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

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

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

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

Structure-Guided Design of Selective Epac1 and Epac2 Agonists

(2015) Frank Schwede et al.   PLOS BIOLOGY

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

Hydrogen peroxide stimulation of CFTR reveals an Epac-mediated, soluble AC-dependent cAMP amplification pathway common to GPCR signalling

(2014) P Ivonnet et al.   BRITISH JOURNAL OF PHARMACOLOGY

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

Rescuing Mutant CFTR: A Multi-task Approach to a Better Outcome in Treating Cystic Fibrosis

(2013) Margarida D. Amaral et al.   CURRENT PHARMACEUTICAL DESIGN

Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi

(2013) Carlos M. Farinha et al.   FEBS Journal

The inner and outer compartments of mitochondria are sites of distinct cAMP/PKA signaling dynamics

(2013) Konstantinos Lefkimmiatis et al.   JOURNAL OF CELL BIOLOGY

Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling

(2012) Stefania Monterisi et al.   BRITISH JOURNAL OF PHARMACOLOGY

Disabled-2 Protein Facilitates Assembly Polypeptide-2-independent Recruitment of Cystic Fibrosis Transmembrane Conductance Regulator to Endocytic Vesicles in Polarized Human Airway Epithelial Cells

(2012) Kristine M. Cihil 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

NHERF1 and CFTR restore tight junction organisation and function in cystic fibrosis airway epithelial cells: role of ezrin and the RhoA/ROCK pathway

(2012) Stefano Castellani et al.   LABORATORY INVESTIGATION

cAMP regulates DEP domain-mediated binding of the guanine nucleotide exchange factor Epac1 to phosphatidic acid at the plasma membrane

(2012) S. V. Consonni et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Antagonistic Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Cell Surface Expression by Protein Kinases WNK4 and Spleen Tyrosine Kinase

(2011) A. I. Mendes et al.   MOLECULAR AND CELLULAR BIOLOGY

Protein kinase A governs a RhoA–RhoGDI protrusion–retraction pacemaker in migrating cells

(2011) Eugene Tkachenko et al.   NATURE CELL BIOLOGY

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

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation

(2011) J P Clancy et al.   THORAX

Epac: Defining a New Mechanism for cAMP Action

(2010) Martijn Gloerich et al.   Annual Review of Pharmacology and Toxicology

FRET-based direct detection of dynamic protein kinase A activity on the sarcoplasmic reticulum in cardiomyocytes

(2010) Shubai Liu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Spatial Regulation of Cyclic AMP-Epac1 Signaling in Cell Adhesion by ERM Proteins

(2010) M. Gloerich et al.   MOLECULAR AND CELLULAR BIOLOGY

Dynasore inhibits removal of wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) from the plasma membrane

(2009) Andrew Young et al.   BIOCHEMICAL JOURNAL

Epac1 mediates protein kinase A–independent mechanism of forskolin-activated intestinal chloride secretion

(2009) Kazi Mirajul Hoque et al.   JOURNAL OF GENERAL PHYSIOLOGY

Direct Spatial Control of Epac1 by Cyclic AMP

(2009) B. Ponsioen et al.   MOLECULAR AND CELLULAR BIOLOGY

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

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry