From the endoplasmic reticulum to the plasma membrane: mechanisms of CFTR folding and trafficking

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

Monomerization and ER Relocalization of GRASP Is a Requisite for Unconventional Secretion of CFTR

(2016) Jiyoon Kim et al.   TRAFFIC

Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity

(2016) Carlos M. Farinha et al.   Frontiers in Chemistry

Repairing the basic defect in cystic fibrosis - one approach is not enough

(2015) Carlos M. Farinha et al.   FEBS Journal

Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation

(2015) Xiaoyan Gong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Restoration of NBD1 Thermal Stability Is Necessary and Sufficient to Correct ∆F508 CFTR Folding and Assembly

(2015) Lihua He et al.   JOURNAL OF MOLECULAR BIOLOGY

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis

(2015) Sandra Pankow et al.   NATURE

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

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

ΔF508 CFTR Surface Stability Is Regulated by DAB2 and CHIP-Mediated Ubiquitination in Post-Endocytic Compartments

(2015) Lianwu Fu et al.   PLoS One

Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells

(2015) Bruce A. Stanton et al.   PLoS One

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

LMTK2-mediated Phosphorylation Regulates CFTR Endocytosis in Human Airway Epithelial Cells

(2014) Simão Luz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Regulatory crosstalk within the mammalian unfolded protein response

(2013) Joseph W. Brewer   CELLULAR AND MOLECULAR LIFE SCIENCES

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

Molecular Motors and Apical CFTR Traffic in Epithelia

(2013) Dmitri Kravtsov et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Endoplasmic Reticulum Protein Quality Control Is Determined by Cooperative Interactions between Hsp/c70 Protein and the CHIP E3 Ligase

(2013) Yoshihiro Matsumura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment

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

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

(2013) Tsukasa Okiyoneda et al.   Nature Chemical Biology

Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells

(2013) Steven M. Snodgrass et al.   PLoS One

HGF Stimulation of Rac1 Signaling Enhances Pharmacological Correction of the Most Prevalent Cystic Fibrosis Mutant F508del-CFTR

(2012) Sónia Moniz et al.   ACS Chemical Biology

Nedd4–2 does not regulate wt-CFTR in human airway epithelial cells

(2012) Katja Koeppen et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

BAG-1 Stabilizes Mutant F508del-CFTR in a Ubiquitin-Like-Domain-Dependent Manner

(2012) Filipa Mendes et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

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

(2012) Lihua He et al.   FASEB JOURNAL

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

FK506 Binding Protein 8 Peptidylprolyl Isomerase Activity Manages a Late Stage of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Folding and Stability

(2012) Darren M. Hutt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Atypical Activation of the Unfolded Protein Response in Cystic Fibrosis Airway Cells Contributes to p38 MAPK-Mediated Innate Immune Responses

(2012) C. J. Blohmke et al.   JOURNAL OF IMMUNOLOGY

Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier–dependent pathway

(2012) Annette Ahner et al.   MOLECULAR BIOLOGY OF THE CELL

A Chaperone Trap Contributes to the Onset of Cystic Fibrosis

(2012) Judith A. Coppinger et al.   PLoS One

Mechanisms of CFTR Folding at the Endoplasmic Reticulum

(2012) Soo Jung Kim et al.   Frontiers in Pharmacology

Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator

(2011) Lianwu Fu et al.   BIOCHEMICAL JOURNAL

Rescue of ΔF508-CFTR Trafficking via a GRASP-Dependent Unconventional Secretion Pathway

(2011) Heon Yung Gee et al.   CELL

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

Contribution of Casein Kinase 2 and Spleen Tyrosine Kinase to CFTR Trafficking and Protein Kinase A-Induced Activity

(2011) S. Luz et al.   MOLECULAR AND CELLULAR BIOLOGY

Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum–associated degradation

(2011) Yoshihiro Matsumura et al.   MOLECULAR BIOLOGY OF THE CELL

Alteration of CFTR transmembrane span integration by disease-causing mutations

(2011) Anna E. Patrick et al.   MOLECULAR BIOLOGY OF THE CELL

Ligand-Driven Vectorial Folding of Ribosome-Bound Human CFTR NBD1

(2011) Amardeep Khushoo et al.   MOLECULAR 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

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

Golgi Bypass: Skirting Around the Heart of Classical Secretion

(2011) A. G. Grieve et al.   Cold Spring Harbor Perspectives in Biology

A Stabilizing Influence: CAL PDZ Inhibition Extends the Half-Life of ΔF508-CFTR

(2010) Patrick R. Cushing et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

The V510D Suppressor Mutation Stabilizes ΔF508-CFTR at the Cell Surface

(2010) Tip W. Loo et al.   BIOCHEMISTRY

c-Cbl Facilitates Endocytosis and Lysosomal Degradation of Cystic Fibrosis Transmembrane Conductance Regulator in Human Airway Epithelial Cells

(2010) Siying Ye et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

α-AP-2 Directs Myosin VI-dependent Endocytosis of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels in the Intestine

(2010) Anne Collaco 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

The Primary Folding Defect and Rescue of ΔF508 CFTR Emerge during Translation of the Mutant Domain

(2010) Hanneke Hoelen et al.   PLoS One

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

(2009) Deborah M. Cholon et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone

(2009) Toby S. Scott-Ward et al.   FEBS Journal

Rescue of ΔF508-CFTR by the SGK1/Nedd4-2 Signaling Pathway

(2009) Hung Caohuy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Membrane-integration Characteristics of Two ABC Transporters, CFTR and P-glycoprotein

(2009) Karl Enquist et al.   JOURNAL OF MOLECULAR BIOLOGY

Rab11b Regulates the Apical Recycling of the Cystic Fibrosis Transmembrane Conductance Regulator in Polarized Intestinal Epithelial Cells

(2009) Mark R. Silvis et al.   MOLECULAR BIOLOGY OF THE CELL

Cooperative Assembly and Misfolding of CFTR Domains In Vivo

(2009) Kai Du et al.   MOLECULAR BIOLOGY OF THE CELL

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

The Relative Binding Affinities of PDZ Partners for CFTR: A Biochemical Basis for Efficient Endocytic Recycling†

(2008) Patrick R. Cushing et al.   BIOCHEMISTRY

BAP31 Interacts with Sec61 Translocons and Promotes Retrotranslocation of CFTRΔF508 via the Derlin-1 Complex

(2008) Bing Wang et al.   CELL

Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation

(2008) Luísa S. Pissarra et al.   CHEMISTRY & BIOLOGY

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

The Mechanism of Cystic Fibrosis Transmembrane Conductance Regulator Transcriptional Repression during the Unfolded Protein Response

(2008) Rafal Bartoszewski et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of N-linked oligosaccharides in the biosynthetic processing of the cystic fibrosis membrane conductance regulator

(2008) X.-b. Chang et al.   JOURNAL OF CELL SCIENCE

Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTRΔF508

(2008) Daisuke Morito et al.   MOLECULAR BIOLOGY OF THE CELL

Sequence-specific Retention and Regulated Integration of a Nascent Membrane Protein by the Endoplasmic Reticulum Sec61 Translocon

(2008) David Pitonzo et al.   MOLECULAR BIOLOGY OF THE CELL

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