-
1
-
-
33645130994
-
Cystic fibrosis since 1938
-
Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med 2006;173:475-482
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 475-482
-
-
Davis, P.B.1
-
2
-
-
0024423668
-
Identification of the cystic fibrosis gene: Genetic analysis
-
Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science 1989;245: 1073-1080
-
(1989)
Science
, vol.245
, pp. 1073-1080
-
-
Kerem, B.1
Rommens, J.M.2
Buchanan, J.A.3
-
3
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B-T, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989;245:1066-1073
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.-T.3
-
4
-
-
33745772850
-
New insights into cystic fibrosis: Molecular switches that regulate CFTCR
-
Guggino WB, Stanton BA. New insights into cystic fibrosis: molecular switches that regulate CFTCR. Nat Rev Mol Cell Biol 2006;7:426-436
-
(2006)
Nat Rev Mol Cell Biol
, vol.7
, pp. 426-436
-
-
Guggino, W.B.1
Stanton, B.A.2
-
5
-
-
0025863209
-
Generation of cAMP-activated chloride currents by expression of CFTCR
-
Anderson MP, Rich DP, Gregory RJ, Smith AE, Welsh MJ. Generation of cAMP-activated chloride currents by expression of CFTCR. Science 1991;251:679-682
-
(1991)
Science
, vol.251
, pp. 679-682
-
-
Anderson, M.P.1
Rich, D.P.2
Gregory, R.J.3
Smith, A.E.4
Welsh, M.J.5
-
6
-
-
0031046796
-
Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A
-
Jia Y, Mathews CJ, Hanrahan JW. Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J Biol Chem 1997;272:4978-4984
-
(1997)
J Biol Chem
, vol.272
, pp. 4978-4984
-
-
Jia, Y.1
Mathews, C.J.2
Hanrahan, J.W.3
-
7
-
-
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 1986;78:1245-1252
-
(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
-
8
-
-
0028982894
-
CFTCR as a cAMP-dependent regulator of sodium channels
-
Stutts MJ, Canessa CM, Olsen JC, et al. CFTCR as a cAMP-dependent regulator of sodium channels. Science 1995;269:847-850
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
-
9
-
-
0025242929
-
Defective intracellular transport and processing of CFTCR is the molecular basis of most cystic fibrosis
-
Cheng SH, Gregory RJ, Marshall J, et al. Defective intracellular transport and processing of CFTCR is the molecular basis of most cystic fibrosis. Cell 1990;63:827-834
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
-
10
-
-
15544371839
-
Assembly of functional CFTCR chloride channels
-
Riordan JR. Assembly of functional CFTCR chloride channels. Annu Rev Physiol 2005;67:701-718
-
(2005)
Annu Rev Physiol
, vol.67
, pp. 701-718
-
-
Riordan, J.R.1
-
11
-
-
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, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998;95:1005-1015
-
(1998)
Cell
, vol.95
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
-
12
-
-
0032530370
-
Coordinated clearance of periciliary liquid and mucus from airway surfaces
-
Matsui H, Randell SH, Peretti SW, Davis CW, Boucher RC. Coordinated clearance of periciliary liquid and mucus from airway surfaces. J Clin Invest 1998;102:1125-1131
-
(1998)
J Clin Invest
, vol.102
, pp. 1125-1131
-
-
Matsui, H.1
Randell, S.H.2
Peretti, S.W.3
Davis, C.W.4
Boucher, R.C.5
-
13
-
-
0034125685
-
Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia
-
Matsui H, Davis CW, Tarran R, Boucher RC. Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia. J Clin Invest 2000;105: 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
-
14
-
-
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. Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia. J Gen Physiol 2006;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
-
15
-
-
0034537007
-
Inhibition of amiloride-sensitive epithelial Na(+) absorption by extracellular nucleotides in human normal and cystic fibrosis airways
-
Mall M, Wissner A, Gonska T, et al. Inhibition of amiloride-sensitive epithelial Na(+) absorption by extracellular nucleotides in human normal and cystic fibrosis airways. Am J Respir Cell Mol Biol 2000;23:755-761
-
(2000)
Am J Respir Cell Mol Biol
, vol.23
, pp. 755-761
-
-
Mall, M.1
Wissner, A.2
Gonska, T.3
-
16
-
-
27444444705
-
Normal and cystic fibrosis airway surface liquid homeostasis: The effects of phasic shear stress and viral infections
-
Tarran R, Button B, Picher M, et al. Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections. J Biol Chem 2005; 280:35751-35759
-
(2005)
J Biol Chem
, vol.280
, pp. 35751-35759
-
-
Tarran, R.1
Button, B.2
Picher, M.3
-
17
-
-
33645964965
-
Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress
-
Tarran R, Button B, Boucher RC. Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress. Annu Rev Physiol 2006;68:543-561
-
(2006)
Annu Rev Physiol
, vol.68
, pp. 543-561
-
-
Tarran, R.1
Button, B.2
Boucher, R.C.3
-
18
-
-
23644434947
-
Viral respiratory infections in cystic fibrosis
-
van Ewijk BE, van der Zalm MM, Wolfs TF, van der Ent CK. Viral respiratory infections in cystic fibrosis. J Cyst Fibres 2005;4(Suppl 2):31-36
-
(2005)
J Cyst Fibres
, vol.4
, Issue.SUPPL. 2
, pp. 31-36
-
-
van Ewijk, B.E.1
van der Zalm, M.M.2
Wolfs, T.F.3
van der Ent, C.K.4
-
19
-
-
0023775479
-
Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis
-
Abman SH, Ogle JW, Butler-Simon N, Rumack CM, Accurso FJ. Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis. J Pediatr 1988;113:826-830
-
(1988)
J Pediatr
, vol.113
, pp. 826-830
-
-
Abman, S.H.1
Ogle, J.W.2
Butler-Simon, N.3
Rumack, C.M.4
Accurso, F.J.5
-
21
-
-
75549102827
-
Studies on pulmonary secretions, I: The overall chemical composition of pulmonary secretions from patients with cystic fibrosis, bronchiectasis, and laryngectomy
-
Matthews LW, Spector S, Lemm J, Potter JL. Studies on pulmonary secretions, I: The overall chemical composition of pulmonary secretions from patients with cystic fibrosis, bronchiectasis, and laryngectomy. Am Rev Respir Dis 1963; 88:199-204
-
(1963)
Am Rev Respir Dis
, vol.88
, pp. 199-204
-
-
Matthews, L.W.1
Spector, S.2
Lemm, J.3
Potter, J.L.4
-
22
-
-
0014108774
-
-
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 1967;96:83-87
-
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 1967;96:83-87
-
-
-
-
23
-
-
36849037262
-
The pathogenesis of fibrocystic disease of the pancreas: A study of 36 cases with special reference to the pulmonary lesions
-
Zuelzer WW, Newton WA Jr. The pathogenesis of fibrocystic disease of the pancreas: a study of 36 cases with special reference to the pulmonary lesions. Pediatrics 1949; 4:53-69
-
(1949)
Pediatrics
, vol.4
, pp. 53-69
-
-
Zuelzer, W.W.1
Newton Jr., W.A.2
-
24
-
-
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 1996; 21:77-83
-
(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
-
25
-
-
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 1996;153:1503-1509
-
(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
-
26
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006; 354:241-250
-
(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
-
27
-
-
30944466084
-
A controlled trial of long-term Inhaled hypertonic saline in patients with cystic fibrosis
-
Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term Inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354:229-240
-
(2006)
N Engl J Med
, vol.354
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
-
28
-
-
0025997759
-
Sodium transport and intracellular sodium activity in cultured human nasal epithelium
-
Willumsen NJ, Boucher RC. Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am J Physiol 1991;261(2 Pt 1):C319-C331
-
(1991)
Am J Physiol
, vol.261
, Issue.2 PART 1
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
29
-
-
0025995248
-
Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium
-
Willumsen NJ, Boucher RC. Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am J Physiol 1991;261(2 Pt 1):C332-C341
-
(1991)
Am J Physiol
, vol.261
, Issue.2 PART 1
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
32
-
-
0024547744
-
Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelium
-
Willumsen NJ, Boucher RC. Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelium. Am J Physiol 1989;256(5 Pt 1):C1054-C1063
-
(1989)
Am J Physiol
, vol.256
, Issue.5 PART 1
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
33
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
Knowles MR, Stutts MJ, Spock A, Fischer N, Gatzy JT, Boucher RC. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 1983;221:1067-1070
-
(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
-
34
-
-
0021352368
-
Bioelectric properties and ion flow across excised human bronchi
-
Knowles M, Murray G, Shallal J, et al. Bioelectric properties and ion flow across excised human bronchi. J Appl Physiol 1984;56:868-877
-
(1984)
J Appl Physiol
, vol.56
, pp. 868-877
-
-
Knowles, M.1
Murray, G.2
Shallal, J.3
-
35
-
-
0024557278
-
2+ ionophores in cystic fibrosis airway epithelia
-
2+ ionophores in cystic fibrosis airway epithelia. Am J Physiol 1989;256(2 Pt 1):C226-C233
-
(1989)
Am J Physiol
, vol.256
, Issue.2 PART 1
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
36
-
-
33748744717
-
Hypertonic saline therapy in cystic fibrosis: Evidence against the proposed mechanism involving aquaporins
-
Levin MH, Sullivan S, Nielson D, Yang B, Finkbeiner WE, Verkman AS. Hypertonic saline therapy in cystic fibrosis: evidence against the proposed mechanism involving aquaporins. J Biol Chem 2006;281:25803-25812
-
(2006)
J Biol Chem
, vol.281
, pp. 25803-25812
-
-
Levin, M.H.1
Sullivan, S.2
Nielson, D.3
Yang, B.4
Finkbeiner, W.E.5
Verkman, A.S.6
-
37
-
-
0034885663
-
The CF salt controversy: In vivo observations and therapeutic approaches
-
Tarran R, Grubb BR, Parsons D, et al. The CF salt controversy: in vivo observations and therapeutic approaches. Mol Cell 2001;8:149-158
-
(2001)
Mol Cell
, vol.8
, pp. 149-158
-
-
Tarran, R.1
Grubb, B.R.2
Parsons, D.3
-
38
-
-
0037438694
-
Increasing concentration of inhaled saline with or without amiloride: Effect on mucociliary clearance in normal subjects
-
Sood N, Bennett WD, Zeman K, et al. Increasing concentration of inhaled saline with or without amiloride: effect on mucociliary clearance in normal subjects. Am J Respir Crit Care Med 2002;167:158-163
-
(2002)
Am J Respir Crit Care Med
, vol.167
, pp. 158-163
-
-
Sood, N.1
Bennett, W.D.2
Zeman, K.3
-
39
-
-
0030814886
-
Ion composition of airway surface liquid of patients with cystic fibrosis as compared to normal and disease-control subjects
-
Knowles MR, Robinson JM, Wood RE, et al. Ion composition of airway surface liquid of patients with cystic fibrosis as compared to normal and disease-control subjects. J Clin Invest 1997;100:2588-2595
-
(1997)
J Clin Invest
, vol.100
, pp. 2588-2595
-
-
Knowles, M.R.1
Robinson, J.M.2
Wood, R.E.3
-
40
-
-
0037216290
-
Desiccation and hypertonicity of the airway surface fluid and thermally induced asthma
-
Kotaru C, Hejal RB, Finigan JH, et al. Desiccation and hypertonicity of the airway surface fluid and thermally induced asthma. J Appl Physiol 2003;94:227-233
-
(2003)
J Appl Physiol
, vol.94
, pp. 227-233
-
-
Kotaru, C.1
Hejal, R.B.2
Finigan, J.H.3
|