Reference on EC 5.6.1.6 - channel-conductance-controlling ATPase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Bradbury, N.A.
Intracellular CFTR localization and function
Physiol. Rev.
79
S175-191
1999
Homo sapiens
Sugita, M.; Yue, Y.; Foskett, J.K.
CFTR Cl- channel and CFTR-associated ATP channel: distinct pores regulated by common gates
EMBO J.
17
898-908
1998
Homo sapiens
Sheppard, D.N.; Welsh, M.J.
Structure and function of the CFTR chloride channel
Physiol. Rev.
79
S23-45
1999
Homo sapiens, Mus musculus, shark, Xenopus sp.
Schwiebert, E.M.; Benos, D.J.; Egan, M.E.; Stutts, M.J.; Guggino, W.B.
CFTR is a conductance regulator as well as a chloride channel
Physiol. Rev.
79
S145-166
1999
Homo sapiens
Weinreich, F.; Riordan, J.R.; Nagel, G.
Dual effects of ADP and adenylylimidodiphosphate on CFTR channel kinetics show binding to two different nucleotide binding sites
J. Gen. Physiol.
114
55-70
1999
Homo sapiens
Illek, B.; Fischer, H.; Machen, T.E.
Genetic disorders of membrane transport. II. Regulation of CFTR by small molecules including HCO3
Am. J. Physiol.
275
G1221-1226
1998
Homo sapiens
Seibert, F.S.; Chang, X.B.; Aleksandrov, A.A.; Clarke, D.M.; Hanrahan, J.W.; Riordan, J.R.
Influence of phosphorylation by protein kinase A on CFTR at the cell surface and endoplasmic reticulum
Biochim. Biophys. Acta
1461
275-283
1999
Homo sapiens
Bear, C.E.; Li, C.; Galley, K.; Wang, Y.; Garami, E.; Ramjeesingh, M.
Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR
J. Bioenerg. Biomembr.
29
465-473
1997
Homo sapiens
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.
Syntaxin 1A is expressed in airway epithelial cells, where it modulates CFTR Cl(-) currents
J. Clin. Invest.
105
377-386
2000
Homo sapiens
Naren, A.P.; Quick, M.W.; Collawn, J.F.; Nelson, D.J.; Kirk, K.L.
Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactions
Proc. Natl. Acad. Sci. USA
95
10972-10977
1998
Homo sapiens
Chen, M.; Zhang, J.T.
Membrane insertion, processing, and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes
Mol. Membr. Biol.
13
33-40
1996
Homo sapiens
Tusnady, G.E.; Bakos, E.; Varadi, A.; Sarkadi, B.
Membrane topology distinguishes a subfamily of the ATP-binding cassette (ABC) transporters
FEBS Lett.
402
1-3
1997
Homo sapiens
Price, M.P.; Ishihara, H.; Sheppard, D.N.; Welsh, M.J.
Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR
J. Biol. Chem.
271
25184-25191
1996
Homo sapiens, Xenopus sp.
Quinton, P.M.; Reddy, M.M.
Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding
Nature
360
79-81
1992
Homo sapiens
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.
Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP binding cassette transporter
Proc. Natl. Acad. Sci. USA
93
5401-5406
1996
Oryctolagus cuniculus, Homo sapiens
Kleizen, B.; Braakman, I.; de Jonge, H.R.
Regulated trafficking of the CFTR chloride channel
Eur. J. Cell Biol.
79
544-556
2000
Homo sapiens
Annereau, J.P.; Ko, Y.H.; Pedersen, P.L.
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
Biochem. J.
371
451-462
2003
Homo sapiens
Howell, L.D.; Borchardt, R.; Kole, J.; Kaz, A.M.; Randak, C.; Cohn, J.A.
Protein kinase A regulates ATP hydrolysis and dimerization by a CFTR (cystic fibrosis transmembrane conductance regulator) domain
Biochem. J.
378
151-159
2004
Homo sapiens
Ramjeesingh, M.; Li, C.; Kogan, I.; Wang, Y.; Huan, L.J.; Bear, C.E.
A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator
Biochemistry
40
10700-10706
2001
Homo sapiens
Ketchum, C.J.; Rajendrakumar, G.V.; Maloney, P.C.
Characterization of the adenosinetriphosphatase and transport activities of purified cystic fibrosis transmembrane conductance regulator
Biochemistry
43
1045-1053
2004
Homo sapiens
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.
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
EMBO J.
23
282-293
2004
Mus musculus
Kogan, I.; Ramjeesingh, M.; Huan, L.J.; Wang, Y.; Bear, C.E.
Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its ATPase activity
J. Biol. Chem.
276
11575-11581
2001
Homo sapiens
Kidd Jackie, F.; Ramjeesingh, M.; Stratford, F.; Huan, L.J.; Bear Christine, E.
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity
J. Biol. Chem.
279
41664-41669
2004
Homo sapiens
Vergani, P.; Nairn, A.C.; Gadsby, D.C.
On the mechanism of MgATP-dependent gating of CFTR Cl- channels
J. Gen. Physiol.
121
17-36
2003
Homo sapiens
Fang, X.; Song, Y.; Hirsch, J.; Galietta, L.J.; Pedemonte, N.; Zemans, R.L.; Dolganov, G.; Verkman, A.S.; Matthay, M.A.
Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells
Am. J. Physiol. Lung Cell Mol. Physiol.
290
L242-L249
2006
Homo sapiens
Tse, W.K.; Au, D.W.; Wong, C.K.
Characterization of ion channel and transporter mRNA expressions in isolated gill chloride and pavement cells of seawater acclimating eels
Biochem. Biophys. Res. Commun.
346
1181-1190
2006
Anguilla japonica
van Barneveld, A.; Stanke, F.; Ballmann, M.; Naim, H.Y.; Tuemmler, B.
Ex vivo biochemical analysis of CFTR in human rectal biopsies
Biochim. Biophys. Acta
1762
393-397
2006
Homo sapiens
Fuller, M.D.; Zhang, Z.R.; Cui, G.; McCarty, N.A.
The block of CFTR by scorpion venom is state-dependent
Biophys. J.
89
3960-3975
2005
Homo sapiens
Galietta, L.J.; Moran, O.
Identification of CFTR activators and inhibitors: chance or design?
Curr. Opin. Pharmacol.
4
497-503
2004
Homo sapiens
Himmel, B.; Nagel, G.
Protein kinase-independent activation of CFTR by phosphatidylinositol phosphates
EMBO Rep.
5
85-90
2004
Homo sapiens
Linsdell, P.
Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channel
Exp. Physiol.
91
123-129
2006
Homo sapiens
Yan, W.; Samaha, F.F.; Ramkumar, M.; Kleyman, T.R.; Rubenstein, R.C.
Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes
J. Biol. Chem.
279
23183-23192
2004
Homo sapiens
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
39051-39057
2004
Homo sapiens
Fischer, H.; Machen, T.E.; Widdicombe, J.H.; Carlson, T.J.; King, S.R.; Chow, J.W.; Illek, B.
A novel extract SB-300 from the stem bark latex of Croton lechleri inhibits CFTR-mediated chloride secretion in human colonic epithelial cells
J. Ethnopharmacol.
93
351-357
2004
Homo sapiens
Ratner, M.A.; Decker, S.E.; Aller, S.G.; Weber, G.; Forrest, J.N.
Mercury toxicity in the shark (Squalus acanthias) rectal gland: apical CFTR chloride channels are inhibited by mercuric chloride
J. Exp. Zool. A
305
259-267
2006
Squalus acanthias
Csanady, L.; Seto-Young, D.; Chan, K.W.; Cenciarelli, C.; Angel, B.B.; Qin, J.; McLachlin, D.T.; Krutchinsky, A.N.; Chait, B.T.; Nairn, A.C.; Gadsby, D.C.
Preferential phosphorylation of R-domain Serine 768 dampens activation of CFTR channels by PKA
J. Gen. Physiol.
125
171-186
2005
Homo sapiens
Csanady, L.; Chan, K.W.; Nairn, A.C.; Gadsby, D.C.
Functional roles of nonconserved structural segments in CFTRs NH2-terminal nucleotide binding domain
J. Gen. Physiol.
125
43-55
2005
Homo sapiens
Carvalho-Oliveira, I.; Efthymiadou, A.; Malho, R.; Nogueira, P.; Tzetis, M.; Kanavakis, E.; Amaral, M.D.; Penque, D.
CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies
J. Histochem. Cytochem.
52
193-203
2004
Homo sapiens
Robert, R.; Norez, C.; Becq, F.
Disruption of CFTR chloride channel alters mechanical properties and cAMP-dependent Cl- transport of mouse aortic smooth muscle cells
J. Physiol.
568
483-495
2005
Mus musculus
Goodstadt, L.; Powell, T.; Figtree, G.A.
17beta-estradiol potentiates the cardiac cystic fibrosis transmembrane conductance regulator chloride current in guinea-pig ventricular myocytes
J. Physiol. Sci.
56
29-37
2006
Cavia porcellus
Ajonuma, L.C.; Tsang, L.L.; Zhang, G.H.; Wong, C.H.; Lau, M.C.; Ho, L.S.; Rowlands, D.K.; Zhou, C.X.; Ng, C.P.; Chen, J.; Xu, P.H.; Zhu, J.X.; Chung, Y.W.; Chan, H.C.
Estrogen-induced abnormally high cystic fibrosis transmembrane conductance regulator expression results in ovarian hyperstimulation syndrome
Mol. Endocrinol.
19
3038-3044
2005
Rattus norvegicus
Hanrahan, J.W.; Wioland, M.A.
Revisiting cystic fibrosis transmembrane conductance regulator structure and function
Proc. Am. Thorac. Soc.
1
17-21
2004
Rattus norvegicus
Stratford, F.L.; Ramjeesingh, M.; Cheung, J.C.; Huan, L.J.; Bear, C.E.
The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer
Biochem. J.
401
581-586
2007
Homo sapiens
Ramjeesingh, M.; Ugwu, F.; Stratford, F.L.; Huan, L.J.; Li, C.; Bear, C.E.
The intact CFTR protein mediates ATPase rather than adenylate kinase activity
Biochem. J.
412
315-321
2008
Homo sapiens
Chang, Y.T.; Chang, M.C.; Su, T.C.; Liang, P.C.; Su, Y.N.; Kuo, C.H.; Wei, S.C.; Wong, J.M.
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis
Clin. Chem.
54
131-138
2008
Homo sapiens
Mense, M.; Vergani, P.; White, D.M.; Altberg, G.; Nairn, A.C.; Gadsby, D.C.
In vivo phosphorylation of CFTR promotes formation of a nucleotide-binding domain heterodimer
EMBO J.
25
4728-4739
2006
Homo sapiens (P13569)
Beck, E.J.; Yang, Y.; Yaemsiri, S.; Raghuram, V.
Conformational changes in a pore-lining helix coupled to cystic fibrosis transmembrane conductance regulator channel gating
J. Biol. Chem.
283
4957-4966
2008
Homo sapiens
Bompadre, S.G.; Li, M.; Hwang, T.C.
Mechanism of G551D-CFTR (cystic fibrosis transmembrane conductance regulator) potentiation by a high affinity ATP analog
J. Biol. Chem.
283
5364-5369
2008
Homo sapiens (P13569)
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
413-422
2006
Homo sapiens (P13569)
Ferrera, L.; Pincin, C.; Moran, O.
Characterization of a 7,8-benzoflavone double effect on CFTR Cl(-) channel activity
J. Membr. Biol.
220
1-9
2007
Homo sapiens
Cui, L.; Aleksandrov, L.; Hou, Y.; Gentzsch, M.; Chen, J.; Riordan, J.R.; Aleksandrov, A.A.
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating
J. Physiol.
572
347-358
2006
Homo sapiens
Scott-Ward, T.S.; Cai, Z.; Dawson, E.S.; Doherty, A.; Da Paula, A.C.; Davidson, H.; Porteous, D.J.; Wainwright, B.J.; Amaral, M.D.; Sheppard, D.N.; Boyd, A.C.
Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR
Proc. Natl. Acad. Sci. USA
104
16365-16370
2007
Homo sapiens, Mus musculus
Serohijos, A.W.; Hegedus, T.; Aleksandrov, A.A.; He, L.; Cui, L.; Dokholyan, N.V.; Riordan, J.R.
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
Proc. Natl. Acad. Sci. USA
105
3256-3261
2008
Homo sapiens
Bajmoczi, M.; Gadjeva, M.; Alper, S.L.; Pier, G.; Golan, D.E.
Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa
Am. J. Physiol. Cell Physiol.
297
C263-C277
2009
Homo sapiens
Aleksandrov, L.; Aleksandrov, A.; Riordan, J.R.
Mg2+-dependent ATP occlusion at the first nucleotide-binding domain (NBD1) of CFTR does not require the second (NBD2)
Biochem. J.
416
129-136
2008
Homo sapiens
Shamsuddin, A.K.; Reddy, M.M.; Quinton, P.M.
Iontophoretic beta-adrenergic stimulation of human sweat glands: possible assay for cystic fibrosis transmembrane conductance regulator activity in vivo
Exp. Physiol.
93
969-981
2008
Homo sapiens
Sun, F.; Mi, Z.; Condliffe, S.B.; Bertrand, C.A.; Gong, X.; Lu, X.; Zhang, R.; Latoche, J.D.; Pilewski, J.M.; Robbins, P.D.; Frizzell, R.A.
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia
FASEB J.
22
3255-3263
2008
Homo sapiens
Bijvelds, M.J.; Bot, A.G.; Escher, J.C.; de Jonge, H.R.
Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator
Gastroenterology
137
976-985
2009
Homo sapiens, Mus musculus
He, L.; Aleksandrov, A.A.; Serohijos, A.W.; Hegedus, T.; Aleksandrov, L.A.; Cui, L.; Dokholyan, N.V.; Riordan, J.R.
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating
J. Biol. Chem.
283
26383-26390
2008
Homo sapiens
Mio, K.; Ogura, T.; Mio, M.; Shimizu, H.; Hwang, T.C.; Sato, C.; Sohma, Y.
Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain
J. Biol. Chem.
283
30300-30310
2008
Homo sapiens
Fatehi, M.; Linsdell, P.
State-dependent access of anions to the cystic fibrosis transmembrane conductance regulator chloride channel pore
J. Biol. Chem.
283
6102-6109
2008
Homo sapiens
Segal, I.; Yaakov, Y.; Adler, S.N.; Blau, H.; Broide, E.; Santo, M.; Yahav, Y.; Klar, A.; Lerner, A.; Aviram, M.; Ellis, I.; Mountford, R.; Shteyer, E.; Kerem, E.; Wilschanski, M.
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis
J. Clin. Gastroenterol.
42
810-814
2008
Homo sapiens
Wang, X.; Bompadre, S.G.; Li, M.; Hwang, T.C.
Mutations at the signature sequence of CFTR create a Cd(2+)-gated chloride channel
J. Gen. Physiol.
133
69-77
2009
Homo sapiens
Huang, S.Y.; Bolser, D.; Liu, H.Y.; Hwang, T.C.; Zou, X.
Molecular modeling of the heterodimer of human CFTRs nucleotide-binding domains using a protein-protein docking approach
J. Mol. Graph. Model.
27
822-828
2009
Homo sapiens (P13569), Homo sapiens
Hwang, T.C.; Sheppard, D.N.
Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation
J. Physiol.
587
2151-2161
2009
Homo sapiens
Aleksandrov, A.A.; Cui, L.; Riordan, J.R.
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator
J. Physiol.
587
2875-2886
2009
Homo sapiens
Rosser, M.F.; Grove, D.E.; Chen, L.; Cyr, D.M.
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
Mol. Biol. Cell
19
4570-4579
2008
Homo sapiens
Barriere, H.; Bagdany, M.; Bossard, F.; Okiyoneda, T.; Wojewodka, G.; Gruenert, D.; Radzioch, D.; Lukacs, G.L.
Revisiting the role of CFTR and counterion permeability in the pH regulation of endocytic organelles
Mol. Biol. Cell
20
3125-3141
2009
Homo sapiens, Mus musculus
Wellhauser, L.; Chiaw, P.K.; Pasyk, S.; Li, C.; Ramjeesingh, M.; Bear, C.E.
A small-molecule modulator interacts directly with DELTAPhe508-CFTR to modify its ATPase activity and conformational stability
Mol. Pharmacol.
75
1430-1438
2009
Homo sapiens
Muallem, D.; Vergani, P.
Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator
Philos. Trans. R. Soc. Lond. B Biol. Sci.
364
247-255
2009
Homo sapiens
Jordan, I.K.; Kota, K.C.; Cui, G.; Thompson, C.H.; McCarty, N.A.
Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters
Proc. Natl. Acad. Sci. USA
105
18865-18870
2008
Homo sapiens
Midha, S.; Khajuria, R.; Shastri, S.; Kabra, M.; Garg, P.K.
Idiopathic chronic pancreatitis in India: phenotypic characterisation and strong genetic susceptibility due to SPINK1 and CFTR gene mutations
Gut
59
800-807
2010
Homo sapiens
Demmers, K.J.; Carter, D.; Fan, S.; Mao, P.; Maqbool, N.J.; McLeod, B.J.; Bartolo, R.; Butt, A.G.
Molecular and functional characterization of the cystic fibrosis transmembrane conductance regulator from the Australian common brushtail possum, Trichosurus vulpecula
J. Comp. Physiol. B
180
545-561
2010
Trichosurus vulpecula (Q5D1Z7), Trichosurus vulpecula
Lewis, H.A.; Wang, C.; Zhao, X.; Hamuro, Y.; Conners, K.; Kearins, M.C.; Lu, F.; Sauder, J.M.; Molnar, K.S.; Coales, S.J.; Maloney, P.C.; Guggino, W.B.; Wetmore, D.R.; Weber, P.C.; Hunt, J.F.
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry
J. Mol. Biol.
396
406-430
2010
Homo sapiens (P13569), Homo sapiens
Kelly, M.; Trudel, S.; Brouillard, F.; Bouillaud, F.; Colas, J.; Nguyen-Khoa, T.; Ollero, M.; Edelman, A.; Fritsch, J.
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition
J. Pharmacol. Exp. Ther.
333
60-69
2010
Homo sapiens
El Hiani, Y.; Linsdell, P.
Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel
Biochemistry
51
3971-3981
2012
Homo sapiens
Galfre, E.; Galeno, L.; Moran, O.
A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution
Cell. Mol. Life Sci.
69
3701-3713
2012
Homo sapiens
Cho, H.J.; Gee, H.Y.; Baek, K.H.; Ko, S.K.; Park, J.M.; Lee, H.; Kim, N.D.; Lee, M.G.; Shin, I.
A small molecule that binds to an ATPase domain of Hsc70 promotes membrane trafficking of mutant cystic fibrosis transmembrane conductance regulator
J. Am. Chem. Soc.
133
20267-20276
2011
Homo sapiens
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
37663-37671
2010
Homo sapiens
Wang, W.; Linsdell, P.
Alternating access to the transmembrane domain of the ATP-binding cassette protein cystic fibrosis transmembrane conductance regulator (ABCC7)
J. Biol. Chem.
287
10156-10165
2012
Homo sapiens
Bai, Y.; Li, M.; Hwang, T.C.
Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7)
J. Gen. Physiol.
138
495-507
2011
Homo sapiens
Shimizu, H.; Yu, Y.C.; Kono, K.; Kubota, T.; Yasui, M.; Li, M.; Hwang, T.C.; Sohma, Y.
A stable ATP binding to the nucleotide binding domain is important for reliable gating cycle in an ABC transporter CFTR
J. Physiol. Sci.
60
353-362
2010
Homo sapiens
Krasilnikov, O.V.; Sabirov, R.Z.; Okada, Y.
ATP hydrolysis-dependent asymmetry of the conformation of CFTR channel pore
J. Physiol. Sci.
61
267-278
2011
Homo sapiens
Tu, J.; Le, G.; Ballard, H.J.
Involvement of the cystic fibrosis transmembrane conductance regulator in the acidosis-induced efflux of ATP from rat skeletal muscle
J. Physiol.
588
4563-4578
2010
Rattus norvegicus
Gout, T.
Role of ATP binding and hydrolysis in the gating of the cystic fibrosis transmembrane conductance regulator
Ann. Thorac. Med.
7
115-121
2012
Homo sapiens (P13569)
Wang, G.
Molecular basis for Fe(III)-independent curcumin potentiation of cystic fibrosis transmembrane conductance regulator activity
Biochemistry
54
2828-2840
2015
Homo sapiens (P13569), Homo sapiens
Zhang, J.; Hwang, T.C.
The fifth transmembrane segment of cystic fibrosis transmembrane conductance regulator contributes to its anion permeation pathway
Biochemistry
54
3839-3850
2015
Homo sapiens (P13569)
Cui, G.; Freeman, C.S.; Knotts, T.; Prince, C.Z.; Kuang, C.; McCarty, N.A.
Two salt bridges differentially contribute to the maintenance of cystic fibrosis transmembrane conductance regulator (CFTR) channel function
J. Biol. Chem.
288
20758-20767
2013
Homo sapiens (P13569), Homo sapiens
Randak, C.O.; Dong, Q.; Ver Heul, A.R.; Elcock, A.H.; Welsh, M.J.
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
J. Biol. Chem.
288
27692-27701
2013
Homo sapiens (P13569)
Wei, S.; Roessler, B.C.; Chauvet, S.; Guo, J.; Hartman, J.L.; Kirk, K.L.
Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps
J. Biol. Chem.
289
19942-19957
2014
Homo sapiens (P13569)
Dong, Q.; Ernst, S.E.; Ostedgaard, L.S.; Shah, V.S.; Ver Heul, A.R.; Welsh, M.J.; Randak, C.O.
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
J. Biol. Chem.
290
14140-14153
2015
Homo sapiens (P13569), Homo sapiens
Corradi, V.; Vergani, P.; Tieleman, D.P.
Cystic fibrosis transmembrane conductance regulator (CFTR): closed and open state channel models
J. Biol. Chem.
290
22891-22906
2015
Homo sapiens (P13569)
Lin, W.Y.; Jih, K.Y.; Hwang, T.C.
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand
J. Gen. Physiol.
144
311-320
2014
Homo sapiens (P13569), Homo sapiens
Ruan, Y.C.; Shum, W.W.; Belleannee, C.; Da Silva, N.; Breton, S.
ATP secretion in the male reproductive tract: essential role of CFTR
J. Physiol.
590
4209-4222
2012
Mus musculus (P26361), Mus musculus C57BL/6 (P26361)
Cai, Z.; Palmai-Pallag, T.; Khuituan, P.; Mutolo, M.J.; Boinot, C.; Liu, B.; Scott-Ward, T.S.; Callebaut, I.; Harris, A.; Sheppard, D.N.
Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating
J. Physiol.
593
2427-2446
2015
Ovis aries (Q00555)
Gao, X.; Hwang, T.C.
Localizing a gate in CFTR
Proc. Natl. Acad. Sci. USA
112
2461-2466
2015
Homo sapiens (P13569)
Aleksandrov, L.A.; Fay, J.F.; Riordan, J.R.
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
Biochemistry
57
5073-5075
2018
Homo sapiens
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.
Cryo-EM Visualization of an active high open probability CFTR anion channel
Biochemistry
57
6234-6246
2018
Gallus gallus (A0M8U4)
Hildebrandt, E.; Khazanov, N.; Kappes, J.C.; Dai, Q.; Senderowitz, H.; Urbatsch, I.L.
Specific stabilization of CFTR by phosphatidylserine
Biochim. Biophys. Acta
1859
289-293
2017
Homo sapiens
Odera, M.; Furuta, T.; Sohma, Y.; Sakurai, M.
Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant DELTAF508-CFTR
Biophys. Physicobiol.
15
33-44
2018
Homo sapiens
Zhang, Z.; Liu, F.; Chen, J.
Conformational changes of CFTR upon phosphorylation and ATP binding
Cell
170
483-491.e8
2017
Danio rerio (Q1LX78), Danio rerio
El Hiani, Y.; Negoda, A.; Linsdell, P.
Cytoplasmic pathway followed by chloride ions to enter the CFTR channel pore
Cell. Mol. Life Sci.
73
1917-1925
2016
Homo sapiens
Moran, O.
The gating of the CFTR channel
Cell. Mol. Life Sci.
74
85-92
2017
Homo sapiens
Hoffmann, B.; Elbahnsi, A.; Lehn, P.; Decout, J.L.; Pietrucci, F.; Mornon, J.P.; Callebaut, I.
Combining theoretical and experimental data to decipher CFTR 3D structures and functions
Cell. Mol. Life Sci.
75
3829-3855
2018
Homo sapiens
Linsdell, P.
Cystic fibrosis transmembrane conductance regulator (CFTR) Making an ion channel out of an active transporter structure
Channels
12
284-290
2018
Homo sapiens
Wei, S.; Roessler, B.C.; Icyuz, M.; Chauvet, S.; Tao, B.; Hartman, J.L.; Kirk, K.L.
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
FASEB J.
30
1247-1262
2016
Homo sapiens
Xie, C.; Cao, X.; Chen, X.; Wang, D.; Zhang, W.K.; Sun, Y.; Hu, W.; Zhou, Z.; Wang, Y.; Huang, P.
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia
FASEB J.
30
1579-1589
2016
Homo sapiens, Mus musculus
Wang, G.; Linsley, R.; Norimatsu, Y.
External Zn2+ binding to cysteine-substituted cystic fibrosis transmembrane conductance regulator constructs regulates channel gating and curcumin potentiation
FEBS J.
283
2458-2475
2016
Homo sapiens
Zwick, M.; Esposito, C.; Hellstern, M.; Seelig, A.
How phosphorylation and ATPase activity regulate anion flux though the cystic fibrosis transmembrane conductance regulator (CFTR)
J. Biol. Chem.
291
14483-14498
2016
Homo sapiens
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.
Channel gating regulation by the cystic fibrosis transmembrane conductance regulator (CFTR) first cytosolic loop
J. Biol. Chem.
291
1854-1865
2016
Homo sapiens
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.
Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator
J. Biol. Chem.
293
17685-17704
2018
Homo sapiens (P13569), Homo sapiens
Chen, J.H.; Xu, W.; Sheppard, D.N.
Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl- channel
J. Physiol.
595
1059-1076
2017
Homo sapiens
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