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Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle

Proceedings of the National Academy of Sciences - PNAS, 2013-03, Vol.110 (11), p.4404-4409

2013

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Positional effects of premature termination codons on the biochemical and biophysical properties of CFTR

The Journal of physiology, 2020-02, Vol.598 (3), p.517-541

2020

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On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator

The Journal of physiology, 2016-06, Vol.594 (12), p.3227-3244

2016

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Gating of the CFTR Cl− channel by ATP‐driven nucleotide‐binding domain dimerisation

The Journal of physiology, 2009-05, Vol.587 (10), p.2151-2161

2009

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Identifying the molecular target sites for CFTR potentiators GLPG1837 and VX-770

The Journal of general physiology, 2019-07, Vol.151 (7), p.912-928

2019

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Localizing a gate in CFTR

Proceedings of the National Academy of Sciences - PNAS, 2015-02, Vol.112 (8), p.2461-2466

2015

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A common mechanism for CFTR potentiators

The Journal of general physiology, 2017-12, Vol.149 (12), p.1105-1118

2017

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Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP‐binding site

The Journal of physiology, 2021-10, Vol.599 (20), p.4625-4642

2021

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Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis

The Journal of physiology, 2019-01, Vol.597 (2), p.543-560

2019

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Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate

Molecular pharmacology, 2016-09, Vol.90 (3), p.275-285

2016

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Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations

PloS one, 2018-12, Vol.13 (12), p.e0209862-e0209862

2018

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Modulation of CFTR gating by permeant ions

The Journal of general physiology, 2015-01, Vol.145 (1), p.47-60

2015

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CLC-0 and CFTR: Chloride Channels Evolved From Transporters

Physiological reviews, 2008-04, Vol.88 (2), p.351-387

2008

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Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7)

The Journal of general physiology, 2011-11, Vol.138 (5), p.495-507

2011

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Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation

The Journal of general physiology, 2010-09, Vol.136 (3), p.293-309

2010

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The most common cystic fibrosis‐associated mutation destabilizes the dimeric state of the nucleotide‐binding domains of CFTR

The Journal of physiology, 2011-06, Vol.589 (11), p.2719-2731

2011

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Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel

The Journal of general physiology, 2010-05, Vol.135 (5), p.399-414

2010

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Nonequilibrium gating of CFTR on an equilibrium theme

Physiology (Bethesda, Md.), 2012-12, Vol.27 (6), p.351-361

2012

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A single amino acid substitution in CFTR converts ATP to an inhibitory ligand

The Journal of general physiology, 2014-10, Vol.144 (4), p.311-320

2014

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G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects

The Journal of general physiology, 2007-04, Vol.129 (4), p.285-298

2007

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Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation

The Journal of general physiology, 2012-10, Vol.140 (4), p.347-359

2012

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On the mechanism of CFTR inhibition by a thiazolidinone derivative

The Journal of general physiology, 2010-12, Vol.136 (6), p.659-671

2010

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Generation of human induced pluripotent stem cells from cystic fibrosis patient carrying nonsense mutation (p.S308X) in CFTR gene

Stem cell research, 2022-04, Vol.60, p.102683-102683, Article 102683

2022

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Potentiation of Disease-associated Cystic Fibrosis Transmembrane Conductance Regulator Mutants by Hydrolyzable ATP Analogs

The Journal of biological chemistry, 2010-06, Vol.285 (26), p.19967-19975

2010

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Identification and Characterization of Novel CFTR Potentiators

Frontiers in pharmacology, 2018-10, Vol.9, p.1221-1221

2018

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Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle

Positional effects of premature termination codons on the biochemical and biophysical properties of CFTR

On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator

Gating of the CFTR Cl− channel by ATP‐driven nucleotide‐binding domain dimerisation

Identifying the molecular target sites for CFTR potentiators GLPG1837 and VX-770

Localizing a gate in CFTR

A common mechanism for CFTR potentiators

Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP‐binding site

Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis

Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate

Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations

Modulation of CFTR gating by permeant ions

CLC-0 and CFTR: Chloride Channels Evolved From Transporters

Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7)

Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation

The most common cystic fibrosis‐associated mutation destabilizes the dimeric state of the nucleotide‐binding domains of CFTR

Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel

Nonequilibrium gating of CFTR on an equilibrium theme

A single amino acid substitution in CFTR converts ATP to an inhibitory ligand

G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects

Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation

On the mechanism of CFTR inhibition by a thiazolidinone derivative

Generation of human induced pluripotent stem cells from cystic fibrosis patient carrying nonsense mutation (p.S308X) in CFTR gene

Potentiation of Disease-associated Cystic Fibrosis Transmembrane Conductance Regulator Mutants by Hydrolyzable ATP Analogs

Identification and Characterization of Novel CFTR Potentiators

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