Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants

The dual phosphodiesterase 3 and 4 inhibitor RPL554 stimulates CFTR and ciliary beating in primary cultures of bronchial epithelia

(2016) Mark J. Turner et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function

(2016) Kavisha Arora et al.   CURRENT DRUG TARGETS

How Phosphorylation and ATPase Activity Regulate Anion Flux though the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

(2016) Matthias Zwick et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Correctors of mutant CFTR enhance subcortical cAMP–PKA signaling through modulating ezrin phosphorylation and cytoskeleton organization

(2016) Anna C. Abbattiscianni et al.   JOURNAL OF CELL SCIENCE

EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1

(2016) Miguel J. Lobo et al.   JOURNAL OF CELL SCIENCE

Characterization and small-molecule stabilization of the multisite tandem binding between 14-3-3 and the R domain of CFTR

(2016) Loes M. Stevers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2016) Csaba Mihályi et al.   eLife

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

Compartmentalized Accumulation of cAMP near Complexes of Multidrug Resistance Protein 4 (MRP4) and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Contributes to Drug-induced Diarrhea

(2015) Changsuk Moon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop

(2015) Annette Ehrhardt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint

(2015) Cláudia A. Loureiro et al.   Science Signaling

A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint

(2015) Cláudia A. Loureiro et al.   Science Signaling

VIP regulates CFTR membrane expression and function in Calu-3 cells by increasing its interaction with NHERF1 and P-ERM in a VPAC1- and PKCε-dependent manner

(2014) Walaa Alshafie et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation

(2014) Stan Pasyk et al.   PROTEOMICS

Revertants, Low Temperature, and Correctors Reveal the Mechanism of F508del-CFTR Rescue by VX-809 and Suggest Multiple Agents for Full Correction

(2013) Carlos M. Farinha et al.   CHEMISTRY & BIOLOGY

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment

(2013) J. P. Holleran et al.   JOURNAL OF CELL SCIENCE

The secret life of CFTR as a calcium-activated chloride channel

(2013) Arnaud Billet et al.   JOURNAL OF PHYSIOLOGY-LONDON

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Allosteric Coupling between the Intracellular Coupling Helix 4 and Regulatory Sites of the First Nucleotide-binding Domain of CFTR

(2013) Jennifer E. Dawson et al.   PLoS One

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

Correction of Both NBD1 Energetics and Domain Interface Is Required to Restore ΔF508 CFTR Folding and Function

(2012) Wael M. Rabeh et al.   CELL

Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein

(2012) Lihua He et al.   FASEB JOURNAL

Conformational Changes Relevant to Channel Activity and Folding within the first Nucleotide Binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator

(2012) Rhea P. Hudson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity

(2012) S. Monterisi et al.   JOURNAL OF CELL SCIENCE

Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis

(2012) Xiubin Liang et al.   MOLECULAR BIOLOGY OF THE CELL

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

Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications

(2011) M. McClure et al.   PROTEIN ENGINEERING DESIGN & SELECTION

CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners

(2010) Chunying Li et al.   Integrative Biology

Regulatory Insertion Removal Restores Maturation, Stability and Function of ΔF508 CFTR

(2010) Andrei A. Aleksandrov et al.   JOURNAL OF MOLECULAR BIOLOGY

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

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

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

Functional Rescue of DeltaF508-CFTR by Peptides Designed to Mimic Sorting Motifs

(2009) Patrick Kim Chiaw et al.   CHEMISTRY & BIOLOGY

NMR evidence for differential phosphorylation-dependent interactions in WT and ΔF508 CFTR

(2009) Voula Kanelis et al.   EMBO JOURNAL

SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia

(2009) Carol A. Bertrand 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

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

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

Na+/H+ Exchanger Regulatory Factor 1 Overexpression-dependent Increase of Cytoskeleton Organization Is Fundamental in the Rescue of F508del Cystic Fibrosis Transmembrane Conductance Regulator in Human Airway CFBE41o- Cells

(2009) Maria Favia et al.   MOLECULAR BIOLOGY OF THE CELL

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

Molecular basis for the ATPase activity of CFTR

(2008) Joanne C. Cheung et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

The intact CFTR protein mediates ATPase rather than adenylate kinase activity

(2008) Mohabir Ramjeesingh et al.   BIOCHEMICAL JOURNAL

Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease

(2008) Stan Pasyk et al.   BIOCHEMICAL JOURNAL

Atomic model of human cystic fibrosis transmembrane conductance regulator: Membrane-spanning domains and coupling interfaces

(2008) J.-P. Mornon et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

Processing Mutations Disrupt Interactions between the Nucleotide Binding and Transmembrane Domains of P-glycoprotein and the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

(2008) Tip W. Loo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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