-
1
-
-
78549279173
-
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation
-
Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordonez CL, Campbell PW, Ashlock MA, Ramsey BW. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 363: 1991-2003, 2010.
-
(2010)
N Engl J Med
, vol.363
, pp. 1991-2003
-
-
Accurso, F.J.1
Rowe, S.M.2
Clancy, J.P.3
Boyle, M.P.4
Dunitz, J.M.5
Durie, P.R.6
Sagel, S.D.7
Hornick, D.B.8
Konstan, M.W.9
Donaldson, S.H.10
Moss, R.B.11
Pilewski, J.M.12
Rubenstein, R.C.13
Uluer, A.Z.14
Aitken, M.L.15
Freedman, S.D.16
Rose, L.M.17
Mayer-Hamblett, N.18
Dong, Q.19
Zha, J.20
Stone, A.J.21
Olson, E.R.22
Ordonez, C.L.23
Campbell, P.W.24
Ashlock, M.A.25
Ramsey, B.W.26
more..
-
2
-
-
0037042224
-
Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis
-
Aleksandrov AA, Aleksandrov L, Riordan JR. Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis. FEBS Lett 518: 183-188, 2002.
-
(2002)
FEBS Lett
, vol.518
, pp. 183-188
-
-
Aleksandrov, A.A.1
Aleksandrov, L.2
Riordan, J.R.3
-
4
-
-
0033759953
-
The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating
-
Aleksandrov AA, Chang X, Aleksandrov L, Riordan JR. The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating. J Physiol 528: 259-265, 2000.
-
(2000)
J Physiol
, vol.528
, pp. 259-265
-
-
Aleksandrov, A.A.1
Chang, X.2
Aleksandrov, L.3
Riordan, J.R.4
-
5
-
-
67649659825
-
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator
-
Aleksandrov AA, Cui L, Riordan JR. Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator. J Physiol 587: 2875-2886, 2009.
-
(2009)
J Physiol
, vol.587
, pp. 2875-2886
-
-
Aleksandrov, A.A.1
Cui, L.2
Riordan, J.R.3
-
6
-
-
70350236733
-
Cystic fibrosis transmembrane conductance regulator: Using differential reactivity toward channelpermeant and channel-impermeant thiol-reactive probes to test a molecular model for the pore
-
Alexander C, Ivetac A, Liu X, Norimatsu Y, Serrano JR, Landstrom A, Sansom M, Dawson DC. Cystic fibrosis transmembrane conductance regulator: using differential reactivity toward channelpermeant and channel-impermeant thiol-reactive probes to test a molecular model for the pore. Biochemistry 48: 10078-10088, 2009.
-
(2009)
Biochemistry
, vol.48
, pp. 10078-10088
-
-
Alexander, C.1
Ivetac, A.2
Liu, X.3
Norimatsu, Y.4
Serrano, J.R.5
Landstrom, A.6
Sansom, M.7
Dawson, D.C.8
-
7
-
-
63449139456
-
Structure of P-glycoprotein reveals a molecular basis for poly-specific drug binding
-
Aller SG, Yu J, Ward A, Weng Y, Chittaboina S, Zhuo R, Harrell PM, Trinh YT, Zhang Q, Urbatsch IL, Chang G. Structure of P-glycoprotein reveals a molecular basis for poly-specific drug binding. Science 323: 1718-1722, 2009.
-
(2009)
Science
, vol.323
, pp. 1718-1722
-
-
Aller, S.G.1
Yu, J.2
Ward, A.3
Weng, Y.4
Chittaboina, S.5
Zhuo, R.6
Harrell, P.M.7
Trinh, Y.T.8
Zhang, Q.9
Urbatsch, I.L.10
Chang, G.11
-
8
-
-
77956237499
-
Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation
-
Bai Y, Li M, Hwang TC. Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation. J Gen Physiol 136: 293-309, 2010.
-
(2010)
J Gen Physiol
, vol.136
, pp. 293-309
-
-
Bai, Y.1
Li, M.2
Hwang, T.C.3
-
9
-
-
80555127440
-
Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7)
-
Bai Y, Li M, Hwang TC. Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7). J Gen Physiol 138: 495-507, 2011.
-
(2011)
J Gen Physiol
, vol.138
, pp. 495-507
-
-
Bai, Y.1
Li, M.2
Hwang, T.C.3
-
10
-
-
34547652267
-
CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices
-
Baker JM, Hudson RP, Kanelis V, Choy WY, Thibodeau PH, Thomas PJ, Forman-Kay JD. CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices. Nature Struct Mol Biol 14: 738-745, 2007.
-
(2007)
Nature Struct Mol Biol
, vol.14
, pp. 738-745
-
-
Baker, J.M.1
Hudson, R.P.2
Kanelis, V.3
Choy, W.Y.4
Thibodeau, P.H.5
Thomas, P.J.6
Forman-Kay, J.D.7
-
11
-
-
0026532895
-
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Bear CE, Li CH, Kartner N, Bridges RJ, Jensen TJ, Ramjeesingh M, Riordan JR. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 68: 809-818, 1992.
-
(1992)
Cell
, vol.68
, pp. 809-818
-
-
Bear, C.E.1
Li, C.H.2
Kartner, N.3
Bridges, R.J.4
Jensen, T.J.5
Ramjeesingh, M.6
Riordan, J.R.7
-
12
-
-
17044383747
-
CFTR gating I: Characterization of the ATP-dependent gating of a phosphorylation-independent CFTR channel (DeltaR-CFTR)
-
Bompadre SG, Ai T, Cho JH, Wang X, Sohma Y, Li M, Hwang TC. CFTR gating I: characterization of the ATP-dependent gating of a phosphorylation-independent CFTR channel (DeltaR-CFTR). J Gen Physiol 125: 361-375, 2005.
-
(2005)
J Gen Physiol
, vol.125
, pp. 361-375
-
-
Bompadre, S.G.1
Ai, T.2
Cho, J.H.3
Wang, X.4
Sohma, Y.5
Li, M.6
Hwang, T.C.7
-
13
-
-
17044401724
-
CFTR gating II: Effects of nucleotide binding on the stability of open states
-
Bompadre SG, Cho JH, Wang X, Zou X, Sohma Y, Li M, Hwang TC. CFTR gating II: Effects of nucleotide binding on the stability of open states. J Gen Physiol 125: 377-394, 2005.
-
(2005)
J Gen Physiol
, vol.125
, pp. 377-394
-
-
Bompadre, S.G.1
Cho, J.H.2
Wang, X.3
Zou, X.4
Sohma, Y.5
Li, M.6
Hwang, T.C.7
-
14
-
-
33947725805
-
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects
-
Bompadre SG, Sohma Y, Li M, Hwang TC. G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol 129: 285-298, 2007.
-
(2007)
J Gen Physiol
, vol.129
, pp. 285-298
-
-
Bompadre, S.G.1
Sohma, Y.2
Li, M.3
Hwang, T.C.4
-
15
-
-
79958088886
-
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis
-
Cai ZW, Liu J, Li HY, Sheppard DN. Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis. Acta Pharmacol Sin 32: 693-701, 2011.
-
(2011)
Acta Pharmacol Sin
, vol.32
, pp. 693-701
-
-
Cai, Z.W.1
Liu, J.2
Li, H.Y.3
Sheppard, D.N.4
-
16
-
-
0028906612
-
The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity
-
Carson MR, Travis SM, Welsh MJ. The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity. J Biol Chem 270: 1711-1717, 1995.
-
(1995)
J Biol Chem
, vol.270
, pp. 1711-1717
-
-
Carson, M.R.1
Travis, S.M.2
Welsh, M.J.3
-
17
-
-
0042025073
-
N-terminal transmembrane domain of the SUR controls trafficking and gating of Kir6 channel subunits
-
Chan KW, Zhang H, Logothetis DE. N-terminal transmembrane domain of the SUR controls trafficking and gating of Kir6 channel subunits. EMBO J 22: 3833-3843, 2003.
-
(2003)
EMBO J
, vol.22
, pp. 3833-3843
-
-
Chan, K.W.1
Zhang, H.2
Logothetis, D.E.3
-
18
-
-
42049096734
-
CLC-0 and CFTR: Chloride channels evolved from transporters
-
Chen TY, Hwang TC. CLC-0 and CFTR: chloride channels evolved from transporters. Physiol Rev 88: 351-387, 2008.
-
(2008)
Physiol Rev
, vol.88
, pp. 351-387
-
-
Chen, T.Y.1
Hwang, T.C.2
-
19
-
-
0033230588
-
Coupled chemical and mechanical reaction steps in a processive Neurospora kinesin
-
Crevel I, Carter N, Schliwa M, Cross R. Coupled chemical and mechanical reaction steps in a processive Neurospora kinesin. EMBO J 18: 5863-5872, 1999.
-
(1999)
EMBO J
, vol.18
, pp. 5863-5872
-
-
Crevel, I.1
Carter, N.2
Schliwa, M.3
Cross, R.4
-
20
-
-
67749084300
-
Application of rate-equilibrium free energy relationship analysis to nonequilibrium ion channel gating mechanisms
-
Csanady L. Application of rate-equilibrium free energy relationship analysis to nonequilibrium ion channel gating mechanisms. J Gen Physiol 134: 129-136, 2009.
-
(2009)
J Gen Physiol
, vol.134
, pp. 129-136
-
-
Csanady, L.1
-
21
-
-
0033817333
-
Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains
-
Csanady L, Chan KW, Seto-Young D, Kopsco DC, Nairn AC, Gadsby DC. Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains. J Gen Physiol 116: 477-500, 2000.
-
(2000)
J Gen Physiol
, vol.116
, pp. 477-500
-
-
Csanady, L.1
Chan, K.W.2
Seto-Young, D.3
Kopsco, D.C.4
Nairn, A.C.5
Gadsby, D.C.6
-
22
-
-
33750499982
-
Thermodynamics of CFTR channel gating: A spreading conformational change initiates an irreversible gating cycle
-
Csanady L, Nairn AC, Gadsby DC. Thermodynamics of CFTR channel gating: a spreading conformational change initiates an irreversible gating cycle. J Gen Physiol 128: 523-533, 2006.
-
(2006)
J Gen Physiol
, vol.128
, pp. 523-533
-
-
Csanady, L.1
Nairn, A.C.2
Gadsby, D.C.3
-
23
-
-
75749153312
-
Strict coupling between CFTR's catalytic cycle and gating of its Cl ion pore revealed by distributions of open channel burst durations
-
Csanady L, Vergani P, Gadsby DC. Strict coupling between CFTR's catalytic cycle and gating of its Cl ion pore revealed by distributions of open channel burst durations. Proc Natl Acad Sci USA 107: 1241-1246, 2010.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 1241-1246
-
-
Csanady, L.1
Vergani, P.2
Gadsby, D.C.3
-
24
-
-
33845739839
-
Domain interdependence in the biosynthetic assembly of CFTR
-
Cui L, Aleksandrov L, Chang XB, Hou YX, He L, Hegedus T, Gentzsch M, Aleksandrov A, Balch WE, Riordan JR. Domain interdependence in the biosynthetic assembly of CFTR. J Mol Biol 365: 981-994, 2007.
-
(2007)
J Mol Biol
, vol.365
, pp. 981-994
-
-
Cui, L.1
Aleksandrov, L.2
Chang, X.B.3
Hou, Y.X.4
He, L.5
Hegedus, T.6
Gentzsch, M.7
Aleksandrov, A.8
Balch, W.E.9
Riordan, J.R.10
-
25
-
-
3943062954
-
ATP-binding cassette transporters in bacteria
-
Davidson AL, Chen J. ATP-binding cassette transporters in bacteria. Ann Rev Biochem 73: 241-268, 2004.
-
(2004)
Ann Rev Biochem
, vol.73
, pp. 241-268
-
-
Davidson, A.L.1
Chen, J.2
-
26
-
-
33748644877
-
Structure of a bacterial multidrug ABC transporter
-
Dawson RJ, Locher KP. Structure of a bacterial multidrug ABC transporter. Nature 443: 180-185, 2006.
-
(2006)
Nature
, vol.443
, pp. 180-185
-
-
Dawson, R.J.1
Locher, K.P.2
-
27
-
-
77957761417
-
Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel
-
El Hiani Y, Linsdell P. Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem 285: 32126-32140, 2010.
-
(2010)
J Biol Chem
, vol.285
, pp. 32126-32140
-
-
El Hiani, Y.1
Linsdell, P.2
-
28
-
-
78049362741
-
Structure of a eukaryotic CLC transporter defines an intermediate state in the transport cycle
-
Feng L, Campbell EB, Hsiung Y, MacKinnon R. Structure of a eukaryotic CLC transporter defines an intermediate state in the transport cycle. Science 330: 635-641, 2010.
-
(2010)
Science
, vol.330
, pp. 635-641
-
-
Feng, L.1
Campbell, E.B.2
Hsiung, Y.3
Mackinnon, R.4
-
29
-
-
0019194038
-
The recognition of maltodextrins by Escherichia coli
-
Ferenci T. The recognition of maltodextrins by Escherichia coli. Eur J Biochem 108: 631-636, 1980.
-
(1980)
Eur J Biochem
, vol.108
, pp. 631-636
-
-
Ferenci, T.1
-
30
-
-
67349185408
-
Ion channels versus ion pumps: The principal difference, in principle
-
Gadsby DC. Ion channels versus ion pumps: the principal difference, in principle. Nat Rev Mol Cell Biol 10: 344-352, 2009.
-
(2009)
Nat Rev Mol Cell Biol
, vol.10
, pp. 344-352
-
-
Gadsby, D.C.1
-
31
-
-
33645307384
-
The ABC protein turned chloride channel whose failure causes cystic fibrosis
-
Gadsby DC, Vergani P, Csanady L. The ABC protein turned chloride channel whose failure causes cystic fibrosis. Nature 440: 477-483, 2006.
-
(2006)
Nature
, vol.440
, pp. 477-483
-
-
Gadsby, D.C.1
Vergani, P.2
Csanady, L.3
-
32
-
-
0034724680
-
Comparison of the functional characteristics of the nucleotide binding domains of multidrug resistance protein 1
-
Gao M, Cui HR, Loe DW, Grant CE, Almquist KC, Cole SP, Deeley RG. Comparison of the functional characteristics of the nucleotide binding domains of multidrug resistance protein 1. J Biol Chem 275: 13098-13108, 2000.
-
(2000)
J Biol Chem
, vol.275
, pp. 13098-13108
-
-
Gao, M.1
Cui, H.R.2
Loe, D.W.3
Grant, C.E.4
Almquist, K.C.5
Cole, S.P.6
Deeley, R.G.7
-
33
-
-
0035682189
-
Overview: ABC transporters and human disease
-
Gottesman MM, Ambudkar SV. Overview: ABC transporters and human disease. J Bioenerg Biomembr 33: 453-458, 2001.
-
(2001)
J Bioenerg Biomembr
, vol.33
, pp. 453-458
-
-
Gottesman, M.M.1
Ambudkar, S.V.2
-
34
-
-
0029117303
-
Conformational states of CFTR associated with channel gating: The role ATP binding and hydrolysis
-
Gunderson KL, Kopito RR. Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis. Cell 82: 231-239, 1995.
-
(1995)
Cell
, vol.82
, pp. 231-239
-
-
Gunderson, K.L.1
Kopito, R.R.2
-
35
-
-
0028070453
-
Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating
-
Gunderson KL, Kopito RR. Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating. J Biol Chem 269: 19349-19353, 1994.
-
(1994)
J Biol Chem
, vol.269
, pp. 19349-19353
-
-
Gunderson, K.L.1
Kopito, R.R.2
-
36
-
-
84860417445
-
ATP hydrolysis at one of the two sites in ABC transporters initiates transport related conformational transitions
-
Gyimesi G, Ramachandran S, Kota P, Dokholyan NV, Sarkadi B, Hegedus T. ATP hydrolysis at one of the two sites in ABC transporters initiates transport related conformational transitions. Biochim Biophys Acta 1808: 2954-2964, 2011.
-
(2011)
Biochim Biophys Acta
, vol.1808
, pp. 2954-2964
-
-
Gyimesi, G.1
Ramachandran, S.2
Kota, P.3
Dokholyan, N.V.4
Sarkadi, B.5
Hegedus, T.6
-
37
-
-
84861310612
-
Crystal structure of a heterodimeric ABC transporter in its inward-facing conformation
-
Hohl M, Briand C, Grutter MG, Seeger MA. Crystal structure of a heterodimeric ABC transporter in its inward-facing conformation. Nature Struct Mol Biol 19: 395-402, 2012.
-
(2012)
Nature Struct Mol Biol
, vol.19
, pp. 395-402
-
-
Hohl, M.1
Briand, C.2
Grutter, M.G.3
Seeger, M.A.4
-
38
-
-
33947154878
-
Structure of an ABC transporter in complex with its binding protein
-
Hollenstein K, Frei DC, Locher KP. Structure of an ABC transporter in complex with its binding protein. Nature 446: 213-216, 2007.
-
(2007)
Nature
, vol.446
, pp. 213-216
-
-
Hollenstein, K.1
Frei, D.C.2
Locher, K.P.3
-
39
-
-
0034617097
-
Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1
-
Hou Y, Cui L, Riordan JR, Chang X. Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1. J Biol Chem 275: 20280-20287, 2000.
-
(2000)
J Biol Chem
, vol.275
, pp. 20280-20287
-
-
Hou, Y.1
Cui, L.2
Riordan, J.R.3
Chang, X.4
-
40
-
-
34548671159
-
Asymmetry in the structure of the ABC transporter-binding protein complex BtuCD-BtuF
-
Hvorup RN, Goetz BA, Niederer M, Hollenstein K, Perozo E, Locher KP. Asymmetry in the structure of the ABC transporter-binding protein complex BtuCD-BtuF. Science 317: 1387-1390, 2007.
-
(2007)
Science
, vol.317
, pp. 1387-1390
-
-
Hvorup, R.N.1
Goetz, B.A.2
Niederer, M.3
Hollenstein, K.4
Perozo, E.5
Locher, K.P.6
-
41
-
-
0028340159
-
Regulation of the gating of cystic fibrosis transmembrane conductance regulator Cl channels by phosphorylation and ATP hydrolysis
-
Hwang TC, Nagel G, Nairn AC, Gadsby DC. Regulation of the gating of cystic fibrosis transmembrane conductance regulator Cl channels by phosphorylation and ATP hydrolysis. Proc Natl Acad Sci USA 91: 4698-4702, 1994.
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, pp. 4698-4702
-
-
Hwang, T.C.1
Nagel, G.2
Nairn, A.C.3
Gadsby, D.C.4
-
42
-
-
65749102092
-
Gating of the CFTR Cl channel by ATP-driven nucleotide-binding domain dimerisation
-
Hwang TC, Sheppard DN. Gating of the CFTR Cl channel by ATP-driven nucleotide-binding domain dimerisation. J Physiol 587: 2151-2161, 2009.
-
(2009)
J Physiol
, vol.587
, pp. 2151-2161
-
-
Hwang, T.C.1
Sheppard, D.N.2
-
43
-
-
0030668944
-
Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis
-
Ishihara H, Welsh MJ. Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis. Am J Physiol Cell Physiol 273: C1278-C1289, 1997.
-
(1997)
Am J Physiol Cell Physiol
, vol.273
-
-
Ishihara, H.1
Welsh, M.J.2
-
44
-
-
33947284958
-
Dynamics and function in a bacterial ABC transporter: Simulation studies of the BtuCDF system and its components
-
Ivetac A, Campbell JD, Sansom MS. Dynamics and function in a bacterial ABC transporter: simulation studies of the BtuCDF system and its components. Biochemistry 46: 2767-2778, 2007.
-
(2007)
Biochemistry
, vol.46
, pp. 2767-2778
-
-
Ivetac, A.1
Campbell, J.D.2
Sansom, M.S.3
-
45
-
-
0022628717
-
Kinetics of unliganded acetylcholine receptor channel gating
-
Jackson MB. Kinetics of unliganded acetylcholine receptor channel gating. Biophys J 49: 663-672, 1986.
-
(1986)
Biophys J
, vol.49
, pp. 663-672
-
-
Jackson, M.B.1
-
46
-
-
84861167188
-
Identification of a novel post-hydrolytic state in CFTR gating
-
Jih KY, Sohma Y, Li M, Hwang TC. Identification of a novel post-hydrolytic state in CFTR gating. J Gen Physiol 139: 359-370, 2012.
-
(2012)
J Gen Physiol
, vol.139
, pp. 359-370
-
-
Jih, K.Y.1
Sohma, Y.2
Li, M.3
Hwang, T.C.4
-
47
-
-
84870795585
-
Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation
-
Jih KY, Sohma Y, Hwang TC. Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation. J Gen Physiol 140: 347-359, 2012.
-
(2012)
J Gen Physiol
, vol.140
, pp. 347-359
-
-
Jih, K.Y.1
Sohma, Y.2
Hwang, T.C.3
-
48
-
-
60549097035
-
Alternating access in maltose transporter mediated by rigid-body rotations
-
Khare D, Oldham ML, Orelle C, Davidson AL, Chen J. Alternating access in maltose transporter mediated by rigid-body rotations. Mol Cell 33: 528-536, 2009.
-
(2009)
Mol Cell
, vol.33
, pp. 528-536
-
-
Khare, D.1
Oldham, M.L.2
Orelle, C.3
Davidson, A.L.4
Chen, J.5
-
49
-
-
10744230777
-
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
-
Lewis HA, Buchanan SG, Burley SK, Conners K, Dickey M, Dorwart M, Fowler R, Gao X, Guggino WB, Hendrickson WA, Hunt JF, Kearins MC, Lorimer D, Maloney PC, Post KW, Rajashankar KR, Rutter ME, Sauder JM, Shriver S, Thibodeau PH, Thomas PJ, 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.
-
(2004)
EMBO J
, vol.23
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
Conners, K.4
Dickey, M.5
Dorwart, M.6
Fowler, R.7
Gao, X.8
Guggino, W.B.9
Hendrickson, W.A.10
Hunt, J.F.11
Kearins, M.C.12
Lorimer, D.13
Maloney, P.C.14
Post, K.W.15
Rajashankar, K.R.16
Rutter, M.E.17
Sauder, J.M.18
Shriver, S.19
Thibodeau, P.H.20
Thomas, P.J.21
Zhang, M.22
Zhao, X.23
Emtage, S.24
more..
-
51
-
-
0037052565
-
The E. coli BtuCD structure: A framework for ABC transporter architecture and mechanism
-
Locher KP, Lee AT, Rees DC. The E. coli BtuCD structure: a framework for ABC transporter architecture and mechanism. Science 296: 1091-1098, 2002.
-
(2002)
Science
, vol.296
, pp. 1091-1098
-
-
Locher, K.P.1
Lee, A.T.2
Rees, D.C.3
-
52
-
-
0033601321
-
ATP binding properties of the nucleotide-binding folds of SUR1
-
Matsuo M, Kioka N, Amachi T, Ueda K. ATP binding properties of the nucleotide-binding folds of SUR1. J Biol Chem 274: 37479-37482,1999.
-
(1999)
J Biol Chem
, vol.274
, pp. 37479-37482
-
-
Matsuo, M.1
Kioka, N.2
Amachi, T.3
Ueda, K.4
-
53
-
-
33750222000
-
In vivo phosphorylation of CFTR promotes formation of a nucleotidebinding domain heterodimer
-
Mense M, Vergani P, White DM, Altberg G, Nairn AC, Gadsby DC. In vivo phosphorylation of CFTR promotes formation of a nucleotidebinding domain heterodimer. EMBO J 25: 4728-4739, 2006.
-
(2006)
EMBO J
, vol.25
, pp. 4728-4739
-
-
Mense, M.1
Vergani, P.2
White, D.M.3
Altberg, G.4
Nairn, A.C.5
Gadsby, D.C.6
-
54
-
-
78651189765
-
On the nature of allosteric transitions: A plausible model
-
Monod J, Wyman J, Changeux JP. On the nature of allosteric transitions: a plausible model. J Mol Biol 12: 88-118, 1965.
-
(1965)
J Mol Biol
, vol.12
, pp. 88-118
-
-
Monod, J.1
Wyman, J.2
Changeux, J.P.3
-
55
-
-
0036843663
-
The significance of molecular slips in transport systems
-
Nelson N, Sacher A, Nelson H. The significance of molecular slips in transport systems. Nature Rev Mol Cell Biol 3: 876-881, 2002.
-
(2002)
Nature Rev Mol Cell Biol
, vol.3
, pp. 876-881
-
-
Nelson, N.1
Sacher, A.2
Nelson, H.3
-
56
-
-
84858633600
-
Cystic fibrosis transmembrane conductance regulator: A molecular model defines the architecture of the anion conduction path and locates a "bottleneck" in the pore
-
Norimatsu Y, Ivetac A, Alexander C, Kirkham J, O'Donnell N, Dawson DC, Sansom MS. Cystic fibrosis transmembrane conductance regulator: a molecular model defines the architecture of the anion conduction path and locates a "bottleneck" in the pore. Biochemistry 51: 2199-2212, 2012.
-
(2012)
Biochemistry
, vol.51
, pp. 2199-2212
-
-
Norimatsu, Y.1
Ivetac, A.2
Alexander, C.3
Kirkham, J.4
O'Donnell, N.5
Dawson, D.C.6
Sansom, M.S.7
-
57
-
-
36549018568
-
Crystal structure of a catalytic intermediate of the maltose transporter
-
Oldham ML, Khare D, Quiocho FA, Davidson AL, Chen J. Crystal structure of a catalytic intermediate of the maltose transporter. Nature 450: 515-521, 2007.
-
(2007)
Nature
, vol.450
, pp. 515-521
-
-
Oldham, M.L.1
Khare, D.2
Quiocho, F.A.3
Davidson, A.L.4
Chen, J.5
-
58
-
-
0035896542
-
Regulation of the cystic fibrosis transmembrane conductance regulator Cl channel by its R domain
-
Ostedgaard LS, Baldursson O, Welsh MJ. Regulation of the cystic fibrosis transmembrane conductance regulator Cl channel by its R domain. J Biol Chem 276: 7689-7692, 2001.
-
(2001)
J Biol Chem
, vol.276
, pp. 7689-7692
-
-
Ostedgaard, L.S.1
Baldursson, O.2
Welsh, M.J.3
-
59
-
-
33846601303
-
An inward-facing conformation of a putative metalchelate-type ABC transporter
-
Pinkett HW, Lee AT, Lum P, Locher KP, Rees DC. An inward-facing conformation of a putative metalchelate-type ABC transporter. Science 315: 373-377, 2007.
-
(2007)
Science
, vol.315
, pp. 373-377
-
-
Pinkett, H.W.1
Lee, A.T.2
Lum, P.3
Locher, K.P.4
Rees, D.C.5
-
60
-
-
80054724207
-
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant
-
Qian F, El Hiani Y, Linsdell P. Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant. Pflügers Arch 462: 559-571, 2011.
-
(2011)
Pflügers Arch
, vol.462
, pp. 559-571
-
-
Qian, F.1
El Hiani, Y.2
Linsdell, P.3
-
61
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordonez C, Elborn JS. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 365: 1663-1672, 2011.
-
(2011)
N Engl J Med
, vol.365
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
Tullis, E.4
Bell, S.C.5
Drevinek, P.6
Griese, M.7
McKone, E.F.8
Wainwright, C.E.9
Konstan, M.W.10
Moss, R.11
Ratjen, F.12
Sermet-Gaudelus, I.13
Rowe, S.M.14
Dong, Q.15
Rodriguez, S.16
Yen, K.17
Ordonez, C.18
Elborn, J.S.19
-
63
-
-
0025270712
-
Steady-state coupling of ion-channel conformations to a transmembrane ion gradient
-
Richard EA, Miller C. Steady-state coupling of ion-channel conformations to a transmembrane ion gradient. Science 247: 1208-1210, 1990.
-
(1990)
Science
, vol.247
, pp. 1208-1210
-
-
Richard, E.A.1
Miller, C.2
-
64
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL, et al. 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.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
66
-
-
40949121607
-
ABC multidrug transporters: Structure, function and role in chemoresistance
-
Sharom FJ. ABC multidrug transporters: structure, function and role in chemoresistance. Pharmacogenomics 9: 105-127, 2008.
-
(2008)
Pharmacogenomics
, vol.9
, pp. 105-127
-
-
Sharom, F.J.1
-
67
-
-
79951822357
-
Cystic fibrosis: CFTR correctors to the rescue
-
Sheppard DN. Cystic fibrosis: CFTR correctors to the rescue. Chem Biol 18: 145-147, 2011.
-
(2011)
Chem Biol
, vol.18
, pp. 145-147
-
-
Sheppard, D.N.1
-
68
-
-
33846521753
-
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
-
Stratford FL, Ramjeesingh M, Cheung JC, Huan LJ, Bear CE. 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.
-
(2007)
Biochem J
, vol.401
, pp. 581-586
-
-
Stratford, F.L.1
Ramjeesingh, M.2
Cheung, J.C.3
Huan, L.J.4
Bear, C.E.5
-
69
-
-
79958076650
-
Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating
-
Szollosi A, Muallem DR, Csanady L, Vergani P. Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating. J Gen Physiol 137: 549-562, 2011.
-
(2011)
J Gen Physiol
, vol.137
, pp. 549-562
-
-
Szollosi, A.1
Muallem, D.R.2
Csanady, L.3
Vergani, P.4
-
70
-
-
77951706563
-
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel
-
Tsai MF, Li M, Hwang TC. Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel. J Gen Physiol 135: 399-414, 2010.
-
(2010)
J Gen Physiol
, vol.135
, pp. 399-414
-
-
Tsai, M.F.1
Li, M.2
Hwang, T.C.3
-
71
-
-
64549132504
-
State-dependent modulation of CFTR gating by pyrophosphate
-
Tsai MF, Shimizu H, Sohma Y, Li M, Hwang TC. State-dependent modulation of CFTR gating by pyrophosphate. J Gen Physiol 133: 405-419, 2009.
-
(2009)
J Gen Physiol
, vol.133
, pp. 405-419
-
-
Tsai, M.F.1
Shimizu, H.2
Sohma, Y.3
Li, M.4
Hwang, T.C.5
-
72
-
-
0033574068
-
Cooperative binding of ATP and MgADP in the sulfonylurea receptor is modulated by glibenclamide
-
Ueda K, Komine J, Matsuo M, Seino S, Amachi T. Cooperative binding of ATP and MgADP in the sulfonylurea receptor is modulated by glibenclamide. Proc Natl Acad Sci USA 96: 1268-1272, 1999.
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 1268-1272
-
-
Ueda, K.1
Komine, J.2
Matsuo, M.3
Seino, S.4
Amachi, T.5
-
73
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci USA 106: 18825-18830, 2009.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 18825-18830
-
-
van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
Burton, B.4
Cao, D.5
Neuberger, T.6
Turnbull, A.7
Singh, A.8
Joubran, J.9
Hazlewood, A.10
Zhou, J.11
McCartney, J.12
Arumugam, V.13
Decker, C.14
Yang, J.15
Young, C.16
Olson, E.R.17
Wine, J.J.18
Frizzell, R.A.19
Ashlock, M.20
Negulescu, P.21
more..
-
74
-
-
33744831154
-
Rescue of Delta- F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F, Straley KS, Cao D, Gonzalez J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, Neuberger T, Olson E, Panchenko V, Rader J, Singh A, Stack JH, Tung R, Grootenhuis PD, Negulescu P. Rescue of Delta- F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 290: L1117-L1130, 2006.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
-
-
van Goor, F.1
Straley, K.S.2
Cao, D.3
Gonzalez, J.4
Hadida, S.5
Hazlewood, A.6
Joubran, J.7
Knapp, T.8
Makings, L.R.9
Miller, M.10
Neuberger, T.11
Olson, E.12
Panchenko, V.13
Rader, J.14
Singh, A.15
Stack, J.H.16
Tung, R.17
Grootenhuis, P.D.18
Negulescu, P.19
-
75
-
-
14544300522
-
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains
-
Vergani P, Lockless SW, Nairn AC, Gadsby DC. CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature 433: 876-880, 2005.
-
(2005)
Nature
, vol.433
, pp. 876-880
-
-
Vergani, P.1
Lockless, S.W.2
Nairn, A.C.3
Gadsby, D.C.4
-
76
-
-
0037246832
-
On the mechanism of MgATP-dependent gating of CFTR Cl channels
-
Vergani P, Nairn AC, Gadsby DC. On the mechanism of MgATP-dependent gating of CFTR Cl channels. J Gen Physiol 121: 17-36, 2003.
-
(2003)
J Gen Physiol
, vol.121
, pp. 17-36
-
-
Vergani, P.1
Nairn, A.C.2
Gadsby, D.C.3
-
77
-
-
33745120825
-
CFTR chloride channel drug discovery: Inhibitors as antidiarrheals and activators for therapy of cystic fibrosis
-
Verkman AS, Lukacs GL, Galietta LJ. CFTR chloride channel drug discovery: inhibitors as antidiarrheals and activators for therapy of cystic fibrosis. Curr Pharmaceut Design 12: 2235-2247, 2006.
-
(2006)
Curr Pharmaceut Design
, vol.12
, pp. 2235-2247
-
-
Verkman, A.S.1
Lukacs, G.L.2
Galietta, L.J.3
-
78
-
-
33947543364
-
Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains
-
Wang W, Bernard K, Li G, Kirk KL. Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains. J Biol Chem 282: 4533-4544, 2007.
-
(2007)
J Biol Chem
, vol.282
, pp. 4533-4544
-
-
Wang, W.1
Bernard, K.2
Li, G.3
Kirk, K.L.4
-
79
-
-
79961146667
-
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore
-
Wang W, El Hiani Y, Linsdell P. Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Gen Physiol 138: 165-178, 2011.
-
(2011)
J Gen Physiol
, vol.138
, pp. 165-178
-
-
Wang, W.1
El Hiani, Y.2
Linsdell, P.3
-
80
-
-
82755162382
-
Thermally unstable gating of the most common cystic fibrosis mutant channel (DeltaF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops
-
Wang W, Okeyo GO, Tao B, Hong JS, Kirk KL. Thermally unstable gating of the most common cystic fibrosis mutant channel (DeltaF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops. J Biol Chem 286: 41937-41948, 2011.
-
(2011)
J Biol Chem
, vol.286
, pp. 41937-41948
-
-
Wang, W.1
Okeyo, G.O.2
Tao, B.3
Hong, J.S.4
Kirk, K.L.5
-
81
-
-
77649256457
-
ATP-independent CFTR channel gating and allosteric modulation by phosphorylation
-
Wang W, Wu J, Bernard K, Li G, Wang G, Bevensee MO, Kirk KL. ATP-independent CFTR channel gating and allosteric modulation by phosphorylation. Proc Natl Acad Sci USA 107: 3888-3893, 2010.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 3888-3893
-
-
Wang, W.1
Wu, J.2
Bernard, K.3
Li, G.4
Wang, G.5
Bevensee, M.O.6
Kirk, K.L.7
-
82
-
-
37649004412
-
Flexibility in the ABC transporter MsbA: Alternating access with a twist
-
Ward A, Reyes CL, Yu J, Roth CB, Chang G. Flexibility in the ABC transporter MsbA: alternating access with a twist. Proc Natl Acad Sci USA 104: 19005-19010, 2007.
-
(2007)
Proc Natl Acad Sci USA
, vol.104
, pp. 19005-19010
-
-
Ward, A.1
Reyes, C.L.2
Yu, J.3
Roth, C.B.4
Chang, G.5
-
83
-
-
84862776940
-
Ivacaftor potentiation of multiple CFTR channels with gating mutations
-
Yu H, Burton B, Huang CJ, Worley J, Cao D, Johnson JP Jr, Urrutia A, Joubran J, Seepersaud S, Sussky K, Hoffman BJ, Van Goor F. Ivacaftor potentiation of multiple CFTR channels with gating mutations. J Cystic Fibros 11: 237-245, 2012.
-
(2012)
J Cystic Fibros
, vol.11
, pp. 237-245
-
-
Yu, H.1
Burton, B.2
Huang, C.J.3
Worley, J.4
Cao, D.5
Johnson Jr., J.P.6
Urrutia, A.7
Joubran, J.8
Seepersaud, S.9
Sussky, K.10
Hoffman, B.J.11
van Goor, F.12
-
84
-
-
0032954806
-
Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme
-
Zeltwanger S, Wang F, Wang GT, Gillis KD, Hwang TC. Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme. J Gen Physiol 113: 541-554, 1999.
-
(1999)
J Gen Physiol
, vol.113
, pp. 541-554
-
-
Zeltwanger, S.1
Wang, F.2
Wang, G.T.3
Gillis, K.D.4
Hwang, T.C.5
|