Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O1 state

Experimental design and analysis and their reporting II: updated and simplified guidance for authors and peer reviewers

(2018) Michael J Curtis et al.   BRITISH JOURNAL OF PHARMACOLOGY

Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor

(2018) Leona Bessonova et al.   THORAX

The IUPHAR/BPS Guide to PHARMACOLOGY in 2018: updates and expansion to encompass the new guide to IMMUNOPHARMACOLOGY

(2017) Simon D Harding et al.   NUCLEIC ACIDS RESEARCH

Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator

(2016) Zhe Zhang et al.   CELL

Experimental design and analysis and their reporting: new guidance for publication in BJP

(2015) Michael J Curtis et al.   BRITISH JOURNAL OF PHARMACOLOGY

Timing of CFTR Pore Opening and Structure of Its Transition State

(2015) Ben Sorum et al.   CELL

Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels

(2015) Luiz A. Poletto Chaves et al.   JOURNAL OF GENERAL PHYSIOLOGY

Impact of the F508del mutation on ovine CFTR, a Cl−channel with enhanced conductance and ATP-dependent gating

(2015) Zhiwei Cai et al.   JOURNAL OF PHYSIOLOGY-LONDON

Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del-CFTR channels

(2014) Z. Kopeikin et al.   Journal of Cystic Fibrosis

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

(2014) Wen-Ying Lin et al.   JOURNAL OF GENERAL PHYSIOLOGY

Structure–activity analysis of a CFTR channel potentiator: Distinct molecular parts underlie dual gating effects

(2014) László Csanády et al.   JOURNAL OF GENERAL PHYSIOLOGY

Modulation of CFTR gating by permeant ions

(2014) Han-I Yeh et al.   JOURNAL OF GENERAL PHYSIOLOGY

Some gating potentiators, including VX-770, diminish  F508-CFTR functional expression

(2014) G. Veit et al.   Science Translational Medicine

Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with aG551DMutation

(2013) Jane C. Davies et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function

(2013) Fredrick Van Goor et al.   Journal of Cystic Fibrosis

Conformational changes in the catalytically inactive nucleotide-binding site of CFTR

(2013) László Csanády et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

(2013) Kang-Yang Jih et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2012) Paul D. W. Eckford et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ivacaftor potentiation of multiple CFTR channels with gating mutations

(2012) Haihui Yu et al.   Journal of Cystic Fibrosis

pHTomato, a red, genetically encoded indicator that enables multiplex interrogation of synaptic activity

(2012) Yulong Li et al.   NATURE NEUROSCIENCE

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

(2011) Kang-Yang Jih et al.   JOURNAL OF PHYSIOLOGY-LONDON

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

(2010) Haruna Miki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Cystic Fibrosis-causing Mutation ΔF508 Affects Multiple Steps in Cystic Fibrosis Transmembrane Conductance Regulator Biogenesis

(2010) Patrick H. Thibodeau et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2010) Ming-Feng Tsai et al.   JOURNAL OF GENERAL PHYSIOLOGY

Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation

(2010) Frank J. Accurso et al.   NEW ENGLAND JOURNAL OF MEDICINE

Strict coupling between CFTR's catalytic cycle and gating of its Cl- ion pore revealed by distributions of open channel burst durations

(2009) L. Csanady et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA