Related references
Note: Only part of the references are listed.Chaperone-Independent Peripheral Quality Control of CFTR by RFFL E3 Ligase
Tsukasa Okiyoneda et al.
DEVELOPMENTAL CELL (2018)
Dissection of the Role of VIMP in Endoplasmic Reticulum-Associated Degradation of CFTRΔF508
Xia Hou et al.
SCIENTIFIC REPORTS (2018)
Pharmacological Inhibition of the Ubiquitin Ligase RNF5 Rescues F508del-CFTR in Cystic Fibrosis Airway Epithelia
Elvira Sondo et al.
CELL CHEMICAL BIOLOGY (2018)
Mutation-specific downregulation of CFTR2 variants by gating potentiators
Radu G. Avramescu et al.
HUMAN MOLECULAR GENETICS (2017)
Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR
Scott H. Donaldson et al.
JOURNAL OF CYSTIC FIBROSIS (2017)
Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
Miklos Bagdany et al.
NATURE COMMUNICATIONS (2017)
Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation
Xiaoyan Gong et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2016)
Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications
Michelle L. McClure et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2016)
SYVN1, NEDD8, and FBXO2 Proteins Regulate F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation
Shyam Ramachandran et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2016)
VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis
Wayne L. Ernst et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2016)
EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1
Miguel J. Lobo et al.
JOURNAL OF CELL SCIENCE (2016)
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations
Gudio Veit et al.
MOLECULAR BIOLOGY OF THE CELL (2016)
Synonymous Codons Direct Cotranslational Folding toward Different Protein Conformations
Florian Buhr et al.
MOLECULAR CELL (2016)
Cystic fibrosis lung environment and Pseudomonas aeruginosa infection
Anjali Y. Bhagirath et al.
BMC PULMONARY MEDICINE (2016)
Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect
Guido Veit et al.
PLOS BIOLOGY (2016)
CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis
Miqueias Lopes-Pacheco
FRONTIERS IN PHARMACOLOGY (2016)
Intracellular Delivery of Peptidyl Ligands by Reversible Cyclization: Discovery of a PDZ Domain Inhibitor that Rescues CFTR Activity
Ziqing Qian et al.
ANGEWANDTE CHEMIE-INTERNATIONAL EDITION (2015)
Elevated Sweat Chloride Levels Due to Arsenic Toxicity
Maitreyi Mazumdar et al.
NEW ENGLAND JOURNAL OF MEDICINE (2015)
A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint
Claudia A. Loureiro et al.
SCIENCE SIGNALING (2015)
Unravelling druggable signalling networks that control F508del-CFTR proteostasis
Ramanath Narayana Hegde et al.
ELIFE (2015)
A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint
Claudia A. Loureiro et al.
SCIENCE SIGNALING (2015)
ΔF508 CFTR Surface Stability Is Regulated by DAB2 and CHIP-Mediated Ubiquitination in Post-Endocytic Compartments
Lianwu Fu et al.
PLOS ONE (2015)
Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR
Carlos M. Farinha et al.
PHARMACOLOGY RESEARCH & PERSPECTIVES (2015)
Potentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action
Puay-Wah Phuan et al.
MOLECULAR PHARMACOLOGY (2015)
S-nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface
Khalequz Zaman et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2014)
Stabilizing Rescued Surface-Localized ΔF508 CFTR by Potentiation of Its Interaction with Na+/H+ Exchanger Regulatory Factor 1
Kavisha Arora et al.
BIOCHEMISTRY (2014)
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR
Yiting Wang et al.
BRITISH JOURNAL OF PHARMACOLOGY (2014)
LMTK2-mediated Phosphorylation Regulates CFTR Endocytosis in Human Airway Epithelial Cells
Simao Luz et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2014)
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction
Julia E. Rasmussen et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2014)
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function
Fredrick Van Goor et al.
JOURNAL OF CYSTIC FIBROSIS (2014)
TGF-Beta Downregulation of Distinct Chloride Channels in Cystic Fibrosis-Affected Epithelia
Hongtao Sun et al.
PLOS ONE (2014)
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression
Guido Veit et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
Deborah M. Cholon et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
Endoplasmic Reticulum Protein Quality Control Is Determined by Cooperative Interactions between Hsp/c70 Protein and the CHIP E3 Ligase
Yoshihiro Matsumura et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
RNF185 Is a Novel E3 Ligase of Endoplasmic Reticulum-associated Degradation (ERAD) That Targets Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
Elma El Khouri et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
Hong Yu Ren et al.
MOLECULAR BIOLOGY OF THE CELL (2013)
Mechanism-based corrector combination restores ΔF508-CFTR folding and function
Tsukasa Okiyoneda et al.
NATURE CHEMICAL BIOLOGY (2013)
Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells
Steven M. Snodgrass et al.
PLOS ONE (2013)
Ubiquitination and Degradation of CFTR by the E3 Ubiquitin Ligase MARCH2 through Its Association with Adaptor Proteins CAL and STX6
Jie Cheng et al.
PLOS ONE (2013)
Detection of Phospho- Sites Generated by Protein Kinase CK2 in CFTR: Mechanistic Aspects of Thr1471 Phosphorylation
Andrea Venerando et al.
PLOS ONE (2013)
Targeting the intracellular environment in cystic fibrosis: restoring autophagy as a novel strategy to circumvent the CFTR defect
Valeria Rachela Villella et al.
FRONTIERS IN PHARMACOLOGY (2013)
Nedd4-2 does not regulate wt-CFTR in human airway epithelial cells
Katja Koeppen et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2012)
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator
Alessandro Luciani et al.
AUTOPHAGY (2012)
Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator
Lianwu Fu et al.
BIOCHEMICAL JOURNAL (2012)
Correction of Both NBD1 Energetics and Domain Interface Is Required to Restore ΔF508 CFTR Folding and Function
Wael M. Rabeh et al.
CELL (2012)
Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration
Lucy A. Clunes et al.
FASEB JOURNAL (2012)
Arsenic Promotes Ubiquitinylation and Lysosomal Degradation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Chloride Channels in Human Airway Epithelial Cells
Jennifer M. Bomberger et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
Use of Kinase Inhibitors to Correct ΔF508-CFTR Function
Agata M. Trzcinska-Daneluti et al.
MOLECULAR & CELLULAR PROTEOMICS (2012)
Mechanisms of CFTR folding at the endoplasmic reticulum
Soo Jung Kim et al.
FRONTIERS IN PHARMACOLOGY (2012)
Nedd4-2 and the regulation of epithelial sodium transport
Daniela Rotin et al.
FRONTIERS IN PHYSIOLOGY (2012)
Antagonistic Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Cell Surface Expression by Protein Kinases WNK4 and Spleen Tyrosine Kinase
Ana Isabel Mendes et al.
MOLECULAR AND CELLULAR BIOLOGY (2011)
Contribution of Casein Kinase 2 and Spleen Tyrosine Kinase to CFTR Trafficking and Protein Kinase A-Induced Activity
Simao Luz et al.
MOLECULAR AND CELLULAR BIOLOGY (2011)
The endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRΔF508
Diane E. Grove et al.
MOLECULAR BIOLOGY OF THE CELL (2011)
Plasma TGF-β1 in Pediatric Cystic Fibrosis: Potential Biomarker of Lung Disease and Response to Therapy
William T. Harris et al.
PEDIATRIC PULMONOLOGY (2011)
Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease
Toshihiro Kimura et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
TorsinA participates in endoplasmic reticulum-associated degradation
Flavia C. Nery et al.
NATURE COMMUNICATIONS (2011)
A Pseudomonas aeruginosa Toxin that Hijacks the Host Ubiquitin Proteolytic System
Jennifer M. Bomberger et al.
PLOS PATHOGENS (2011)
Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures
Deborah M. Cholon et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2010)
A Stabilizing Influence: CAL PDZ Inhibition Extends the Half-Life of Delta F508-CFTR
Patrick R. Cushing et al.
ANGEWANDTE CHEMIE-INTERNATIONAL EDITION (2010)
c-Cbl Facilitates Endocytosis and Lysosomal Degradation of Cystic Fibrosis Transmembrane Conductance Regulator in Human Airway Epithelial Cells
Siying Ye et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
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
Maria Favia et al.
MOLECULAR BIOLOGY OF THE CELL (2010)
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis
Darren M. Hutt et al.
NATURE CHEMICAL BIOLOGY (2010)
Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy
Nadzeya V. Marozkina et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane
Tsukasa Okiyoneda et al.
SCIENCE (2010)
Cadmium Regulates the Expression of the CFTR Chloride Channel in Human Airway Epithelial Cells
Jessica Rennolds et al.
TOXICOLOGICAL SCIENCES (2010)
CARP2 deficiency does not alter induction of NF-κB by TNFα
Afsar U. Ahmed et al.
CURRENT BIOLOGY (2009)
Mechanistic Insight into Control of CFTR by AMPK
Patthara Kongsuphol et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Rescue of ΔF508-CFTR by the SGK1/Nedd4-2 Signaling Pathway
Hung Caohuy et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
The Deubiquitinating Enzyme USP10 Regulates the Post-endocytic Sorting of Cystic Fibrosis Transmembrane Conductance Regulator in Airway Epithelial Cells
Jennifer M. Bomberger et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic
Rina Glozman et al.
JOURNAL OF CELL BIOLOGY (2009)
Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
Karoly Varga et al.
BIOCHEMICAL JOURNAL (2008)
Role of calnexin in the ER quality control and productive folding of CFrR;: differential effect of calnexin knockout on wild-type and ΔF508 CFTR
Tsukasa Okiyoneda et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2008)
Gp78 cooperates with RMA1 in endoplasmic reticulum-associated degradation of CFTRΔF508
Daisuke Morito et al.
MOLECULAR BIOLOGY OF THE CELL (2008)
Assembly and Misassembly of Cystic Fibrosis Transmembrane Conductance Regulator: Folding Defects Caused by Deletion of F508 Occur Before and After the Calnexin-dependent Association of Membrane Spanning Domain (MSD) 1 and MSD2
Meredith F. N. Rosser et al.
MOLECULAR BIOLOGY OF THE CELL (2008)
The role of the UPS in cystic fibrosis
Emma L. Turnbull et al.
BMC BIOCHEMISTRY (2007)
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein
Ying Wang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
Targeting CAL as a negative regulator of ΔF508-CFTR cell-surface expression -: An RNA interference and structure-based muta genetic approach
Michael Wolde et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR
William R. Thelin et al.
JOURNAL OF CLINICAL INVESTIGATION (2007)
Peptide microarray analysis of substrate specificity of the transmembrane Ser/Thr kinase KPI-2 reveals reactivity with cystic fibrosis transmembrane conductance regulator and phosphorylase
Hong Wang et al.
MOLECULAR & CELLULAR PROTEOMICS (2006)
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator
J. Michael Younger et al.
CELL (2006)
Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells
A Swiatecka-Urban et al.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2006)
The short apical membrane half-life of rescued ΔF508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of ΔF508-CFTR in polarized human airway epithelial cells
A Swiatecka-Urban et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Folding of CFTR is predominantly cotranslational
B Kleizen et al.
MOLECULAR CELL (2005)
Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes
M Sharma et al.
JOURNAL OF CELL BIOLOGY (2004)
ΔF508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression
T Okiyoneda et al.
MOLECULAR BIOLOGY OF THE CELL (2004)
Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL
J Cheng et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Hop: more than an Hsp70/Hsp90 adaptor protein
OO Odunuga et al.
BIOESSAYS (2004)
CFTR and chaperones - Processing and degradation
MD Amaral
JOURNAL OF MOLECULAR NEUROSCIENCE (2004)
Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA
V Chappe et al.
JOURNAL OF PHYSIOLOGY-LONDON (2003)
A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates
MR Silvis et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Early infection and progression of cystic fibrosis lung disease
C Koch
PEDIATRIC PULMONOLOGY (2002)
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
GC Meacham et al.
NATURE CELL BIOLOGY (2001)
The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins
P Connell et al.
NATURE CELL BIOLOGY (2001)
Airway nitric oxide levels in cystic fibrosis patients are related to a polymorphism in the neuronal nitric oxide synthase gene
H Grasemann et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2000)
Cystic fibrosis transmembrane conductance regulator has an altered structure when its maturation is inhibited
EYJ Chen et al.
BIOCHEMISTRY (2000)
Share Paper
