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

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

Identification of a novel post-hydrolytic state in CFTR gating

(2012) Kang-Yang Jih et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

(2012) Kang-Yang Jih et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

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

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

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

(2010) Yonghong Bai 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

State-dependent modulation of CFTR gating by pyrophosphate

(2009) Ming-Feng Tsai et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

(2009) Tzyh-Chang Hwang et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

The intact CFTR protein mediates ATPase rather than adenylate kinase activity

(2008) Mohabir Ramjeesingh et al.   BIOCHEMICAL JOURNAL