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CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
scientific article published on 3 December 2011
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3643519
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17 January 2020
title
CFTR: folding, misfolding and correcting the ΔF508 conformational defect
(English)
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Europe PubMed Central
PMC publication ID
3643519
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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22138491%20AND%20SRC:MED&resulttype=core&format=json
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17 January 2020
author
Gergely L Lukacs
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1
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Europe PubMed Central
PMC publication ID
3643519
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17 January 2020
author name string
A S Verkman
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2
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Europe PubMed Central
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3643519
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17 January 2020
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English
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3 December 2011
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17 January 2020
published in
Trends in Molecular Medicine
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3643519
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17 January 2020
volume
18
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17 January 2020
issue
2
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Europe PubMed Central
PMC publication ID
3643519
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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22138491%20AND%20SRC:MED&resulttype=core&format=json
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17 January 2020
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81-91
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Europe PubMed Central
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17 January 2020
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Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide‐binding domain 1
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29 September 2017
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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
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29 September 2017
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating
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29 September 2017
Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding
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29 September 2017
Correctors promote folding of the CFTR in the endoplasmic reticulum.
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29 September 2017
Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.
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29 September 2017
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
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CFTR function and prospects for therapy
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29 September 2017
Gp78 cooperates with RMA1 in endoplasmic reticulum-associated degradation of CFTRDeltaF508.
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29 September 2017
Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect
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Correctors of protein trafficking defects identified by a novel high-throughput screening assay
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29 September 2017
Domain interdependence in the biosynthetic assembly of CFTR.
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29 September 2017
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis
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Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator
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F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive
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Folding of CFTR is predominantly cotranslational
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29 September 2017
Assembly of functional CFTR chloride channels
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29 September 2017
Side chain and backbone contributions of Phe508 to CFTR folding
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29 September 2017
The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.
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29 September 2017
A foldable CFTR{Delta}F508 biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase
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29 September 2017
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
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29 September 2017
Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines
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29 September 2017
Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes
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29 September 2017
A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
E3 ubiquitin ligase that recognizes sugar chains
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Degradation of CFTR by the ubiquitin-proteasome pathway
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
29 September 2017
Ligand-driven vectorial folding of ribosome-bound human CFTR NBD1
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
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27 June 2018
Molecular models of the open and closed states of the whole human CFTR protein
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
Processing mutations disrupt interactions between the nucleotide binding and transmembrane domains of P-glycoprotein and the cystic fibrosis transmembrane conductance regulator (CFTR).
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
27 June 2018
A chemical corrector modifies the channel function of F508del-CFTR.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Molecular dynamics analysis of the wild type and dF508 mutant structures of the human CFTR-nucleotide binding domain 1.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Atomic model of human cystic fibrosis transmembrane conductance regulator: membrane-spanning domains and coupling interfaces
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
The folding and evolution of multidomain proteins
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
5 September 2018
Correctors Enhance Maturation of ΔF508 CFTR by Promoting Interactions between the Two Halves of the Molecule
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
4 December 2018
Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
4 December 2018
Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3643519
retrieved
4 December 2018
DeltaF508 mutation increases conformational flexibility of CFTR protein
1 reference
stated in
PubMed
reference URL
https://pubmed.ncbi.nlm.nih.gov/22138491
retrieved
12 December 2020
based on heuristic
inferred from PubMed ID database lookup
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
1 reference
stated in
PubMed
reference URL
https://pubmed.ncbi.nlm.nih.gov/22138491
retrieved
12 December 2020
based on heuristic
inferred from PubMed ID database lookup
Identifiers
DOI
10.1016/J.MOLMED.2011.10.003
1 reference
stated in
Europe PubMed Central
PMC publication ID
3643519
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22138491%20AND%20SRC:MED&resulttype=core&format=json
retrieved
17 January 2020
PMC publication ID
3643519
1 reference
stated in
Europe PubMed Central
PMC publication ID
3643519
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22138491%20AND%20SRC:MED&resulttype=core&format=json
retrieved
17 January 2020
PubMed publication ID
22138491
1 reference
stated in
Europe PubMed Central
PMC publication ID
3643519
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22138491%20AND%20SRC:MED&resulttype=core&format=json
retrieved
17 January 2020
ResearchGate publication ID
51850682
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