-
1
-
-
84975744535
-
Cystic fibrosis
-
Elborn, J.S., Cystic fibrosis. Lancet 388 (2016), 2519–2531.
-
(2016)
Lancet
, vol.388
, pp. 2519-2531
-
-
Elborn, J.S.1
-
2
-
-
84961933652
-
Progress in therapies for cystic fibrosis
-
De Boeck, K., Amaral, M.D., Progress in therapies for cystic fibrosis. Lancet Respir Med 4 (2016), 662–674.
-
(2016)
Lancet Respir Med
, vol.4
, pp. 662-674
-
-
De Boeck, K.1
Amaral, M.D.2
-
3
-
-
0036194724
-
Mucus clearance as a primary innate defense mechanism for mammalian airways
-
Knowles, M.R., Boucher, R.C., Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest 109 (2002), 571–577.
-
(2002)
J Clin Invest
, vol.109
, pp. 571-577
-
-
Knowles, M.R.1
Boucher, R.C.2
-
4
-
-
0020610435
-
Abnormal ion permeation through cystic fibrosis respiratory epithelium
-
Knowles, M.R., Stutts, M.J., Spock, A., Fischer, N., Gatzy, J.T., Boucher, R.C., Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221 (1983), 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
-
5
-
-
0024424270
-
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
-
Riordan, J.R., Rommens, J.M., Kerem, B., Alon, N., Rozmahel, R., Grzelczak, Z., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245 (1989), 1066–1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
-
6
-
-
0025931429
-
Nucleoside triphosphates are required to open the CFTR chloride channel
-
Anderson, M.P., Berger, H.A., Rich, D.P., Gregory, R.J., Smith, A.E., Welsh, M.J., Nucleoside triphosphates are required to open the CFTR chloride channel. Cell 67 (1991), 775–784.
-
(1991)
Cell
, vol.67
, pp. 775-784
-
-
Anderson, M.P.1
Berger, H.A.2
Rich, D.P.3
Gregory, R.J.4
Smith, A.E.5
Welsh, M.J.6
-
7
-
-
85016073190
-
Molecular structure of the human CFTR ion channel
-
Liu, F., Zhang, Z., Csanady, L., Gadsby, D.C., Chen, J., Molecular structure of the human CFTR ion channel. Cell 169 (2017), 85–95 e8.
-
(2017)
Cell
, vol.169
, pp. 85-95 e8
-
-
Liu, F.1
Zhang, Z.2
Csanady, L.3
Gadsby, D.C.4
Chen, J.5
-
8
-
-
14544300522
-
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains
-
Vergani, P., Lockless, S.W., Nairn, A.C., Gadsby, D.C., CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature 433 (2005), 876–880.
-
(2005)
Nature
, vol.433
, pp. 876-880
-
-
Vergani, P.1
Lockless, S.W.2
Nairn, A.C.3
Gadsby, D.C.4
-
9
-
-
0141513675
-
Prolonged nonhydrolytic interaction of nucleotide with CFTR's NH2-terminal nucleotide binding domain and its role in channel gating
-
Basso, C., Vergani, P., Nairn, A.C., Gadsby, D.C., Prolonged nonhydrolytic interaction of nucleotide with CFTR's NH2-terminal nucleotide binding domain and its role in channel gating. J Gen Physiol 122 (2003), 333–348.
-
(2003)
J Gen Physiol
, vol.122
, pp. 333-348
-
-
Basso, C.1
Vergani, P.2
Nairn, A.C.3
Gadsby, D.C.4
-
10
-
-
0033514315
-
Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator)
-
Ramjeesingh, M., Li, C., Garami, E., Huan, L.J., Galley, K., Wang, Y., et al. Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator). Biochemistry 38 (1999), 1463–1468.
-
(1999)
Biochemistry
, vol.38
, pp. 1463-1468
-
-
Ramjeesingh, M.1
Li, C.2
Garami, E.3
Huan, L.J.4
Galley, K.5
Wang, Y.6
-
11
-
-
84956572102
-
Timing of CFTR pore opening and structure of its transition state
-
Sorum, B., Czege, D., Csanady, L., Timing of CFTR pore opening and structure of its transition state. Cell 163 (2015), 724–733.
-
(2015)
Cell
, vol.163
, pp. 724-733
-
-
Sorum, B.1
Czege, D.2
Csanady, L.3
-
12
-
-
84880024804
-
Conformational changes in the catalytically inactive nucleotide-binding site of CFTR
-
Csanady, L., Mihalyi, C., Szollosi, A., Torocsik, B., Vergani, P., Conformational changes in the catalytically inactive nucleotide-binding site of CFTR. J Gen Physiol 142 (2013), 61–73.
-
(2013)
J Gen Physiol
, vol.142
, pp. 61-73
-
-
Csanady, L.1
Mihalyi, C.2
Szollosi, A.3
Torocsik, B.4
Vergani, P.5
-
13
-
-
78549242002
-
Optimization of the degenerated interfacial ATP binding site improves the function of disease-related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels
-
Tsai, M.F., Jih, K.Y., Shimizu, H., Li, M., Hwang, T.C., Optimization of the degenerated interfacial ATP binding site improves the function of disease-related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels. J Biol Chem 285 (2010), 37663–37671.
-
(2010)
J Biol Chem
, vol.285
, pp. 37663-37671
-
-
Tsai, M.F.1
Jih, K.Y.2
Shimizu, H.3
Li, M.4
Hwang, T.C.5
-
14
-
-
84928229130
-
Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels
-
Chaves, L.A., Gadsby, D.C., Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels. J Gen Physiol 145 (2015), 261–283.
-
(2015)
J Gen Physiol
, vol.145
, pp. 261-283
-
-
Chaves, L.A.1
Gadsby, D.C.2
-
15
-
-
79958076650
-
Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating
-
Szollosi, A., Muallem, D.R., Csanady, L., Vergani, P., Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating. J Gen Physiol 137 (2011), 549–562.
-
(2011)
J Gen Physiol
, vol.137
, pp. 549-562
-
-
Szollosi, A.1
Muallem, D.R.2
Csanady, L.3
Vergani, P.4
-
16
-
-
77951706563
-
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel
-
Tsai, M.F., Li, M., Hwang, T.C., Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel. J Gen Physiol 135 (2010), 399–414.
-
(2010)
J Gen Physiol
, vol.135
, pp. 399-414
-
-
Tsai, M.F.1
Li, M.2
Hwang, T.C.3
-
17
-
-
84979662859
-
Obligate coupling of CFTR pore opening to tight nucleotide-binding domain dimerization
-
Mihalyi, C., Torocsik, B., Csanady, L., Obligate coupling of CFTR pore opening to tight nucleotide-binding domain dimerization. Elife, 5, 2016.
-
(2016)
Elife
, vol.5
-
-
Mihalyi, C.1
Torocsik, B.2
Csanady, L.3
-
18
-
-
33749039521
-
The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics
-
Zhou, Z., Wang, X., Liu, H.Y., Zou, X., Li, M., Hwang, T.C., The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics. J Gen Physiol 128 (2006), 413–422.
-
(2006)
J Gen Physiol
, vol.128
, pp. 413-422
-
-
Zhou, Z.1
Wang, X.2
Liu, H.Y.3
Zou, X.4
Li, M.5
Hwang, T.C.6
-
19
-
-
0036521974
-
Mutation of Walker-A lysine 464 in cystic fibrosis transmembrane conductance regulator reveals functional interaction between its nucleotide-binding domains
-
Powe, A.C. Jr., Al-Nakkash, L., Li, M., Hwang, T.C., Mutation of Walker-A lysine 464 in cystic fibrosis transmembrane conductance regulator reveals functional interaction between its nucleotide-binding domains. J Physiol 539 (2002), 333–346.
-
(2002)
J Physiol
, vol.539
, pp. 333-346
-
-
Powe, A.C.1
Al-Nakkash, L.2
Li, M.3
Hwang, T.C.4
-
20
-
-
0037246832
-
On the mechanism of MgATP-dependent gating of CFTR Cl- channels
-
Vergani, P., Nairn, A.C., Gadsby, D.C., On the mechanism of MgATP-dependent gating of CFTR Cl- channels. J Gen Physiol 121 (2003), 17–36.
-
(2003)
J Gen Physiol
, vol.121
, pp. 17-36
-
-
Vergani, P.1
Nairn, A.C.2
Gadsby, D.C.3
-
21
-
-
0029117303
-
Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis
-
Gunderson, K.L., Kopito, R.R., Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis. Cell 82 (1995), 231–239.
-
(1995)
Cell
, vol.82
, pp. 231-239
-
-
Gunderson, K.L.1
Kopito, R.R.2
-
22
-
-
33947725805
-
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects
-
Bompadre, S.G., Sohma, Y., Li, M., Hwang, T.C., G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol 129 (2007), 285–298.
-
(2007)
J Gen Physiol
, vol.129
, pp. 285-298
-
-
Bompadre, S.G.1
Sohma, Y.2
Li, M.3
Hwang, T.C.4
-
23
-
-
85007565989
-
New horizons for cystic fibrosis treatment
-
Fajac, I., De Boeck, K., New horizons for cystic fibrosis treatment. Pharmacol Ther 170 (2017), 205–211.
-
(2017)
Pharmacol Ther
, vol.170
, pp. 205-211
-
-
Fajac, I.1
De Boeck, K.2
-
24
-
-
77950810893
-
Regulation of the epithelial Na + channel and airway surface liquid volume by serine proteases
-
Gaillard, E.A., Kota, P., Gentzsch, M., Dokholyan, N.V., Stutts, M.J., Tarran, R., Regulation of the epithelial Na + channel and airway surface liquid volume by serine proteases. Pflugers Arch 460 (2010), 1–17.
-
(2010)
Pflugers Arch
, vol.460
, pp. 1-17
-
-
Gaillard, E.A.1
Kota, P.2
Gentzsch, M.3
Dokholyan, N.V.4
Stutts, M.J.5
Tarran, R.6
-
25
-
-
0027958441
-
Amiloride-sensitive epithelial Na + channel is made of three homologous subunits
-
Canessa, C.M., Schild, L., Buell, G., Thorens, B., Gautschi, I., Horisberger, J.D., et al. Amiloride-sensitive epithelial Na + channel is made of three homologous subunits. Nature 367 (1994), 463–467.
-
(1994)
Nature
, vol.367
, pp. 463-467
-
-
Canessa, C.M.1
Schild, L.2
Buell, G.3
Thorens, B.4
Gautschi, I.5
Horisberger, J.D.6
-
26
-
-
67650488822
-
SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage
-
Garcia-Caballero, A., Rasmussen, J.E., Gaillard, E., Watson, M.J., Olsen, J.C., Donaldson, S.H., et al. SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage. Proc Natl Acad Sci U S A 106 (2009), 11412–11417.
-
(2009)
Proc Natl Acad Sci U S A
, vol.106
, pp. 11412-11417
-
-
Garcia-Caballero, A.1
Rasmussen, J.E.2
Gaillard, E.3
Watson, M.J.4
Olsen, J.C.5
Donaldson, S.H.6
-
27
-
-
78649743537
-
Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking
-
Myerburg, M.M., Harvey, P.R., Heidrich, E.M., Pilewski, J.M., Butterworth, M.B., Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking. Am J Respir Cell Mol Biol 43 (2010), 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
-
28
-
-
77957767326
-
The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na + channel
-
Gentzsch, M., Dang, H., Dang, Y., Garcia-Caballero, A., Suchindran, H., Boucher, R.C., et al. The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na + channel. J Biol Chem 285 (2010), 32227–32232.
-
(2010)
J Biol Chem
, vol.285
, pp. 32227-32232
-
-
Gentzsch, M.1
Dang, H.2
Dang, Y.3
Garcia-Caballero, A.4
Suchindran, H.5
Boucher, R.C.6
-
29
-
-
58149505557
-
Plasmin activates epithelial Na + channels by cleaving the gamma subunit
-
Passero, C.J., Mueller, G.M., Rondon-Berrios, H., Tofovic, S.P., Hughey, R.P., Kleyman, T.R., Plasmin activates epithelial Na + channels by cleaving the gamma subunit. J Biol Chem 283 (2008), 36586–36591.
-
(2008)
J Biol Chem
, vol.283
, pp. 36586-36591
-
-
Passero, C.J.1
Mueller, G.M.2
Rondon-Berrios, H.3
Tofovic, S.P.4
Hughey, R.P.5
Kleyman, T.R.6
-
30
-
-
2442427527
-
Epithelial sodium channels are activated by furin-dependent proteolysis
-
Hughey, R.P., Bruns, J.B., Kinlough, C.L., Harkleroad, K.L., Tong, Q., Carattino, M.D., et al. Epithelial sodium channels are activated by furin-dependent proteolysis. J Biol Chem 279 (2004), 18111–18114.
-
(2004)
J Biol Chem
, vol.279
, 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
-
31
-
-
59449104675
-
ENaC proteolytic regulation by channel-activating protease 2
-
Garcia-Caballero, A., Dang, Y., He, H., Stutts, M.J., ENaC proteolytic regulation by channel-activating protease 2. J Gen Physiol 132 (2008), 521–535.
-
(2008)
J Gen Physiol
, vol.132
, pp. 521-535
-
-
Garcia-Caballero, A.1
Dang, Y.2
He, H.3
Stutts, M.J.4
-
32
-
-
0034815863
-
Na + transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437
-
Bridges, R.J., Newton, B.B., Pilewski, J.M., Devor, D.C., Poll, C.T., Hall, R.L., Na + transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437. Am J Physiol Lung Cell Mol Physiol 281 (2001), L16–23.
-
(2001)
Am J Physiol Lung Cell Mol Physiol
, vol.281
, pp. L16-23
-
-
Bridges, R.J.1
Newton, B.B.2
Pilewski, J.M.3
Devor, D.C.4
Poll, C.T.5
Hall, R.L.6
-
33
-
-
65649148597
-
Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease
-
Coote, K., Atherton-Watson, H.C., Sugar, R., Young, A., MacKenzie-Beevor, A., Gosling, M., et al. Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease. J Pharmacol Exp Ther 329 (2009), 764–774.
-
(2009)
J Pharmacol Exp Ther
, vol.329
, pp. 764-774
-
-
Coote, K.1
Atherton-Watson, H.C.2
Sugar, R.3
Young, A.4
MacKenzie-Beevor, A.5
Gosling, M.6
-
34
-
-
84988921360
-
Inhibition of protease-epithelial Sodium Channel signaling improves mucociliary function in cystic fibrosis airways
-
Reihill, J.A., Walker, B., Hamilton, R.A., Ferguson, T.E., Elborn, J.S., Stutts, M.J., et al. Inhibition of protease-epithelial Sodium Channel signaling improves mucociliary function in cystic fibrosis airways. Am J Respir Crit Care Med 194 (2016), 701–710.
-
(2016)
Am J Respir Crit Care Med
, vol.194
, pp. 701-710
-
-
Reihill, J.A.1
Walker, B.2
Hamilton, R.A.3
Ferguson, T.E.4
Elborn, J.S.5
Stutts, M.J.6
-
35
-
-
85054859347
-
Epithelial Na + channel inhibitors for the treatment of cystic fibrosis
-
Smith, N.J., Solovay, C.F., Epithelial Na + channel inhibitors for the treatment of cystic fibrosis. Pharm Pat Anal 6 (2017), 179–188.
-
(2017)
Pharm Pat Anal
, vol.6
, pp. 179-188
-
-
Smith, N.J.1
Solovay, C.F.2
-
36
-
-
85029682826
-
SPX-101 is a novel ENaC-targeted therapeutic for cystic fibrosis that restores mucus transport
-
Scott, D.W., Walker, M.P., Sesma, J., Wu, B., Stuhlmiller, T.J., Sabater, J.R., et al. SPX-101 is a novel ENaC-targeted therapeutic for cystic fibrosis that restores mucus transport. Am J Respir Crit Care Med, 2017.
-
(2017)
Am J Respir Crit Care Med
-
-
Scott, D.W.1
Walker, M.P.2
Sesma, J.3
Wu, B.4
Stuhlmiller, T.J.5
Sabater, J.R.6
-
37
-
-
84878888912
-
Pharmacological characterization of a novel ENaCalpha siRNA (GSK2225745) with potential for the treatment of cystic fibrosis
-
Clark, K.L., Hughes, S.A., Bulsara, P., Coates, J., Moores, K., Parry, J., et al. Pharmacological characterization of a novel ENaCalpha siRNA (GSK2225745) with potential for the treatment of cystic fibrosis. Mol Ther Nucleic Acids, e65, 2013, 2.
-
(2013)
Mol Ther Nucleic Acids
, vol.e65
, pp. 2
-
-
Clark, K.L.1
Hughes, S.A.2
Bulsara, P.3
Coates, J.4
Moores, K.5
Parry, J.6
-
38
-
-
85018789132
-
Delivery of ENaC siRNA to epithelial cells mediated by a targeted nanocomplex: a therapeutic strategy for cystic fibrosis
-
Manunta, M.D., Tagalakis, A.D., Attwood, M., Aldossary, A.M., Barnes, J.L., Munye, M.M., et al. Delivery of ENaC siRNA to epithelial cells mediated by a targeted nanocomplex: a therapeutic strategy for cystic fibrosis. Sci Rep, 7, 2017, 700.
-
(2017)
Sci Rep
, vol.7
, pp. 700
-
-
Manunta, M.D.1
Tagalakis, A.D.2
Attwood, M.3
Aldossary, A.M.4
Barnes, J.L.5
Munye, M.M.6
-
39
-
-
54949112835
-
TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity
-
Caputo, A., Caci, E., Ferrera, L., Pedemonte, N., Barsanti, C., Sondo, E., et al. TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity. Science 322 (2008), 590–594.
-
(2008)
Science
, vol.322
, pp. 590-594
-
-
Caputo, A.1
Caci, E.2
Ferrera, L.3
Pedemonte, N.4
Barsanti, C.5
Sondo, E.6
-
40
-
-
51549120559
-
Expression cloning of TMEM16A as a calcium-activated chloride channel subunit
-
Schroeder, B.C., Cheng, T., Jan, Y.N., Jan, L.Y., Expression cloning of TMEM16A as a calcium-activated chloride channel subunit. Cell 134 (2008), 1019–1029.
-
(2008)
Cell
, vol.134
, pp. 1019-1029
-
-
Schroeder, B.C.1
Cheng, T.2
Jan, Y.N.3
Jan, L.Y.4
-
41
-
-
55249091085
-
TMEM16A confers receptor-activated calcium-dependent chloride conductance
-
Yang, Y.D., Cho, H., Koo, J.Y., Tak, M.H., Cho, Y., Shim, W.S., et al. TMEM16A confers receptor-activated calcium-dependent chloride conductance. Nature 455 (2008), 1210–1215.
-
(2008)
Nature
, vol.455
, pp. 1210-1215
-
-
Yang, Y.D.1
Cho, H.2
Koo, J.Y.3
Tak, M.H.4
Cho, Y.5
Shim, W.S.6
-
42
-
-
84871959313
-
Dynamic modulation of ANO1/TMEM16A HCO3(−) permeability by Ca2 +/calmodulin
-
Jung, J., Nam, J.H., Park, H.W., Oh, U., Yoon, J.H., Lee, M.G., Dynamic modulation of ANO1/TMEM16A HCO3(−) permeability by Ca2 +/calmodulin. Proc Natl Acad Sci U S A 110 (2013), 360–365.
-
(2013)
Proc Natl Acad Sci U S A
, vol.110
, pp. 360-365
-
-
Jung, J.1
Nam, J.H.2
Park, H.W.3
Oh, U.4
Yoon, J.H.5
Lee, M.G.6
-
43
-
-
84870370950
-
Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia
-
Scudieri, P., Caci, E., Bruno, S., Ferrera, L., Schiavon, M., Sondo, E., et al. Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia. J Physiol 590 (2012), 6141–6155.
-
(2012)
J Physiol
, vol.590
, pp. 6141-6155
-
-
Scudieri, P.1
Caci, E.2
Bruno, S.3
Ferrera, L.4
Schiavon, M.5
Sondo, E.6
-
44
-
-
84867067619
-
Calcium-activated chloride channel TMEM16A modulates mucin secretion and airway smooth muscle contraction
-
Huang, F., Zhang, H., Wu, M., Yang, H., Kudo, M., Peters, C.J., et al. Calcium-activated chloride channel TMEM16A modulates mucin secretion and airway smooth muscle contraction. Proc Natl Acad Sci U S A 109 (2012), 16354–16359.
-
(2012)
Proc Natl Acad Sci U S A
, vol.109
, pp. 16354-16359
-
-
Huang, F.1
Zhang, H.2
Wu, M.3
Yang, H.4
Kudo, M.5
Peters, C.J.6
-
45
-
-
84930818625
-
Characterization of the effects of Cl(−) channel modulators on TMEM16A and bestrophin-1 Ca(2)(+) activated Cl(−) channels
-
Liu, Y., Zhang, H., Huang, D., Qi, J., Xu, J., Gao, H., et al. Characterization of the effects of Cl(−) channel modulators on TMEM16A and bestrophin-1 Ca(2)(+) activated Cl(−) channels. Pflugers Arch 467 (2015), 1417–1430.
-
(2015)
Pflugers Arch
, vol.467
, pp. 1417-1430
-
-
Liu, Y.1
Zhang, H.2
Huang, D.3
Qi, J.4
Xu, J.5
Gao, H.6
-
46
-
-
84971373876
-
Ani9, a novel potent small-molecule ANO1 inhibitor with negligible effect on ANO2
-
Seo, Y., Lee, H.K., Park, J., Jeon, D.K., Jo, S., Jo, M., et al. Ani9, a novel potent small-molecule ANO1 inhibitor with negligible effect on ANO2. PLoS One, 11, 2016, e0155771.
-
(2016)
PLoS One
, vol.11
-
-
Seo, Y.1
Lee, H.K.2
Park, J.3
Jeon, D.K.4
Jo, S.5
Jo, M.6
-
47
-
-
34247165457
-
Thiocyanate transport in resting and IL-4-stimulated human bronchial epithelial cells: role of pendrin and anion channels
-
Pedemonte, N., Caci, E., Sondo, E., Caputo, A., Rhoden, K., Pfeffer, U., et al. Thiocyanate transport in resting and IL-4-stimulated human bronchial epithelial cells: role of pendrin and anion channels. J Immunol 178 (2007), 5144–5153.
-
(2007)
J Immunol
, vol.178
, pp. 5144-5153
-
-
Pedemonte, N.1
Caci, E.2
Sondo, E.3
Caputo, A.4
Rhoden, K.5
Pfeffer, U.6
-
48
-
-
81755189072
-
Novel role for pendrin in orchestrating bicarbonate secretion in cystic fibrosis transmembrane conductance regulator (CFTR)-expressing airway serous cells
-
Garnett, J.P., Hickman, E., Burrows, R., Hegyi, P., Tiszlavicz, L., Cuthbert, A.W., et al. Novel role for pendrin in orchestrating bicarbonate secretion in cystic fibrosis transmembrane conductance regulator (CFTR)-expressing airway serous cells. J Biol Chem 286 (2011), 41069–41082.
-
(2011)
J Biol Chem
, vol.286
, pp. 41069-41082
-
-
Garnett, J.P.1
Hickman, E.2
Burrows, R.3
Hegyi, P.4
Tiszlavicz, L.5
Cuthbert, A.W.6
-
49
-
-
84973137939
-
Inhibitors of pendrin anion exchange identified in a small molecule screen increase airway surface liquid volume in cystic fibrosis
-
Haggie, P.M., Phuan, P.W., Tan, J.A., Zlock, L., Finkbeiner, W.E., Verkman, A.S., Inhibitors of pendrin anion exchange identified in a small molecule screen increase airway surface liquid volume in cystic fibrosis. FASEB J 30 (2016), 2187–2197.
-
(2016)
FASEB J
, vol.30
, pp. 2187-2197
-
-
Haggie, P.M.1
Phuan, P.W.2
Tan, J.A.3
Zlock, L.4
Finkbeiner, W.E.5
Verkman, A.S.6
-
50
-
-
84940962718
-
Targeting ion channels in cystic fibrosis
-
Mall, M.A., Galietta, L.J., Targeting ion channels in cystic fibrosis. J Cyst Fibros 14 (2015), 561–570.
-
(2015)
J Cyst Fibros
, vol.14
, pp. 561-570
-
-
Mall, M.A.1
Galietta, L.J.2
-
51
-
-
0033864526
-
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia
-
Devor, D.C., Bridges, R.J., Pilewski, J.M., Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am J Physiol Cell Physiol 279 (2000), C461–79.
-
(2000)
Am J Physiol Cell Physiol
, vol.279
, pp. C461-79
-
-
Devor, D.C.1
Bridges, R.J.2
Pilewski, J.M.3
-
52
-
-
0346103648
-
Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
-
Coakley, R.D., Grubb, B.R., Paradiso, A.M., Gatzy, J.T., Johnson, L.G., Kreda, S.M., et al. Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc Natl Acad Sci U S A 100 (2003), 16083–16088.
-
(2003)
Proc Natl Acad Sci U S A
, vol.100
, pp. 16083-16088
-
-
Coakley, R.D.1
Grubb, B.R.2
Paradiso, A.M.3
Gatzy, J.T.4
Johnson, L.G.5
Kreda, S.M.6
-
53
-
-
84956707544
-
Airway acidification initiates host defense abnormalities in cystic fibrosis mice
-
Shah, V.S., Meyerholz, D.K., Tang, X.X., Reznikov, L., Abou Alaiwa, M., Ernst, S.E., et al. Airway acidification initiates host defense abnormalities in cystic fibrosis mice. Science 351 (2016), 503–507.
-
(2016)
Science
, vol.351
, pp. 503-507
-
-
Shah, V.S.1
Meyerholz, D.K.2
Tang, X.X.3
Reznikov, L.4
Abou Alaiwa, M.5
Ernst, S.E.6
|