-
1
-
-
0001752544
-
Cystic fibrosis
-
eds Scriver CR, et al. (McGraw-Hill, New York), 8th Ed.
-
Welsh MJ, Ramsey BW, Accurso F, Cutting GR (2001) Cystic fibrosis. The Metabolic and Molecular Basis of Inherited Disease, eds Scriver CR, et al. (McGraw-Hill, New York), 8th Ed.
-
(2001)
The Metabolic and Molecular Basis of Inherited Disease
-
-
Welsh, M.J.1
Ramsey, B.W.2
Accurso, F.3
Cutting, G.R.4
-
2
-
-
0032913809
-
Physiological basis of cystic fibrosis: A historical perspective
-
Quinton PM (1999) Physiological basis of cystic fibrosis: A historical perspective. Physiol Rev 79(1, Suppl):S3-S22.
-
(1999)
Physiol Rev
, vol.79
, Issue.1 SUPPL.
-
-
Quinton, P.M.1
-
3
-
-
0032910501
-
The genesis of cystic fibrosis lung disease
-
Wine JJ (1999) The genesis of cystic fibrosis lung disease. J Clin Invest 103:309-312.
-
(1999)
J Clin Invest
, vol.103
, pp. 309-312
-
-
Wine, J.J.1
-
6
-
-
33645130994
-
Cystic fibrosis since 1938
-
Davis PB (2006) Cystic fibrosis since 1938. Am J Respir Crit Care Med 173:475-482.
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 475-482
-
-
Davis, P.B.1
-
7
-
-
34047166052
-
Airway surface dehydration in cystic fibrosis: Pathogenesis and therapy
-
Boucher RC (2007) Airway surface dehydration in cystic fibrosis: Pathogenesis and therapy. Annu Rev Med 58:157-170.
-
(2007)
Annu Rev Med
, vol.58
, pp. 157-170
-
-
Boucher, R.C.1
-
8
-
-
0019809960
-
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
-
Knowles M, Gatzy JT, Boucher RCJ (1981) Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 305:1489-1495. (Pubitemid 12244203)
-
(1981)
New England Journal of Medicine
, vol.305
, Issue.25
, pp. 1489-1495
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
9
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
Knowles MR, et al. (1983) Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221:1067-1070. (Pubitemid 13041960)
-
(1983)
Science
, vol.221
, Issue.4615
, pp. 1067-1070
-
-
Knowles, M.R.1
Stutts, M.J.2
Spock, A.3
-
13
-
-
0026645366
-
cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia
-
Smith JJ, 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
-
14
-
-
2442453783
-
Standardized Procedure for Measurement of Nasal Potential Difference: An Outcome Measure in Multicenter Cystic Fibrosis Clinical Trials
-
DOI 10.1002/ppul.10448
-
Standaert TA, et al. (2004) Standardized procedure for measurement of nasal potential difference: An outcome measure in multicenter cystic fibrosis clinical trials. Pediatr Pulmonol 37:385-392. (Pubitemid 38620059)
-
(2004)
Pediatric Pulmonology
, vol.37
, Issue.5
, pp. 385-392
-
-
Standaert, T.A.1
Boitano, L.2
Emerson, J.3
Milgram, L.J.H.4
Konstan, M.W.5
Hunter, J.6
Berclaz, P.-Y.7
Brass, L.8
Zeitlin, P.L.9
Hammond, K.10
Davies, Z.11
Foy, C.12
Noone, P.G.13
Knowles, M.R.14
-
15
-
-
36348979793
-
Sodium channels and cystic fibrosis
-
DOI 10.1378/chest.07-0288
-
Donaldson SH, Boucher RC (2007) Sodium channels and cystic fibrosis. Chest 132: 1631-1636. (Pubitemid 350156022)
-
(2007)
Chest
, vol.132
, Issue.5
, pp. 1631-1636
-
-
Donaldson, S.H.1
Boucher, R.C.2
-
16
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts MJ, et al. (1995) CFTR as a cAMP-dependent regulator of sodium channels. Science 269:847-850.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
-
17
-
-
18944361982
-
CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes
-
DOI 10.1113/jphysiol.2004.079046
-
Nagel G, et al. (2005) CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes. J Physiol 564:671-682. (Pubitemid 40704136)
-
(2005)
Journal of Physiology
, vol.564
, Issue.3
, pp. 671-682
-
-
Nagel, G.1
Barbry, P.2
Chabot, H.3
Brochiero, E.4
Hartung, K.5
Grygorczyk, R.6
-
18
-
-
52949154301
-
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs
-
Rogers CS, et al. (2008) Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science 321:1837-1841.
-
(2008)
Science
, vol.321
, pp. 1837-1841
-
-
Rogers, C.S.1
-
19
-
-
77749237132
-
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis
-
Meyerholz DK, Stoltz DA, Pezzulo AA, Welsh MJ (2010) Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. Am J Pathol 176:1377-1389.
-
(2010)
Am J Pathol
, vol.176
, pp. 1377-1389
-
-
Meyerholz, D.K.1
Stoltz, D.A.2
Pezzulo, A.A.3
Welsh, M.J.4
-
20
-
-
79952779452
-
The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs
-
Ostedgaard LS, et al. (2011) The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. Sci Transl Med 3: 74ra24.
-
(2011)
Sci Transl Med
, vol.3
-
-
Ostedgaard, L.S.1
-
21
-
-
77952974496
-
Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth
-
Stoltz DA, et al. (2010) Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2(29): 29ra31.
-
(2010)
Sci Transl Med
, vol.2
, Issue.29
-
-
Stoltz, D.A.1
-
22
-
-
78649915334
-
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia
-
Chen J-H, et al. (2010) Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell 143:911-923.
-
(2010)
Cell
, vol.143
, pp. 911-923
-
-
Chen, J.-H.1
-
23
-
-
0037274990
-
ENaC-CFTR interactions: The role of electrical coupling of ion fluxes explored in an epithelial cell model
-
Horisberger JD (2003) ENaC-CFTR interactions: The role of electrical coupling of ion fluxes explored in an epithelial cell model. Pflugers Arch 445:522-528.
-
(2003)
Pflugers Arch
, vol.445
, pp. 522-528
-
-
Horisberger, J.D.1
-
24
-
-
0025883059
-
An analytical model of ionic movements in airway epithelial cells
-
Duszyk M, French AS (1991) An analytical model of ionic movements in airway epithelial cells. J Theor Biol 151:231-247.
-
(1991)
J Theor Biol
, vol.151
, pp. 231-247
-
-
Duszyk, M.1
French, A.S.2
-
25
-
-
0036367311
-
An in vitro model of differentiated human airway epithelia: Methods and evaluation of primary cultures
-
ed Wise C (Humana, Totowa, NJ)
-
Karp PH, et al. (2002) An in vitro model of differentiated human airway epithelia: Methods and evaluation of primary cultures. Epithelial Cell Culture Protocols, ed Wise C (Humana, Totowa, NJ), Vol 188, pp 115-137.
-
(2002)
Epithelial Cell Culture Protocols
, vol.188
, pp. 115-137
-
-
Karp, P.H.1
-
27
-
-
0020626528
-
Inhibition of chloride secretion by furosemide in canine tracheal epithelium
-
Welsh MJ (1983) Inhibition of chloride secretion by furosemide in canine tracheal epithelium. J Membr Biol 71:219-226.
-
(1983)
J Membr Biol
, vol.71
, pp. 219-226
-
-
Welsh, M.J.1
-
28
-
-
0015857672
-
Edge damage effect on electrical measurements of frog skin
-
Helman SI, Miller DA (1973) Edge damage effect on electrical measurements of frog skin. Am J Physiol 225:972-977.
-
(1973)
Am J Physiol
, vol.225
, pp. 972-977
-
-
Helman, S.I.1
Miller, D.A.2
-
29
-
-
78649647597
-
- transport in mucin secretion: Lessons from cystic fibrosis
-
- transport in mucin secretion: Lessons from cystic fibrosis. Am J Physiol Cell Physiol 299:C1222-C1233.
-
(2010)
Am J Physiol Cell Physiol
, vol.299
-
-
Quinton, P.M.1
-
30
-
-
0032160101
-
Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro
-
Zabner J, Smith JJ, Karp PH, Widdicombe JH, Welsh MJ (1998) Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro. Mol Cell 2:397-403. (Pubitemid 128379280)
-
(1998)
Molecular Cell
, vol.2
, Issue.3
, pp. 397-403
-
-
Zabner, J.1
Smith, J.J.2
Karp, P.H.3
Widdicombe, J.H.4
Welsh, M.J.5
-
32
-
-
0036896008
-
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion
-
DOI 10.1172/JCI200216112
-
Ma T, et al. (2002) Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J Clin Invest 110: 1651-1658. (Pubitemid 35424243)
-
(2002)
Journal of Clinical Investigation
, vol.110
, Issue.11
, pp. 1651-1658
-
-
Ma, T.1
Thiagarajah, J.R.2
Yang, H.3
Sonawane, N.D.4
Folli, C.5
Galietta, L.J.V.6
Verkman, A.S.7
-
34
-
-
0032962320
-
Pathophysiology of gene-targeted mouse models for cystic fibrosis
-
Grubb BR, Boucher RC (1999) Pathophysiology of gene-targeted mouse models for cystic fibrosis. Physiol Rev 79(1, Suppl):S193-S214. (Pubitemid 29058915)
-
(1999)
Physiological Reviews
, vol.79
, Issue.1 SUPPL. 1
-
-
Grubb, B.R.1
Boucher, R.C.2
-
35
-
-
77956379893
-
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis
-
Sun X, et al. (2010) Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest 120:3149-3160.
-
(2010)
J Clin Invest
, vol.120
, pp. 3149-3160
-
-
Sun, X.1
-
36
-
-
0032912589
-
Structure and function of the CFTR chloride channel
-
Sheppard DN, Welsh MJ (1999) Structure and function of the CFTR chloride channel. Physiol Rev 79(1, Suppl):S23-S45. (Pubitemid 29058908)
-
(1999)
Physiological Reviews
, vol.79
, Issue.1 SUPPL. 1
-
-
Sheppard, D.N.1
Welsh, M.J.2
-
37
-
-
0025997759
-
Sodium transport and intracellular sodium activity in cultured human nasal epithelium
-
Willumsen NJ, Boucher RCJ (1991) Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am J Physiol 261:C319-C331.
-
(1991)
Am J Physiol
, vol.261
-
-
Willumsen, N.J.1
Boucher, R.C.J.2
-
38
-
-
0025995248
-
Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium
-
Willumsen NJ, Boucher RCJ (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.J.2
-
39
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
DOI 10.1016/S0092-8674(00)81724-9
-
Matsui H, et al. (1998) Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95:1005-1015. (Pubitemid 29019052)
-
(1998)
Cell
, vol.95
, Issue.7
, 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
-
40
-
-
0027363212
-
Altered fluid transport across airway epithelium in cystic fibrosis
-
Jiang C, Finkbeiner WE, Widdicombe JH, McCray PB, Jr., Miller SS (1993) Altered fluid transport across airway epithelium in cystic fibrosis. Science 262:424-427. (Pubitemid 23353385)
-
(1993)
Science
, vol.262
, Issue.5132
, pp. 424-427
-
-
Jiang, C.1
Finkbeiner, W.E.2
Widdicombe, J.H.3
McCray Jr., P.B.4
Miller, S.S.5
-
41
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
Van Goor F, et al. (2009) Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci USA 106:18825-18830.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 18825-18830
-
-
Van Goor, F.1
-
42
-
-
79960003498
-
Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching
-
10.1096/fj.10-179549
-
Derichs N, Jin BJ, Song Y, Finkbeiner WE, Verkman AS (2011) Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching. FASEB J, 10.1096/fj.10-179549.
-
(2011)
FASEB J
-
-
Derichs, N.1
Jin, B.J.2
Song, Y.3
Finkbeiner, W.E.4
Verkman, A.S.5
-
43
-
-
79952216879
-
Quantification of periciliary fluid (PCL) height in human airway biopsies is feasible, but not suitable as a biomarker
-
Griesenbach U, et al. (2011) Quantification of periciliary fluid (PCL) height in human airway biopsies is feasible, but not suitable as a biomarker. Am J Respir Cell Mol Biol 44:309-315.
-
(2011)
Am J Respir Cell Mol Biol
, vol.44
, pp. 309-315
-
-
Griesenbach, U.1
-
44
-
-
2442427527
-
Epithelial Sodium Channels Are Activated by Furin-dependent Proteolysis
-
DOI 10.1074/jbc.C400080200
-
Hughey RP, et al. (2004) Epithelial sodium channels are activated by furin-dependent proteolysis. J Biol Chem 279:18111-18114. (Pubitemid 38623222)
-
(2004)
Journal of Biological Chemistry
, vol.279
, Issue.18
, pp. 18111-18114
-
-
Hughey, R.P.1
Bruns, J.B.2
Kinlough, C.L.3
Harkleroad, K.L.4
Tong, Q.5
Carattino, M.D.6
Johnson, J.P.7
Stockand, J.D.8
Kleyman, T.R.9
-
45
-
-
27844476338
-
+ channel trafficking
-
+ channel trafficking. Endocrinology 146:5079-5085.
-
(2005)
Endocrinology
, vol.146
, pp. 5079-5085
-
-
Snyder, P.M.1
-
46
-
-
78649743537
-
Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking
-
Myerburg MM, 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 Respir Cell Mol Biol 43:712-719.
-
(2010)
Am J Respir 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
-
48
-
-
34447523813
-
Cystic fibrosis: Lessons from the sweat gland
-
Bethesda
-
Quinton PM (2007) Cystic fibrosis: Lessons from the sweat gland. Physiology (Bethesda) 22:212-225.
-
(2007)
Physiology
, vol.22
, pp. 212-225
-
-
Quinton, P.M.1
-
49
-
-
33646348165
-
Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands
-
DOI 10.1074/jbc.M512766200
-
Joo NS, Irokawa T, Robbins RC, Wine JJ (2006) Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands. J Biol Chem 281:7392-7398. (Pubitemid 43847509)
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.11
, pp. 7392-7398
-
-
Nam, S.J.1
Irokawa, T.2
Robbins, R.C.3
Wine, J.J.4
|