The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.

No abstract is available for this paper.

• Publication year

  1993

• Venue

  Proceedings of the National Academy of Sciences of the United States of America

• Publication date

  1993-10-15

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1073/PNAS.90.20.9480PMID 7692448PMCID PMC47592
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland.

  1992cited by this paper
• Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.

  1992cited by this paper
• Immunoglobulin heavy chain-binding protein binds to misfolded mutant insulin receptors with mutations in the extracellular domain.

  1992cited by this paper
• Cystic fibrosis: molecular biology and therapeutic implications.

  1992cited by this paper
• Protein folding in the cell

  1992cited by this paper
• Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

  1992cited by this paper
• The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.

  1992cited by this paper
• Examining the function and regulation of hsp 70 in cells subjected to metabolic stress

  1992cited by this paper
• Regulation of plasma membrane recycling by CFTR.

  1992cited by this paper
• In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium.

  1992cited by this paper
• Interaction of heat‐shock protein 70 with p53 translated in vitro: evidence for interaction with dimeric p53 and for a role in the regulation of p53 conformation.

  1992cited by this paper
• Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR).

  1992cited by this paper
• The mechanism of Z alpha 1-antitrypsin accumulation in the liver.

  1992cited by this paper
• Submucosal glands are the predominant site of CFTR expression in the human bronchus

  1992cited by this paper
• Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.

  1991cited by this paper
• Defective acidification of intracellular organelles in cystic fibrosis

  1991cited by this paper
• Heavy chain binding protein recognizes incompletely disulfide-bonded forms of vesicular stomatitis virus G protein.

  1990cited by this paper
• Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.

  1990cited by this paper
• Interaction of Hsp 70 with newly synthesized proteins: implications for protein folding and assembly.

  1990cited by this paper
• Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.

  1990cited by this paper
• Identification of the cystic fibrosis gene: genetic analysis.

  1989cited by this paper
• Immunocytochemical localization of BiP to the rough endoplasmic reticulum: evidence for protein sorting by selective retention.

  1989cited by this paper
• Identification of the cystic fibrosis gene: chromosome walking and jumping.

  1989cited by this paper
• Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

  1989cited by this paper
• 70K heat shock related proteins stimulate protein translocation into microsomes

  1988cited by this paper
• The Lebanese allele at the low density lipoprotein receptor locus. Nonsense mutation produces truncated receptor that is retained in endoplasmic reticulum.

  1987cited by this paper
• Posttranslational association of immunoglobulin heavy chain binding protein with nascent heavy chains in nonsecreting and secreting hybridomas

  1986cited by this paper
• Uncoating ATPase is a member of the 70 kilodalton family of stress proteins.

  1986cited by this paper
• A very strong enhancer is located upstream of an immediate early gene of human cytomegalovirus.

  1985cited by this paper
• The nucleotide sequence of the chick cytoplasmic beta-actin gene.

  1983cited by this paper
• Effect of weak bases on the intralysosomal pH in mouse peritoneal macrophages

  1981cited by this paper

• p.Phe508del-CFTR Trafficking: A Protein Quality Control Perspective Through UPR, UPS, and Autophagy

  2025cites this paper
• Functional Consequences of CFTR Interactions in Cystic Fibrosis

  2024cites this paper
• Molecular basis of structural stability of Irisin: A combined molecular dynamics simulation and in vitro studies for Urea-induced denaturation

  2023cites this paper
• CFTR Modulators: From Mechanism to Targeted Therapeutics.

  2022cites this paper
• Evaluation of Aminopyrrolidine Amide to improve chloride transport in CFTR-defective cells.

  2022cites this paper
• Molecular mechanisms of cystic fibrosis – how mutations lead to misfunction and guide therapy

  2022cites this paper
• The Distribution and Role of the CFTR Protein in the Intracellular Compartments

  2021cites this paper
• Revisiting CFTR Interactions: Old Partners and New Players

  2021influential citation
• Co-Chaperones in Targeting and Delivery of Misfolded Proteins to the 26S Proteasome

  2020cites this paper
• Molecular Physiology and Pharmacology of the Cystic Fibrosis Transmembrane Conductance Regulator

  2020cites this paper
• Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR

  2020cites this paper
• Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators

  2020cites this paper
• Molecular Mechanisms In Cftr-F508del Degradation And The Functional Defect Of Cftr Absence In Rabbits

  2020cites this paper
• The Probable, Possible, and Novel Functions of ERp29

  2020cites this paper
• Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for Cystic Fibrosis

  2020cites this paper
• Role of HSP70 in Plasticity and Memory

  2019cites this paper
• Targeting DNAJB9, a novel ER luminal co-chaperone, to rescue ΔF508-CFTR

  2019cites this paper
• Modulation of the unfolded protein response pathway as an antiviral approach in airway epithelial cells

  2019cites this paper
• An Intriguing Involvement of Mitochondria in Cystic Fibrosis

  2019cites this paper
• Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum

  2019cites this paper
• TG2 regulates the heat‐shock response by the post‐translational modification of HSF1

  2018cites this paper
• Peripheral Protein Quality Control as a Novel Drug Target for CFTR Stabilizer

  2018cites this paper
• Cystic fibrosis transmembrane conductance regulator—emerging regulator of cancer

  2018cites this paper
• CFTR and Cystic Fibrosis: From Structure to Function

  2018cites this paper
• Lipid Nanoparticle-Delivered Chemically Modified mRNA Restores Chloride Secretion in Cystic Fibrosis.

  2018cites this paper
• Clinical Implications of Osmolytes in Various Human Diseases

  2017cites this paper
• Approaches for discovering novel bioactive small molecules targeting autophagy

  2017cites this paper
• The role of intracellular Ca2+ in cigarette smoke-induced CFTR internalisation

  2017cites this paper
• Rare ER protein misfolding-mistrafficking disorders: Therapeutic developments.

  2017cites this paper
• N-Alpha-Acetyltransferases and Regulation of CFTR Expression

  2016cites this paper
• Identifizierung von Inhibitoren des Hsp90-Aha1 Chaperonkomplexes zur Korrektur des Faltungsdefektes von CFTR\(\Delta\)F508 bei Mukoviszidose

  2016cites this paper
• Effects of 17-DMAG Administration on Autophagy Flux in Mouse Skeletal Muscle

  2016cites this paper
• Structural basis of urea-induced unfolding: Unraveling the folding pathway of hemochromatosis factor E.

  2016cites this paper
• CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis

  2016cites this paper
• Correctors Rescue CFTR Mutations in Nucleotide‐Binding Domain 1 (NBD1) by Modulating Proteostasis

  2016cites this paper
• Macrophage Polarization and Function in Cystic Fibrosis

  2016cites this paper
• Ion Channels and Transporters in Lung Function and Disease Trafficking and function of the cystic fibrosis transmembrane conductance regulator : a complex network of posttranslational modifications

  2016cites this paper
• From the endoplasmic reticulum to the plasma membrane: mechanisms of CFTR folding and trafficking

  2016cites this paper
• Implication of Molten Globule on the Function and Toxicity of a Protein

  2016cites this paper
• Personalized Medicine in Respiratory Disease: Role of Proteomics.

  2016cites this paper
• Synonymous codon usage affects the expression of wild type and F508del CFTR.

  2015cites this paper
• Rescue of NBD2 Mutants N1303K and S1235R of CFTR by Small-Molecule Correctors and Transcomplementation

  2015cites this paper
• Role of FGF Receptors in Rescue of ΔF508-CFTR

  2015cites this paper
• Involvement of the Cdc42 Pathway in CFTR Post-Translational Turnover and in Its Plasma Membrane Stability in Airway Epithelial Cells

  2015cites this paper
• Expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiae.

  2015cites this paper
• Targeting Molecular Chaperones for the Treatment of Cystic Fibrosis: Is It a Viable Approach?

  2015cites this paper
• SERCA and PMCA pumps contribute to the deregulation of Ca2+ homeostasis in human CF epithelial cells.

  2015cites this paper
• Modulation of Proteostasis for the Efficient Intracellular Transport of the ΔF508 Mutant of Cystic Fibrosis Transmembrane Conductance Regulator

  2014cites this paper
• Quantitative Proteomic Profiling Reveals Differentially Regulated Proteins in Cystic Fibrosis Cells

  2014cites this paper
• Complement yourself: transcomplementation rescues partially folded mutant proteins

  2014influential citation
• The Modulation of the Cystic Fibrosis Transmembrane Conductance Regulator Protein by Sphingosine-1-Phosphate in the Cerebral Microvascular System

  2014cites this paper
• CFTR structure and cystic fibrosis.

  2014cites this paper
• Biosynthesis of cystic fibrosis transmembrane conductance regulator.

  2014cites this paper
• Cytoskeleton and CFTR.

  2014cites this paper
• Cystic fibrosis transmembrane conductance regulator degradation: cross‐talk between the ubiquitylation and SUMOylation pathways

  2013cites this paper
• Stimulation de cellules épithéliales bronchiques humaines par la GnRH : effet sur le transport ionique médié par le CFTR

  2013cites this paper
• Cystic Fibrosis: CFTR, Complications, and Prospective Therapies

  2013influential citation
• Effects of Sleep and Wake on Oligodendrocytes and Their Precursors

  2013cites this paper
• How early studies on secreted and membrane protein quality control gave rise to the ER associated degradation (ERAD) pathway: the early history of ERAD.

  2013cites this paper
• Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier–dependent pathway

  2013influential citation
• CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) IN HUMAN LEUKOCYTES

  2013cites this paper
• Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis.

  2013cites this paper
• Chapter 58 – Cystic Fibrosis

  2013cites this paper
• The effect of NO-donors on chloride efflux, intracellular Ca(2+) concentration and mRNA expression of CFTR and ENaC in cystic fibrosis airway epithelial cells.

  2013cites this paper
• Hsp90 prevents interaction between CHIP and HERG proteins to facilitate maturation of wild-type and mutant HERG proteins.

  2013cites this paper
• On the design of broad based screening assays to identify potential pharmacological chaperones of protein misfolding diseases.

  2013cites this paper
• Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic Fibrosis

  2012cites this paper
• Dexamethasone Regulates CFTR Expression in Calu-3 Cells with the Involvement of Chaperones HSP70 and HSP90

  2012cites this paper
• Mechanisms of CFTR Folding at the Endoplasmic Reticulum

  2012influential citation
• Human Heat Shock Protein 105/110 kDa (Hsp105/110) Regulates Biogenesis and Quality Control of Misfolded Cystic Fibrosis Transmembrane Conductance Regulator at Multiple Levels*

  2012cites this paper
• Improving Cell Surface Functional Expression of Delta F508 CFTR: A Quest for Therapeutic Targets

  2012cites this paper
• The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology.

  2012cites this paper
• BAG-1 Stabilizes Mutant F508del-CFTR in a Ubiquitin-Like-Domain-Dependent Manner

  2012cites this paper
• HSP70 decreases receptor-dependent phosphorylation of Smad2 and blocks TGF-β-induced epithelial-mesenchymal transition.

  2011cites this paper
• Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies.

  2011cites this paper
• FKBP38 Peptidylprolyl Isomerase Promotes the Folding of Cystic Fibrosis Transmembrane Conductance Regulator in the Endoplasmic Reticulum*

  2011cites this paper
• 4-Phenylbutyrate Stimulates Hsp70 Expression through the Elp2 Component of Elongator and STAT-3 in Cystic Fibrosis Epithelial Cells*

  2011cites this paper
• Les protéines Gα12 et Gα13 dans la mucoviscidose : Rôle dans la dégradation de la protéine CFTR mutée F508del et dans le contrôle des jonctions intercellulaires.

  2011influential citation
• ERp29 Regulates ΔF508 and Wild-type Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Trafficking to the Plasma Membrane in Cystic Fibrosis (CF) and Non-CF Epithelial Cells*

  2011cites this paper
• Biochemical and biophysical approaches to probe CFTR structure.

  2011cites this paper
• Ligand-driven vectorial folding of ribosome-bound human CFTR NBD1.

  2011cites this paper
• Functional Dissection of FKBP38 in the Biogenesis of Cystic Fibrosis Transmembrane Conductance Regulator in the Endoplasmic Reticulum

  2011cites this paper
• Sorting Signals, Domain Conformation and Interdomain Interactions in CFTR Misprocessing and Rescue

  2010influential citation
• Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue

  2010influential citation
• A novel ER J-protein DNAJB12 accelerates ER-associated degradation of membrane proteins including CFTR.

  2010cites this paper
• Advances in Pharmacological Treatment of Cystic Fibrosis

  2010cites this paper
• Computational analysis of gene regulation in animal sleep deprivation.

  2010cites this paper
• Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies.

  2010cites this paper
• Sorting signals, domain conformation and interdomain interactions in CFTR misprocessing and rescue

  2010cites this paper
• Folding Biology of Cystic Fibrosis: A Consortium‐Based Approach to Disease

  2010cites this paper
• Applications of proteomic technologies for understanding the premature proteolysis of CFTR

  2010cites this paper
• Proteomics uncovering possible key players in F508del-CFTR processing and trafficking

  2010cites this paper
• Role of Hsp105 in CFTR biogenesis

  2010cites this paper
• Structural and functional diversity among eukaryotic Hsp70 nucleotide exchange factors.

  2009cites this paper
• 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

  2009cites this paper
• N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic

  2009cites this paper
• Gene and Cell Therapy for the Treatment of Cystic Fibrosis

  2009cites this paper
• Cysteine String Protein Promotes Proteasomal Degradation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Increasing Its Interaction with the C Terminus of Hsp70-interacting Protein and Promoting CFTR Ubiquitylation*

  2009cites this paper
• Lipid Rafts, Lipopolysaccharide and Stress Proteins in Infectious Diseases

  2009cites this paper
• Mouse Pheromone Receptors: The Molecular Basis of Surface Trafficking and Ligand Selectivity

  2009cites this paper