Structure, Gating, and Regulation of the CFTR Anion Channel

Structure of Transmembrane Helix 8 and Possible Membrane Defects in CFTR

(2018) Valentina Corradi et al.   BIOPHYSICAL JOURNAL

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

(2018) Emily Langron et al.   BRITISH JOURNAL OF PHARMACOLOGY

A common mechanism for CFTR potentiators

(2017) Han-I Yeh et al.   JOURNAL OF GENERAL PHYSIOLOGY

Electrostatic tuning of the pre- and post-hydrolytic open states in CFTR

(2017) Jingyao Zhang et al.   JOURNAL OF GENERAL PHYSIOLOGY

High-Potency Phenylquinoxalinone Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activators

(2017) Jung-Ho Son et al.   JOURNAL OF MEDICINAL CHEMISTRY

Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl−channel

(2017) Jeng-Haur Chen et al.   JOURNAL OF PHYSIOLOGY-LONDON

Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation

(2017) Onur Cil et al.   Translational Research

Asymmetry of movements in CFTR's two ATP sites during pore opening serves their distinct functions

(2017) Ben Sorum et al.   eLife

Exploring conformational equilibria of a heterodimeric ABC transporter

(2017) M Hadi Timachi et al.   eLife

Positioning of extracellular loop 1 affects pore gating of the cystic fibrosis transmembrane conductance regulator

(2016) Daniel T. Infield et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain

(2016) Onofrio Laselva et al.   BIOCHEMICAL PHARMACOLOGY

Anion conductance selectivity mechanism of the CFTR chloride channel

(2016) Paul Linsdell   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator

(2016) Zhe Zhang et al.   CELL

Role of CFTR in epithelial physiology

(2016) Vinciane Saint-Criq et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Spatial positioning of CFTR’s pore-lining residues affirms an asymmetrical contribution of transmembrane segments to the anion permeation pathway

(2016) Xiaolong Gao et al.   JOURNAL OF GENERAL PHYSIOLOGY

Mechanistic diversity in ATP-binding cassette (ABC) transporters

(2016) Kaspar P Locher   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines

(2016) Jinhong Park et al.   PLoS One

Relationships among CFTR expression, HCO3−secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies

(2016) Viral S. Shah et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

(2016) V. S. Shah et al.   SCIENCE

Mechanistic diversity in ATP-binding cassette (ABC) transporters

(2016) Kaspar P Locher   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A single active catalytic site is sufficient to promote transport in P-glycoprotein

(2016) Orsolya Bársony et al.   Scientific Reports

Obligate coupling of CFTR pore opening to tight nucleotide-binding domain dimerization

(2016) Csaba Mihályi et al.   eLife

Progress in therapies for cystic fibrosis

(2016) Kris De Boeck et al.   Lancet Respiratory Medicine

Murine and human CFTR exhibit different sensitivities to CFTR potentiators

(2015) Guiying Cui et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

The Fifth Transmembrane Segment of Cystic Fibrosis Transmembrane Conductance Regulator Contributes to Its Anion Permeation Pathway

(2015) Jingyao Zhang et al.   BIOCHEMISTRY

Interactions between permeant and blocking anions inside the CFTR chloride channel pore

(2015) Paul Linsdell   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Timing of CFTR Pore Opening and Structure of Its Transition State

(2015) Ben Sorum et al.   CELL

Cytoplasmic pathway followed by chloride ions to enter the CFTR channel pore

(2015) Yassine El Hiani et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Potential sites of CFTR activation by tyrosine kinases

(2015) Arnaud Billet et al.   Channels

Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation

(2015) Arnaud Billet et al.   FASEB JOURNAL

Functional Architecture of the Cytoplasmic Entrance to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore

(2015) Yassine El Hiani et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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 Primary Human Airway Epithelia

(2015) Qian Dong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

(2015) Valentina Corradi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Secretory diarrhoea: mechanisms and emerging therapies

(2015) Jay R. Thiagarajah et al.   Nature Reviews Gastroenterology & Hepatology

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Localizing a gate in CFTR

(2015) Xiaolong Gao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells

(2014) Maria Favia et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics

(2014) Jean-Paul Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

An Electrostatic Interaction at the Tetrahelix Bundle Promotes Phosphorylation-dependent Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Opening

(2014) Wei Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Conserved Allosteric Hot Spots in the Transmembrane Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channels and Multidrug Resistance Protein (MRP) Pumps

(2014) Shipeng Wei et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Three charged amino acids in extracellular loop 1 are involved in maintaining the outer pore architecture of CFTR

(2014) Guiying Cui et al.   JOURNAL OF GENERAL PHYSIOLOGY

Catalyst-like modulation of transition states for CFTR channel opening and closing: New stimulation strategy exploits nonequilibrium gating

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

Structure of an antibacterial peptide ATP-binding cassette transporter in a novel outward occluded state

(2014) H. G. Choudhury et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

(2014) D. M. Cholon et al.   Science Translational Medicine

Cysteine Scanning of CFTR’s First Transmembrane Segment Reveals Its Plausible Roles in Gating and Permeation

(2013) Xiaolong Gao et al.   BIOPHYSICAL JOURNAL

Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain

(2013) Aswathy Sebastian et al.   GENE

Converting Nonhydrolyzable Nucleotides to Strong Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Agonists by Gain of Function (GOF) Mutations

(2013) George Okeyo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Reinterpreting the Action of ATP Analogs on KATPChannels

(2013) David Ortiz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Increased apical Na+permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport

(2013) Donal L. O’Donoghue et al.   JOURNAL OF PHYSIOLOGY-LONDON

Relative contribution of different transmembrane segments to the CFTR chloride channel pore

(2013) Wuyang Wang et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF 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

Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions

(2013) Z. Bozoky et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structures of ABCB10, a human ATP-binding cassette transporter in apo- and nucleotide-bound states

(2013) C. A. Shintre 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

Demonstration of Phosphoryl Group Transfer Indicates That the ATP-binding Cassette (ABC) Transporter Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Exhibits Adenylate Kinase Activity

(2012) Christoph O. Randak 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

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

Crystal structure of a heterodimeric ABC transporter in its inward-facing conformation

(2012) Michael Hohl et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Molecular Mechanism of Pancreatic and Salivary Gland Fluid and HCO3− Secretion

(2012) Min Goo Lee et al.   PHYSIOLOGICAL REVIEWS

A Pharmacologic Approach to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Smoking Related Lung Disease

(2012) Peter A. Sloane et al.   PLoS One

Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung

(2011) Ahmed Lazrak et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

A Unified View of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gating: Combining the Allosterism of a Ligand-gated Channel with the Enzymatic Activity of an ATP-binding Cassette (ABC) Transporter

(2011) Kevin L. Kirk et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Thermally Unstable Gating of the Most Common Cystic Fibrosis Mutant Channel (ΔF508)

(2011) Wei Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore

(2011) Wuyang Wang et al.   JOURNAL OF GENERAL PHYSIOLOGY

Mutant cycles at CFTR’s non-canonical ATP-binding site support little interface separation during gating

(2011) Andras Szollosi et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

(2011) Yonghong Bai et al.   JOURNAL OF GENERAL PHYSIOLOGY

Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant

(2011) Feng Qian et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Differential contribution of TM6 and TM12 to the pore of CFTR identified by three sulfonylurea-based blockers

(2011) Guiying Cui et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance

(2011) O. A. Itani et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Snapshots of the maltose transporter during ATP hydrolysis

(2011) M. L. Oldham et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

(2011) Gergely L. Lukacs et al.   TRENDS IN MOLECULAR MEDICINE

Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia

(2010) Jeng-Haur Chen et al.   CELL

Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl−]i and Its Role in Pancreatic Bicarbonate Secretion

(2010) Hyun Woo Park et al.   GASTROENTEROLOGY

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 Transmembrane Conductance Regulator Impedes Proteolytic Stimulation of the Epithelial Na+Channel

(2010) Martina Gentzsch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterization of an Asymmetric Occluded State of P-glycoprotein with Two Bound Nucleotides

(2010) Alena Siarheyeva et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Changes in Accessibility of Cytoplasmic Substances to the Pore Associated with Activation of the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel

(2010) Yassine El Hiani et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Involvement of F1296 and N1303 of CFTR in induced-fit conformational change in response to ATP binding at NBD2

(2010) Andras Szollosi et al.   JOURNAL OF GENERAL PHYSIOLOGY

On the mechanism of CFTR inhibition by a thiazolidinone derivative

(2010) Zoia Kopeikin et al.   JOURNAL OF GENERAL PHYSIOLOGY

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

ATP-independent CFTR channel gating and allosteric modulation by phosphorylation

(2010) Wei Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

AMP-activated protein kinase phosphorylation of the R domain inhibits PKA stimulation of CFTR

(2009) J Darwin King et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A

(2009) Tamás Hegedűs et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

The mechanism of ABC transporters: general lessons from structural and functional studies of an antigenic peptide transporter

(2009) Erik Procko et al.   FASEB JOURNAL

Adenylate Kinase and AMP Signaling Networks: Metabolic Monitoring, Signal Communication and Body Energy Sensing

(2009) Petras Dzeja et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

State-dependent modulation of CFTR gating by pyrophosphate

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

Application of rate-equilibrium free energy relationship analysis to nonequilibrium ion channel gating mechanisms

(2009) László Csanády   JOURNAL OF GENERAL PHYSIOLOGY

CFTR Functions as a Bicarbonate Channel in Pancreatic Duct Cells

(2009) Hiroshi Ishiguro et al.   JOURNAL OF GENERAL PHYSIOLOGY

Novel Residues Lining the CFTR Chloride Channel Pore Identified by Functional Modification of Introduced Cysteines

(2009) Mohammad Fatehi et al.   JOURNAL OF MEMBRANE BIOLOGY

Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator

(2009) Andrei A. Aleksandrov et al.   JOURNAL OF PHYSIOLOGY-LONDON

Cystic fibrosis

(2009) Brian P O'Sullivan et al.   LANCET

Ion channels versus ion pumps: the principal difference, in principle

(2009) David C. Gadsby   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

Evidence for phosphorylation of serine 753 in CFTR using a novel metal-ion affinity resin and matrix-assisted laser desorption mass spectrometry

(2009) David C. A. Neville et al.   PROTEIN SCIENCE

Structure of P-Glycoprotein Reveals a Molecular Basis for Poly-Specific Drug Binding

(2009) S. G. Aller et al.   SCIENCE

The intact CFTR protein mediates ATPase rather than adenylate kinase activity

(2008) Mohabir Ramjeesingh et al.   BIOCHEMICAL JOURNAL

Nanomolar CFTR Inhibition by Pore-Occluding Divalent Polyethylene Glycol-Malonic Acid Hydrazides

(2008) N.D. Sonawane et al.   CHEMISTRY & BIOLOGY

Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediate Regulation of Channel Gating

(2008) Lihua He et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanistic Insight into Control of CFTR by AMPK

(2008) Patthara Kongsuphol et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small-Molecule CFTR Inhibitors Slow Cyst Growth in Polycystic Kidney Disease

(2008) B. Yang et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Structure and mechanism of ATP-binding cassette transporters

(2008) K. P Locher   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

(2008) A. W. R. Serohijos et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA