-
2
-
-
0025868103
-
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity
-
Anderson MP, Gregory RJ, Thompson S, Souza DW, Paul S, Mulligan RC, Smith AE, Welsh MJ. Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science 253: 202-205, 1991.
-
(1991)
Science
, vol.253
, pp. 202-205
-
-
Anderson, M.P.1
Gregory, R.J.2
Thompson, S.3
Souza, D.W.4
Paul, S.5
Mulligan, R.C.6
Smith, A.E.7
Welsh, M.J.8
-
3
-
-
64549085967
-
SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia
-
Bertrand CA, Zhang R, Pilewski JM, Frizzell RA. SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia. J Gen Physiol 133: 421-438, 2009.
-
(2009)
J Gen Physiol
, vol.133
, pp. 421-438
-
-
Bertrand, C.A.1
Zhang, R.2
Pilewski, J.M.3
Frizzell, R.A.4
-
4
-
-
34147174942
-
Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia
-
Button B, Picher M, Boucher RC. Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia. J Physiol 580: 577-592, 2007.
-
(2007)
J Physiol
, vol.580
, pp. 577-592
-
-
Button, B.1
Picher, M.2
Boucher, R.C.3
-
5
-
-
78649915334
-
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia
-
Chen JH, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector MV, Reznikov LR, Launspach JL, Chaloner K, Zabner J, Welsh MJ. Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell 143: 911-923, 2010.
-
(2010)
Cell
, vol.143
, pp. 911-923
-
-
Chen, J.H.1
Stoltz, D.A.2
Karp, P.H.3
Ernst, S.E.4
Pezzulo, A.A.5
Moninger, T.O.6
Rector, M.V.7
Reznikov, L.R.8
Launspach, J.L.9
Chaloner, K.10
Zabner, J.11
Welsh, M.J.12
-
6
-
-
0026640380
-
Defective epithelial chloride transport in a gene targeted mouse model of cystic fibrosis
-
Clarke LL, Grubb BR, Gabriel SE, Smithies O, Koller BH, 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
-
7
-
-
0025094612
-
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer
-
Drumm ML, Pope HA, Cliff WH, Rommens JM, Marvin SA, Tsui LC, Collins FS, Frizzell RA, Wilson JM. Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell 62: 1227-1233, 1990.
-
(1990)
Cell
, vol.62
, pp. 1227-1233
-
-
Drumm, M.L.1
Pope, H.A.2
Cliff, W.H.3
Rommens, J.M.4
Marvin, S.A.5
Tsui, L.C.6
Collins, F.S.7
Frizzell, R.A.8
Wilson, J.M.9
-
8
-
-
0028588619
-
Cell specific expression of epithelial sodium channel α, β, and γ subunits in aldosterone- responsive epithelia from the rat: Localization by in situ hybridization and immunocytochemistry
-
Duc C, Farman N, Canessa CM, Bonvalet JP, Rossier BC. Cell specific expression of epithelial sodium channel α, β, and γ subunits in aldosterone- responsive epithelia from the rat: localization by in situ hybridization and immunocytochemistry. J Cell Biol 127: 1907-1921, 1994.
-
(1994)
J Cell Biol
, vol.127
, pp. 1907-1921
-
-
Duc, C.1
Farman, N.2
Canessa, C.M.3
Bonvalet, J.P.4
Rossier, B.C.5
-
9
-
-
0034880332
-
Alterations in airway ion transport in NKCC1-deficient mice
-
Grubb BR, Pace AJ, Lee E, Koller BH, Boucher RC. Alterations in airway ion transport in NKCC1-deficient mice. Am J Physiol Cell Physiol 281: C615-C623, 2001.
-
(2001)
Am J Physiol Cell Physiol
, vol.281
-
-
Grubb, B.R.1
Pace, A.J.2
Lee, E.3
Koller, B.H.4
Boucher, R.C.5
-
11
-
-
33644836771
-
Culture of murine nasal epithelia: Model for cystic fibrosis
-
Grubb BR, Rogers TD, Diggs PC, Boucher RC, Ostrowski LE. Culture of murine nasal epithelia: model for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 290: L270-L277, 2006.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
-
-
Grubb, B.R.1
Rogers, T.D.2
Diggs, P.C.3
Boucher, R.C.4
Ostrowski, L.E.5
-
12
-
-
0026738951
-
Cell-specific expression of a Clara cell secretory protein-human growth hormone gene in the bronchiolar epithelium of transgenic mice
-
Hackett BP, Gitlin JD. Cell-specific expression of a Clara cell secretory protein-human growth hormone gene in the bronchiolar epithelium of transgenic mice. Proc Natl Acad Sci USA 89: 9079-9083, 1992.
-
(1992)
Proc Natl Acad Sci USA
, vol.89
, pp. 9079-9083
-
-
Hackett, B.P.1
Gitlin, J.D.2
-
13
-
-
80052525018
-
Measurement of the airway surface liquid volume with simple light refraction microscopy
-
Harvey PR, Tarran R, Garoff S, Myerburg MM. Measurement of the airway surface liquid volume with simple light refraction microscopy. Am J Respir Cell Mol Biol 45: 592-599, 2011.
-
(2011)
Am J Respir Cell Mol Biol
, vol.45
, pp. 592-599
-
-
Harvey, P.R.1
Tarran, R.2
Garoff, S.3
Myerburg, M.M.4
-
14
-
-
0035923676
-
Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells
-
Huang P, Lazarowski ER, Tarran R, Milgram SL, Boucher RC, Stutts MJ. Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells. Proc Natl Acad Sci USA 98: 14120-14125, 2001.
-
(2001)
Proc Natl Acad Sci USA
, 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
-
15
-
-
79959952950
-
+ conductance
-
+ conductance. Proc Natl Acad Sci USA 108: 10260-10265, 2011.
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, pp. 10260-10265
-
-
Itani, O.A.1
Chen, J.H.2
Karp, P.H.3
Ernst, S.4
Keshavjee, S.5
Parekh, K.6
Klesney-Tait, J.7
Zabner, J.8
Welsh, M.J.9
-
16
-
-
0035095067
-
Expression and localization of epithelial aquaporins in the adult human lung
-
Kreda SM, Gynn MC, Fenstermacher DA, Boucher RC, Gabriel SE. Expression and localization of epithelial aquaporins in the adult human lung. Am J Respir Cell Mol Biol 24: 224-234, 2001.
-
(2001)
Am J Respir Cell Mol Biol
, vol.24
, pp. 224-234
-
-
Kreda, S.M.1
Gynn, M.C.2
Fenstermacher, D.A.3
Boucher, R.C.4
Gabriel, S.E.5
-
17
-
-
18244377969
-
Characterization of wild-type and ΔF508 cystic fibrosis transmembrane regulator in human respiratory epithelia
-
Kreda SM, Mall M, Mengos A, Rochelle L, Yankaskas J, Riordan JR, Boucher RC. Characterization of wild-type and ΔF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. Mol Biol Cell 16: 2154-2167, 2005.
-
(2005)
Mol Biol Cell
, vol.16
, pp. 2154-2167
-
-
Kreda, S.M.1
Mall, M.2
Mengos, A.3
Rochelle, L.4
Yankaskas, J.5
Riordan, J.R.6
Boucher, R.C.7
-
19
-
-
0347361558
-
The ΔF508 mutation results in loss of CFTR function and mature protein in native human colon
-
Mall M, Kreda SM, Mengos A, Jensen TJ, Hirtz S, Seydewitz HH, Yankaskas J, Kunzelmann K, Riordan JR, Boucher RC. The ΔF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology 126: 32-41, 2004.
-
(2004)
Gastroenterology
, vol.126
, pp. 32-41
-
-
Mall, M.1
Kreda, S.M.2
Mengos, A.3
Jensen, T.J.4
Hirtz, S.5
Seydewitz, H.H.6
Yankaskas, J.7
Kunzelmann, K.8
Riordan, J.R.9
Boucher, R.C.10
-
20
-
-
77956240072
-
Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with βENaC-overexpressing mice
-
Mall MA, Button B, Johannesson B, Zhou Z, Livraghi A, Caldwell RA, Schubert SC, Schultz C, O'Neal WK, Pradervand S, Hummler E, Rossier BC, Grubb BR, Boucher RC. Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with βENaC-overexpressing mice. J Biol Chem 285: 26945-26955, 2010.
-
(2010)
J Biol Chem
, vol.285
, pp. 26945-26955
-
-
Mall, M.A.1
Button, B.2
Johannesson, B.3
Zhou, Z.4
Livraghi, A.5
Caldwell, R.A.6
Schubert, S.C.7
Schultz, C.8
O'Neal, W.K.9
Pradervand, S.10
Hummler, E.11
Rossier, B.C.12
Grubb, B.R.13
Boucher, R.C.14
-
21
-
-
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, Randell SH, Gatzy JT, Davis CW, Boucher RC. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95: 1005-1015, 1998.
-
(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
-
22
-
-
0142244084
-
Targeting expression of a transgene to the airway surface epithelium using a ciliated cell-specific promoter
-
Ostrowski LE, Hutchins JR, Zakel K, O'Neal WK. Targeting expression of a transgene to the airway surface epithelium using a ciliated cell-specific promoter. Mol Ther 8: 637-645, 2003.
-
(2003)
Mol Ther
, vol.8
, pp. 637-645
-
-
Ostrowski, L.E.1
Hutchins, J.R.2
Zakel, K.3
O'Neal, W.K.4
-
23
-
-
34848847214
-
Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice
-
Ostrowski LE, Yin W, Diggs PS, Rogers TD, O'Neal WK, Grubb BR. Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice. Gene Ther 14: 1492- 1501, 2007.
-
(2007)
Gene Ther 14: 1492-
, pp. 1501
-
-
Ostrowski, L.E.1
Yin, W.2
Diggs, P.S.3
Rogers, T.D.4
O'Neal, W.K.5
Grubb, B.R.6
-
24
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem BT, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL, Drumm ML, Iannuzzi MC, Collins FS, Tsui LC Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1066-1073, 1989.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.T.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
Drumm, M.L.11
Iannuzzi, M.C.12
Collins, F.S.13
Tsui, L.C.14
-
25
-
-
77952974496
-
Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth
-
Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starner TD, Horswill AR, Brogden KA, Prather RS, Richter SS, Shilyansky J, McCray PB Jr, Zabner J, Welsh MJ. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2: 29-31, 2010.
-
(2010)
Sci Transl Med
, vol.2
, pp. 29-31
-
-
Stoltz, D.A.1
Meyerholz, D.K.2
Pezzulo, A.A.3
Ramachandran, S.4
Rogan, M.P.5
Davis, G.J.6
Hanfland, R.A.7
Wohlford-Lenane, C.8
Dohrn, C.L.9
Bartlett, J.A.10
Nelson, G.A.11
Chang, E.H.12
Taft, P.J.13
Ludwig, P.S.14
Estin, M.15
Hornick, E.E.16
Launspach, J.L.17
Samuel, M.18
Rokhlina, T.19
Karp, P.H.20
Ostedgaard, L.S.21
Uc, A.22
Starner, T.D.23
Horswill, A.R.24
Brogden, K.A.25
Prather, R.S.26
Richter, S.S.27
Shilyansky, J.28
McCray Jr., P.B.29
Zabner, J.30
Welsh, M.J.31
more..
-
26
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier BC, Boucher RC. CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850, 1995.
-
(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
-
27
-
-
0036012529
-
Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro
-
Tarran R, Boucher RC. Thin-film measurements of airway surface liquid volume/composition and mucus transport rates in vitro. Methods Mol Med 70: 479-492, 2002.
-
(2002)
Methods Mol Med
, vol.70
, pp. 479-492
-
-
Tarran, R.1
Boucher, R.C.2
-
28
-
-
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 AM, Ribeiro CM, Lazarowski ER, Zhang L, Collins PL, Pickles RJ, Fredberg JJ, Boucher RC. Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections. J Biol Chem 280: 35751-35759, 2005.
-
(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
-
29
-
-
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 127: 591-604, 2006.
-
(2006)
J Gen Physiol
, vol.127
, pp. 591-604
-
-
Tarran, R.1
Trout, L.2
Donaldson, S.H.3
Boucher, R.C.4
-
30
-
-
0025787393
-
In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator
-
Trezise AEO, Buchwald M. In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator. Nature 353: 434-437, 1991.
-
(1991)
Nature
, vol.353
, pp. 434-437
-
-
Trezise, A.E.O.1
Buchwald, M.2
-
31
-
-
3543080347
-
Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts
-
Wang XF, Reddy MM, Quinton PM. Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts. Exp Physiol 89: 417-425, 2004.
-
(2004)
Exp Physiol
, vol.89
, pp. 417-425
-
-
Wang, X.F.1
Reddy, M.M.2
Quinton, P.M.3
-
32
-
-
22444446355
-
-
-
-. Am J Physiol Cell Physiol 289: C493-C505, 2005.
-
(2005)
Am J Physiol Cell Physiol
, vol.289
-
-
Xu, J.1
Henriksnas, J.2
Barone, S.3
Witte, D.4
Shull, G.E.5
Forte, J.G.6
Holm, L.7
Soleimani, M.8
-
33
-
-
2542500548
-
Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes
-
Yan W, Samaha FF, Ramkumar M, Kleyman TR, Rubenstein RC. Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes. J Biol Chem 279: 23183-23192, 2004.
-
(2004)
J Biol Chem
, vol.279
, pp. 23183-23192
-
-
Yan, W.1
Samaha, F.F.2
Ramkumar, M.3
Kleyman, T.R.4
Rubenstein, R.C.5
-
34
-
-
0042317111
-
Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating
-
Yang H, Shelat AA, Guy RK, Gopinath VS, Ma T, Du K, Lukacs GL, Taddei A, Folli C, Pedemonte N, Galietta LJV, Verkman AS. Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating. J Biol Chem 278: 35079-35085, 2003.
-
(2003)
J Biol Chem
, vol.278
, pp. 35079-35085
-
-
Yang, H.1
Shelat, A.A.2
Guy, R.K.3
Gopinath, V.S.4
Ma, T.5
du, K.6
Lukacs, G.L.7
Taddei, A.8
Folli, C.9
Pedemonte, N.10
Galietta, L.J.V.11
Verkman, A.S.12
-
35
-
-
0028892299
-
A mouse model for the ΔF508 allele of cystic fibrosis
-
Zeiher BG, Eichwald E, Zabner J, Smith JJ, Puga AP, McCray PB Jr, Capecchi MR, Welsh MJ, Thomas KR. A mouse model for the ΔF508 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
-
36
-
-
0025760318
-
Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene
-
Zielenski J, Rozmahel R, Bozon D, Kerem BS, Grzelczak Z, Riordan JR, Rommens J, Tsui LC. Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Genomics 10: 214-228, 1991.
-
(1991)
Genomics
, vol.10
, pp. 214-228
-
-
Zielenski, J.1
Rozmahel, R.2
Bozon, D.3
Kerem, B.S.4
Grzelczak, Z.5
Riordan, J.R.6
Rommens, J.7
Tsui, L.C.8
|