-
1
-
-
79953183131
-
Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy
-
Kim Chiaw, P., Eckford, P. D., Bear, C. E. Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy. Essays Biochem. 50, (1), 233-248 (2011).
-
(2011)
Essays Biochem
, vol.50
, Issue.1
, pp. 233-248
-
-
Kim Chiaw, P.1
Eckford, P.D.2
Bear, C.E.3
-
2
-
-
0026532895
-
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Bear, C. E., et al. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell. 68, (4), 809-818 (1992).
-
(1992)
Cell
, vol.68
, Issue.4
, pp. 809-818
-
-
Bear, C.E.1
-
3
-
-
80052338842
-
Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants
-
Cai, Z., Sohma, Y., Bompadre, S. G., Sheppard, D. N., Hwang, T. C. Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants. Methods Mol Biol. 741, 419-441 (2011).
-
(2011)
Methods Mol Biol
, vol.741
, pp. 419-441
-
-
Cai, Z.1
Sohma, Y.2
Bompadre, S.G.3
Sheppard, D.N.4
Hwang, T.C.5
-
5
-
-
0024423668
-
Identification of the cystic fibrosis gene: Genetic analysis
-
Kerem, B., et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 245, (4922), 1073-1080 (1989).
-
(1989)
Science
, vol.245
, Issue.4922
, pp. 1073-1080
-
-
Kerem, B.1
-
6
-
-
0027209522
-
Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR
-
Yang, Y., et al. Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. Hum Mol Genet. 2, (8), 1253-1261 (1993).
-
(1993)
Hum Mol Genet
, vol.2
, Issue.8
, pp. 1253-1261
-
-
Yang, Y.1
-
7
-
-
84862909346
-
Requirements for efficient correction of DeltaF508 CFTR revealed by analyses of evolved sequences
-
Mendoza, J. L., et al. Requirements for efficient correction of DeltaF508 CFTR revealed by analyses of evolved sequences. Cell. 148, (1-2), 164-274 (2012).
-
(2012)
Cell
, vol.148
, Issue.1-2
, pp. 164-274
-
-
Mendoza, J.L.1
-
8
-
-
84862908028
-
Correction of both NBD1 energetics and domain interface is required to restore DeltaF508 CFTR folding and function
-
Rabeh, W. M., et al. Correction of both NBD1 energetics and domain interface is required to restore DeltaF508 CFTR folding and function. Cell. 148, (1-2), 150-163 (2012).
-
(2012)
Cell
, vol.148
, Issue.1-2
, pp. 150-163
-
-
Rabeh, W.M.1
-
9
-
-
84860271643
-
Allosteric modulation balances thermodynamic stability and restores function of DeltaF508. CFTR
-
Aleksandrov, A. A., et al. Allosteric modulation balances thermodynamic stability and restores function of DeltaF508. CFTR. J Mol Biol. 419, (1-2), 41-60 (2012).
-
(2012)
J Mol Biol
, vol.419
, Issue.1-2
, pp. 41-60
-
-
Aleksandrov, A.A.1
-
10
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey, B. W., et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 365, (18), 1663-1672 (2011).
-
(2011)
N Engl J Med
, vol.365
, Issue.18
, pp. 1663-1672
-
-
Ramsey, B.W.1
-
11
-
-
0029063514
-
Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells
-
Riordan, C. R., et al. Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells. J. Biol. Chem. 270, (28), 17033-17043 (1995).
-
(1995)
J. Biol. Chem
, vol.270
, Issue.28
, pp. 17033-17043
-
-
Riordan, C.R.1
-
12
-
-
84863767664
-
Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae
-
Ryan, L., Rimington, T., Cant, N., Ford, R. C. Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae. JoVE. (61), (2012).
-
(2012)
Jove
, Issue.61
-
-
Ryan, L.1
Rimington, T.2
Cant, N.3
Ford, R.C.4
-
13
-
-
0027049358
-
Partial purification of the cystic fibrosis transmembrane conductance regulator
-
Ostedgaard, L. S., Welsh, M. J. Partial purification of the cystic fibrosis transmembrane conductance regulator. J Biol Chem. 267, (36), 26142-26149 (1992).
-
(1992)
J Biol Chem
, vol.267
, Issue.36
, pp. 26142-26149
-
-
Ostedgaard, L.S.1
Welsh, M.J.2
-
14
-
-
0027581172
-
One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system
-
Peng, S., et al. One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system. Protein Expr Purif. 4, (2), 95-100 (1993).
-
(1993)
Protein Expr Purif
, vol.4
, Issue.2
, pp. 95-100
-
-
Peng, S.1
-
15
-
-
0030669141
-
A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Ramjeesingh, M., et al. A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR). Biochem J. 327, (1), 17-21 (1997).
-
(1997)
Biochem J
, vol.327
, Issue.1
, pp. 17-21
-
-
Ramjeesingh, M.1
-
16
-
-
4544378176
-
Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Rosenberg, M. F., Kamis, A. B., Aleksandrov, L. A., Ford, R. C., Riordan, J. R. Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR). J Biol Chem. 279, (37), 39051-39057 (2004).
-
(2004)
J Biol Chem
, vol.279
, Issue.37
, pp. 39051-39057
-
-
Rosenberg, M.F.1
Kamis, A.B.2
Aleksandrov, L.A.3
Ford, R.C.4
Riordan, J.R.5
-
17
-
-
0031788294
-
Purification and reconstitution of epithelial chloride channel cystic fibrosis transmembrane conductance regulator
-
Ramjeesingh, M., et al. Purification and reconstitution of epithelial chloride channel cystic fibrosis transmembrane conductance regulator. Methods Enzymol. 294, 227-246 (1999).
-
(1999)
Methods Enzymol
, vol.294
, pp. 227-246
-
-
Ramjeesingh, M.1
-
18
-
-
0029187824
-
Purification of recombinant protein derived from the baculovirus expression system using glutathione affinity agarose
-
Sorscher, E. J., Sommerfelt, M. A. Purification of recombinant protein derived from the baculovirus expression system using glutathione affinity agarose. Methods Mol Biol. 3, 337-348 (1995).
-
(1995)
Methods Mol Biol
, vol.3
, pp. 337-348
-
-
Sorscher, E.J.1
Sommerfelt, M.A.2
-
19
-
-
84868243529
-
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner
-
Eckford, P. D., Li, C., Ramjeesingh, M., Bear, C. E. Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner. J Biol Chem. 287, (44), 36639-36649 (2012).
-
(2012)
J Biol Chem
, vol.287
, Issue.44
, pp. 36639-36649
-
-
Eckford, P.D.1
Li, C.2
Ramjeesingh, M.3
Bear, C.E.4
-
20
-
-
84055217548
-
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator
-
Alkhouri, B., et al. Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator. J Med Chem. 54, (24), 8693-8701 (2011).
-
(2011)
J Med Chem
, vol.54
, Issue.24
, pp. 8693-8701
-
-
Alkhouri, B.1
-
21
-
-
84901398808
-
VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface
-
Eckford, P. D., et al. VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface. Chem Biol. 21, (5), 666-678 (2014).
-
(2014)
Chem Biol
, vol.21
, Issue.5
, pp. 666-678
-
-
Eckford, P.D.1
-
22
-
-
77956237958
-
A chemical corrector modifies the channel function of F508del- CFTR
-
Kim Chiaw, P., Wellhauser, L., Huan, L. J., Ramjeesingh, M., Bear, C. E. A chemical corrector modifies the channel function of F508del- CFTR. Mol.Pharmacol. 78, (3), 411-418 (2010).
-
(2010)
Mol.Pharmacol
, vol.78
, Issue.3
, pp. 411-418
-
-
Kim Chiaw, P.1
Wellhauser, L.2
Huan, L.J.3
Ramjeesingh, M.4
Bear, C.E.5
-
23
-
-
59349091561
-
Chloride channels as drug targets
-
Verkman, A. S., Galietta, L. J. Chloride channels as drug targets. Nat Rev Drug Discov. 8, (2), 153-171 (2009).
-
(2009)
Nat Rev Drug Discov
, vol.8
, Issue.2
, pp. 153-171
-
-
Verkman, A.S.1
Galietta, L.J.2
-
24
-
-
59849091513
-
Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease
-
Pasyk, S., Li, C., Ramjeesingh, M., Bear, C. E. Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. Biochem J. 418, (1), 185-190 (2009).
-
(2009)
Biochem J
, vol.418
, Issue.1
, pp. 185-190
-
-
Pasyk, S.1
Li, C.2
Ramjeesingh, M.3
Bear, C.E.4
-
25
-
-
19944364842
-
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods
-
Norez, C., et al. Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. J Cyst Fibros. 3, (2), 119-121 (2004).
-
(2004)
J Cyst Fibros
, vol.3
, Issue.2
, pp. 119-121
-
-
Norez, C.1
-
26
-
-
80051966444
-
Structural basis for alginate secretion across the bacterial outer membrane
-
Whitney, J. C., et al. Structural basis for alginate secretion across the bacterial outer membrane. Proc Natl Acad Sci U S A. 108, (32), 13083-13088 (2011).
-
(2011)
Proc Natl Acad Sci U S A
, vol.108
, Issue.32
, pp. 13083-13088
-
-
Whitney, J.C.1
-
27
-
-
84887168855
-
Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane
-
Islam, S. T., et al. Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane. mBio. 4, (5), e00678-e00613 (2013).
-
(2013)
Mbio
, vol.4
, Issue.5
-
-
Islam, S.T.1
-
28
-
-
84941198099
-
Ch. 16. Reliable Lab Solutions: Essential Ion Channel MethodsReliable Lab Solutions
-
Academic Press
-
Ramjeesingh, M., et al. Ch. 16. Reliable Lab Solutions: Essential Ion Channel MethodsReliable Lab Solutions. Conn, P. M. 337-357 Academic Press (2010).
-
(2010)
Conn, P. M
, pp. 337-357
-
-
Ramjeesingh, M.1
-
29
-
-
66849129301
-
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability
-
Wellhauser, L., et al. A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Mol Pharmacol. 75, (6), 1430-1438 (2009).
-
(2009)
Mol Pharmacol
, vol.75
, Issue.6
, pp. 1430-1438
-
-
Wellhauser, L.1
-
30
-
-
63449116167
-
Functional reconstitution of purified human Hv1 H+ channels
-
Lee, S. Y., Letts, J. A., MacKinnon, R. Functional reconstitution of purified human Hv1 H+ channels. J Mol Biol. 387, (5), 1055-1060 (2009).
-
(2009)
J Mol Biol
, vol.387
, Issue.5
, pp. 1055-1060
-
-
Lee, S.Y.1
Letts, J.A.2
Mackinnon, R.3
-
31
-
-
0027311276
-
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites
-
Chang, X. B., et al. Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J. Biol. Chem. 268, (15), 11304-11311 (1993).
-
(1993)
J. Biol. Chem
, vol.268
, Issue.15
, pp. 11304-11311
-
-
Chang, X.B.1
-
32
-
-
0032617252
-
Regulation of CFTR Cl- ion channels by phosphorylation and dephosphorylation
-
Gadsby, D. C., Nairn, A. C. Regulation of CFTR Cl- ion channels by phosphorylation and dephosphorylation. Adv.Second Messenger Phosphoprotein Res. 33, 79-106 (1999).
-
(1999)
Adv.Second Messenger Phosphoprotein Res
, vol.33
, pp. 79-106
-
-
Gadsby, D.C.1
Nairn, A.C.2
-
33
-
-
0029904733
-
ATPase activity of the cystic fibrosis transmembrane conductance regulator
-
Li, C., et al. ATPase activity of the cystic fibrosis transmembrane conductance regulator. J Biol Chem. 271, (45), 28463-28468 (1996).
-
(1996)
J Biol Chem
, vol.271
, Issue.45
, pp. 28463-28468
-
-
Li, C.1
-
34
-
-
0036896008
-
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion
-
Ma, T., et al. Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion. J Clin Invest. 110, (11), 1651-1658 (2002).
-
(2002)
J Clin Invest
, vol.110
, Issue.11
, pp. 1651-1658
-
-
Ma, T.1
-
35
-
-
78650045527
-
On the mechanism of CFTR inhibition by a thiazolidinone derivative
-
Kopeikin, Z., Sohma, Y., Li, M., Hwang, T. C. On the mechanism of CFTR inhibition by a thiazolidinone derivative. J Gen Physiol. 136, (6), 659-671 (2010).
-
(2010)
J Gen Physiol
, vol.136
, Issue.6
, pp. 659-671
-
-
Kopeikin, Z.1
Sohma, Y.2
Li, M.3
Hwang, T.C.4
-
36
-
-
3543080347
-
Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts
-
Wang, X. F., Reddy, M. M., Quinton, P. M. Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts. Exp Physiol. 89, (4), 417-425 (2004).
-
(2004)
Exp Physiol
, vol.89
, Issue.4
, pp. 417-425
-
-
Wang, X.F.1
Reddy, M.M.2
Quinton, P.M.3
|