
-
Becq, F., T. J. Jensen, X.-B. Chang, A. Savoia, J. M. Rommens, L.-C. Tsui, M. Buchwald, J. R. Riordan, and J. W. Hanrahan. 1994. Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc. Natl. Acad. Sci. USA 91:9160-9164.
-
(1994)
Proc. Natl. Acad. Sci. USA
, vol.91
, pp. 9160-9164
-
-
Becq, F.1
Jensen, T.J.2
Chang, X.-B.3
Savoia, A.4
Rommens, J.M.5
Tsui, L.-C.6
Buchwald, M.7
Riordan, J.R.8
Hanrahan, J.W.9
-
12
-
-
0031888308
-
Effect of IBMX and alkaline phosphatase inhibitors on Cl secretion in G551D cystic fibrosis mutant mice
-
Smith, S. N., S. J. Delaney, J. R. Dorin, R. Farley, D. M. Geddes, D. J. Porteous, B. J. Wainwright, and E. W. F. W. Alton. 1998. Effect of IBMX and alkaline phosphatase inhibitors on Cl secretion in G551D cystic fibrosis mutant mice. Am. J. Physiol. 274:C492-C499.
-
(1998)
Am. J. Physiol.
, vol.274
-
-
Smith, S.N.1
Delaney, S.J.2
Dorin, J.R.3
Farley, R.4
Geddes, D.M.5
Porteous, D.J.6
Wainwright, B.J.7
Alton, E.W.F.W.8
-
13
-
-
0027586201
-
Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia
-
Grubb, B. R., E. Lazarowski, M. Knowles, and R. C. Boucher. 1993. Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia. Am. J. Respir. Cell Mol. Biol. 8:454-460.
-
(1993)
Am. J. Respir. Cell Mol. Biol.
, vol.8
, pp. 454-460
-
-
Grubb, B.R.1
Lazarowski, E.2
Knowles, M.3
Boucher, R.C.4
-
14
-
-
0029556721
-
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells
-
Kelley, T. J., L. Al-Nakkash, and M. L. Drumm. 1995. CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells. Am. J. Respir. Cell Mol. Biol. 113:657-664.
-
(1995)
Am. J. Respir. Cell Mol. Biol.
, vol.113
, pp. 657-664
-
-
Kelley, T.J.1
Al-Nakkash, L.2
Drumm, M.L.3
-
15
-
-
0022639865
-
Pharmacology of the bypiridines: Amrinone and milrinone
-
Alousi, A. A., and D. C. Johnson. 1986. Pharmacology of the bypiridines: amrinone and milrinone. Circulation 73:11110-11124.
-
(1986)
Circulation
, vol.73
, pp. 11110-11124
-
-
Alousi, A.A.1
Johnson, D.C.2
-
16
-
-
0029162301
-
A pharmacokinetic and pharmacodynamic evaluation of milrinone in adults undergoing cardiac surgery
-
Butterworth, J. F., IV, R. L. Hines, R. L. Royster, and R. L. James. 1995. A pharmacokinetic and pharmacodynamic evaluation of milrinone in adults undergoing cardiac surgery. Anesth. Analg. 81:783-792.
-
(1995)
Anesth. Analg.
, vol.81
, pp. 783-792
-
-
Butterworth J.F. IV1
Hines, R.L.2
Royster, R.L.3
James, R.L.4
-
17
-
-
0029556721
-
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells
-
Kelley, T. J., L. Al-Nakkash, C. U. Cotton, and M. L. Drumm. 1995. CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells. Am. J. Respir. Cell Mol. Biol. 13:657-664.
-
(1995)
Am. J. Respir. Cell Mol. Biol.
, vol.13
, pp. 657-664
-
-
Kelley, T.J.1
Al-Nakkash, L.2
Cotton, C.U.3
Drumm, M.L.4
-
18
-
-
0008600915
-
Inhibition of specific phosphodiesterases in CF airway epithelial cells activates mutant CFTRs
-
Drumm, M. L., and T. J. Kelley. 1995. Inhibition of specific phosphodiesterases in CF airway epithelial cells activates mutant CFTRs. Pediatr. Pulmonol. 20(Suppl. 12):150-151.
-
(1995)
Pediatr. Pulmonol.
, vol.20
, Issue.SUPPL. 12
, pp. 150-151
-
-
Drumm, M.L.1
Kelley, T.J.2
-
19
-
-
0030900255
-
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant DF508 in murine nasal epithelium
-
Kelley, T. J., K. Thomas, L. J. H. Milgram, and M. L. Drumm. 1997. In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant DF508 in murine nasal epithelium. Proc. Natl. Acad. Sci. USA 94: 2604-2608.
-
(1997)
Proc. Natl. Acad. Sci. USA
, vol.94
, pp. 2604-2608
-
-
Kelley, T.J.1
Thomas, K.2
Milgram, L.J.H.3
Drumm, M.L.4
-
20
-
-
0028469238
-
Ultrastructural localization of variant forms of cystic fibrosis transmembrane regulator in human bronchial epithelia of xenographs
-
Yang, Y., J. F. Engelhardt, and J. M. Wilson. 1994. Ultrastructural localization of variant forms of cystic fibrosis transmembrane regulator in human bronchial epithelia of xenographs. Am. J. Respir. Cell Mol. Biol. 11:7-15.
-
(1994)
Am. J. Respir. Cell Mol. Biol.
, vol.11
, pp. 7-15
-
-
Yang, Y.1
Engelhardt, J.F.2
Wilson, J.M.3
-
21
-
-
9044236531
-
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations
-
Delaney, S. J., E. W. F. W. Alton, S. N. Smith, D. P. Lunn, R. Farley, P. K. Lovelock, S. A. Thompson, D. A. Hume, D. Lamb, D. J. Porteous, J. R. Dorin, and B. J. Wainwright. 1996. Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations. EMBO J. 15:955-963.
-
(1996)
EMBO J.
, vol.15
, pp. 955-963
-
-
Delaney, S.J.1
Alton, E.W.F.W.2
Smith, S.N.3
Lunn, D.P.4
Farley, R.5
Lovelock, P.K.6
Thompson, S.A.7
Hume, D.A.8
Lamb, D.9
Porteous, D.J.10
Dorin, J.R.11
Wainwright, B.J.12
-
22
-
-
0029153220
-
Generation and characterization of a delta F508 cystic fibrosis mouse model
-
Colledge, W. H., B. S. Abella, K. W. Southern, R. Ratcliff, C. Jiang, S. H. Cheng, L. J. Macvinish, J. R. Anderson, A. W. Cuthbert, and M. J. Evans. 1995. Generation and characterization of a delta F508 cystic fibrosis mouse model. Nat. Genet. 10:445-452.
-
(1995)
Nat. Genet.
, vol.10
, pp. 445-452
-
-
Colledge, W.H.1
Abella, B.S.2
Southern, K.W.3
Ratcliff, R.4
Jiang, C.5
Cheng, S.H.6
Macvinish, L.J.7
Anderson, J.R.8
Cuthbert, A.W.9
Evans, M.J.10
-
23
-
-
0028020058
-
Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium
-
Middleton, P. G., D. M. Geddes, and E. W. F. W. Alton. 1994. Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium. Eur. Respir. J. 7:2050-2056.
-
(1994)
Eur. Respir. J.
, vol.7
, pp. 2050-2056
-
-
Middleton, P.G.1
Geddes, D.M.2
Alton, E.W.F.W.3
-
24
-
-
0028991826
-
In vivo nasal potential difference techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis
-
Knowles, M. R., A. M. Paradiso, and R. C. Boucher. 1995. In vivo nasal potential difference techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum. Gene Ther. 6:445-455.
-
(1995)
Hum. Gene Ther.
, vol.6
, pp. 445-455
-
-
Knowles, M.R.1
Paradiso, A.M.2
Boucher, R.C.3
-
25
-
-
0029086914
-
Direct depressant effect of phosphodiesterase inhibitors on ATPase activity of rat cardiac myofibrils
-
Polla, B., V. Cappelli, M. Canepari, M. C. Zanardi, and C. Reggiani. 1995. Direct depressant effect of phosphodiesterase inhibitors on ATPase activity of rat cardiac myofibrils. Can. J. Physiol. Pharmacol. 73:661-664.
-
(1995)
Can. J. Physiol. Pharmacol.
, vol.73
, pp. 661-664
-
-
Polla, B.1
Cappelli, V.2
Canepari, M.3
Zanardi, M.C.4
Reggiani, C.5
-
26
-
-
0028919433
-
Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: Comparison with humans
-
Smith, S. N., D. M. Steel, P. G. Middleton, F. M. Munkonge, D. M. Geddes, N. J. Caplen, D. J. Porteous, J. R. Dorin, and E. W. F. W. Alton. 1995. Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans. Am. J. Physiol. 268:C297-C307.
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Smith, S.N.1
Steel, D.M.2
Middleton, P.G.3
Munkonge, F.M.4
Geddes, D.M.5
Caplen, N.J.6
Porteous, D.J.7
Dorin, J.R.8
Alton, E.W.F.W.9
-
28
-
-
0024443061
-
Forskolin, a specific stimulator of adenylate cyclase or a diterpene with multiple sites of action?
-
Laurenza, A., E. M. Sutkowski, and K. B. Seamon. 1989. Forskolin, a specific stimulator of adenylate cyclase or a diterpene with multiple sites of action? Trends Pharmacol. Sci. 10:442-147.
-
(1989)
Trends Pharmacol. Sci.
, vol.10
, pp. 442-1147
-
-
Laurenza, A.1
Sutkowski, E.M.2
Seamon, K.B.3
|