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Intracellular CFTR localization and function
1999
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Physiol. Rev.
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CFTR Cl- channel and CFTR-associated ATP channel: distinct pores regulated by common gates
1998
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Structure and function of the CFTR chloride channel
1999
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CFTR is a conductance regulator as well as a chloride channel
1999
Schwiebert, E.M.; Benos, D.J.; Egan, M.E.; Stutts, M.J.; Guggino, W.B.
Physiol. Rev.
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Dual effects of ADP and adenylylimidodiphosphate on CFTR channel kinetics show binding to two different nucleotide binding sites
1999
Weinreich, F.; Riordan, J.R.; Nagel, G.
J. Gen. Physiol.
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Genetic disorders of membrane transport. II. Regulation of CFTR by small molecules including HCO3
1998
Illek, B.; Fischer, H.; Machen, T.E.
Am. J. Physiol.
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Influence of phosphorylation by protein kinase A on CFTR at the cell surface and endoplasmic reticulum
1999
Seibert, F.S.; Chang, X.B.; Aleksandrov, A.A.; Clarke, D.M.; Hanrahan, J.W.; Riordan, J.R.
Biochim. Biophys. Acta
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Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR
1997
Bear, C.E.; Li, C.; Galley, K.; Wang, Y.; Garami, E.; Ramjeesingh, M.
J. Bioenerg. Biomembr.
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Syntaxin 1A is expressed in airway epithelial cells, where it modulates CFTR Cl(-) currents
2000
Naren, A.P.; Di, A.; Cormet-Boyaka, E.; Boyaka, P.N.; McGhee, J.R.; Zhou, W.; Akagawa, K.; Fujiwara, T.; Thome, U.; Engelhardt, J.F.; Nelson, D.J.; Kirk, K.L.
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Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions
1998
Naren, A.P.; Quick, M.W.; Collawn, J.F.; Nelson, D.J.; Kirk, K.L.
Proc. Natl. Acad. Sci. USA
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Membrane insertion, processing, and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes
1996
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Mol. Membr. Biol.
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Membrane topology distinguishes a subfamily of the ATP-binding cassette (ABC) transporters
1997
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Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR
1996
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Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding
1992
Quinton, P.M.; Reddy, M.M.
Nature
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Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP binding cassette transporter
1996
Van Kuijck, M.A.; van Aubel, R.A.M.H.; Busch, A.E.; Lang, F.; Russell, F.G.M.; Bindels, R.J.M.; van Os, C.H.; Deen, P.M.T.
Proc. Natl. Acad. Sci. USA
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Regulated trafficking of the CFTR chloride channel
2000
Kleizen, B.; Braakman, I.; de Jonge, H.R.
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Cystic fibrosis transmembrane conductance regulator: the NBF1+R (nucleotide-binding fold 1 and regulatory domain) segment acting alone catalyses a Co2+/Mn2+/Mg2+-ATPase activity markedly inhibited by both Cd2+ and the transition-state analogue orthovanadate
2003
Annereau, J.P.; Ko, Y.H.; Pedersen, P.L.
Biochem. J.
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Protein kinase A regulates ATP hydrolysis and dimerization by a CFTR (cystic fibrosis transmembrane conductance regulator) domain
2004
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A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator
2001
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Biochemistry
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Characterization of the adenosinetriphosphatase and transport activities of purified cystic fibrosis transmembrane conductance regulator
2004
Ketchum, C.J.; Rajendrakumar, G.V.; Maloney, P.C.
Biochemistry
43
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Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
2004
Lewis, H.A.; Buchanan, S.G.; Burley, S.K.; Conners, K.; Dickey, M.; Dorwart, M.; Fowler, R.; Gao, X.; Guggino, W.B.; Hendrickson, W.A.; Hunt, J.F.; Kearins, M.C.; Lorimer, D.; Maloney, P.C.; Post, K.W.; Rajashankar, K.R.; Rutter, M.E.; Sauder, J.M.; Shriver, S.; Thibodeau, P.H.; Thomas, P.J.; Zhang, M.; Zhao, X.; Emtage, S.
EMBO J.
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Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its ATPase activity
2001
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J. Biol. Chem.
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On the mechanism of MgATP-dependent gating of CFTR Cl- channels
2003
Vergani, P.; Nairn, A.C.; Gadsby, D.C.
J. Gen. Physiol.
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Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR)
2004
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J. Biol. Chem.
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Functional roles of nonconserved structural segments in CFTRs NH2-terminal nucleotide binding domain
2005
Csanady, L.; Chan, K.W.; Nairn, A.C.; Gadsby, D.C.
J. Gen. Physiol.
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Revisiting cystic fibrosis transmembrane conductance regulator structure and function
2004
Hanrahan, J.W.; Wioland, M.A.
Proc. Am. Thorac. Soc.
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Conformational changes in a pore-lining helix coupled to cystic fibrosis transmembrane conductance regulator channel gating
2008
Beck, E.J.; Yang, Y.; Yaemsiri, S.; Raghuram, V.
J. Biol. Chem.
283
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Mg2+-dependent ATP occlusion at the first nucleotide-binding domain (NBD1) of CFTR does not require the second (NBD2)
2008
Aleksandrov, L.; Aleksandrov, A.; Riordan, J.R.
Biochem. J.
416
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Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator
2009
Bijvelds, M.J.; Bot, A.G.; Escher, J.C.; de Jonge, H.R.
Gastroenterology
137
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Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating
2008
He, L.; Aleksandrov, A.A.; Serohijos, A.W.; Hegedus, T.; Aleksandrov, L.A.; Cui, L.; Dokholyan, N.V.; Riordan, J.R.
J. Biol. Chem.
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Mutations at the signature sequence of CFTR create a Cd(2+)-gated chloride channel
2009
Wang, X.; Bompadre, S.G.; Li, M.; Hwang, T.C.
J. Gen. Physiol.
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Molecular modeling of the heterodimer of human CFTRs nucleotide-binding domains using a protein-protein docking approach
2009
Huang, S.Y.; Bolser, D.; Liu, H.Y.; Hwang, T.C.; Zou, X.
J. Mol. Graph. Model.
27
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Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation
2009
Hwang, T.C.; Sheppard, D.N.
J. Physiol.
587
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Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator
2009
Aleksandrov, A.A.; Cui, L.; Riordan, J.R.
J. Physiol.
587
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Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2
2008
Rosser, M.F.; Grove, D.E.; Chen, L.; Cyr, D.M.
Mol. Biol. Cell
19
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A small-molecule modulator interacts directly with DELTAPhe508-CFTR to modify its ATPase activity and conformational stability
2009
Wellhauser, L.; Chiaw, P.K.; Pasyk, S.; Li, C.; Ramjeesingh, M.; Bear, C.E.
Mol. Pharmacol.
75
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Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator
2009
Muallem, D.; Vergani, P.
Philos. Trans. R. Soc. Lond. B Biol. Sci.
364
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Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters
2008
Jordan, I.K.; Kota, K.C.; Cui, G.; Thompson, C.H.; McCarty, N.A.
Proc. Natl. Acad. Sci. USA
105
18865-18870
Molecular and functional characterization of the cystic fibrosis transmembrane conductance regulator from the Australian common brushtail possum, Trichosurus vulpecula
2010
Demmers, K.J.; Carter, D.; Fan, S.; Mao, P.; Maqbool, N.J.; McLeod, B.J.; Bartolo, R.; Butt, A.G.
J. Comp. Physiol. B
180
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A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution
2012
Galfre, E.; Galeno, L.; Moran, O.
Cell. Mol. Life Sci.
69
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Role of ATP binding and hydrolysis in the gating of the cystic fibrosis transmembrane conductance regulator
2012
Gout, T.
Ann. Thorac. Med.
7
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ATP and AMP mutually influence their interaction with the ATP-binding cassette (ABC) adenylate kinase cystic fibrosis transmembrane conductance regulator (CFTR) at separate binding sites
2013
Randak, C.O.; Dong, Q.; Ver Heul, A.R.; Elcock, A.H.; Welsh, M.J.
J. Biol. Chem.
288
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Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps
2014
Wei, S.; Roessler, B.C.; Chauvet, S.; Guo, J.; Hartman, J.L.; Kirk, K.L.
J. Biol. Chem.
289
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Mutating the conserved Q-loop glutamine 1291 selectively disrupts adenylate kinase-dependent channel gating of the ATP-binding cassette (ABC) adenylate kinase cystic fibrosis transmembrane conductance regulator (CFTR) and reduces channel function in prima
2015
Dong, Q.; Ernst, S.E.; Ostedgaard, L.S.; Shah, V.S.; Ver Heul, A.R.; Welsh, M.J.; Randak, C.O.
J. Biol. Chem.
290
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A single amino acid substitution in CFTR converts ATP to an inhibitory ligand
2014
Lin, W.Y.; Jih, K.Y.; Hwang, T.C.
J. Gen. Physiol.
144
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Localizing a gate in CFTR
2015
Gao, X.; Hwang, T.C.
Proc. Natl. Acad. Sci. USA
112
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R-domain phosphorylation by protein kinase A stimulates dissociation of unhydrolyzed ATP from the first nucleotide-binding site of the cystic fibrosis transmembrane conductance regulator
2018
Aleksandrov, L.A.; Fay, J.F.; Riordan, J.R.
Biochemistry
57
5073-5075
Cryo-EM Visualization of an active high open probability CFTR anion channel
2018
Fay, J.F.; Aleksandrov, L.A.; Jensen, T.J.; Cui, L.L.; Kousouros, J.N.; He, L.; Aleksandrov, A.A.; Gingerich, D.S.; Riordan, J.R.; Chen, J.Z.
Biochemistry
57
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Specific stabilization of CFTR by phosphatidylserine
2017
Hildebrandt, E.; Khazanov, N.; Kappes, J.C.; Dai, Q.; Senderowitz, H.; Urbatsch, I.L.
Biochim. Biophys. Acta
1859
289-293
Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant DELTAF508-CFTR
2018
Odera, M.; Furuta, T.; Sohma, Y.; Sakurai, M.
Biophys. Physicobiol.
15
33-44
Conformational changes of CFTR upon phosphorylation and ATP binding
2017
Zhang, Z.; Liu, F.; Chen, J.
Cell
170
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Cytoplasmic pathway followed by chloride ions to enter the CFTR channel pore
2016
El Hiani, Y.; Negoda, A.; Linsdell, P.
Cell. Mol. Life Sci.
73
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The gating of the CFTR channel
2017
Moran, O.
Cell. Mol. Life Sci.
74
85-92
Combining theoretical and experimental data to decipher CFTR 3D structures and functions
2018
Hoffmann, B.; Elbahnsi, A.; Lehn, P.; Decout, J.L.; Pietrucci, F.; Mornon, J.P.; Callebaut, I.
Cell. Mol. Life Sci.
75
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Cystic fibrosis transmembrane conductance regulator (CFTR) Making an ion channel out of an active transporter structure
2018
Linsdell, P.
Channels
12
284-290
Long-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channels
2016
Wei, S.; Roessler, B.C.; Icyuz, M.; Chauvet, S.; Tao, B.; Hartman, J.L.; Kirk, K.L.
FASEB J.
30
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Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia
2016
Xie, C.; Cao, X.; Chen, X.; Wang, D.; Zhang, W.K.; Sun, Y.; Hu, W.; Zhou, Z.; Wang, Y.; Huang, P.
FASEB J.
30
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External Zn2+ binding to cysteine-substituted cystic fibrosis transmembrane conductance regulator constructs regulates channel gating and curcumin potentiation
2016
Wang, G.; Linsley, R.; Norimatsu, Y.
FEBS J.
283
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How phosphorylation and ATPase activity regulate anion flux though the cystic fibrosis transmembrane conductance regulator (CFTR)
2016
Zwick, M.; Esposito, C.; Hellstern, M.; Seelig, A.
J. Biol. Chem.
291
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Channel gating regulation by the cystic fibrosis transmembrane conductance regulator (CFTR) first cytosolic loop
2016
Ehrhardt, A.; Chung, W.J.; Pyle, L.C.; Wang, W.; Nowotarski, K.; Mulvihill, C.M.; Ramjeesingh, M.; Hong, J.; Velu, S.E.; Lewis, H.A.; Atwell, S.; Aller, S.; Bear, C.E.; Lukacs, G.L.; Kirk, K.L.; Sorscher, E.J.
J. Biol. Chem.
291
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Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator
2018
Wang, C.; Aleksandrov, A.A.; Yang, Z.; Forouhar, F.; Proctor, E.A.; Kota, P.; An, J.; Kaplan, A.; Khazanov, N.; Boel, G.; Stockwell, B.R.; Senderowitz, H.; Dokholyan, N.V.; Riordan, J.R.; Brouillette, C.G.; Hunt, J.F.
J. Biol. Chem.
293
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Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl- channel
2017
Chen, J.H.; Xu, W.; Sheppard, D.N.
J. Physiol.
595
1059-1076
Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations
2018
Zhang, J.; Yu, Y.C.; Yeh, J.T.; Hwang, T.C.
PLoS ONE
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e0209862
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