Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis (Q37885153)

24 August 2017

1 reference

title

Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis (English)

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24 August 2017

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Zhi-wei Cai

1

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24 August 2017

Jia Liu

series ordinal

2

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24 August 2017

Hong-yu Li

series ordinal

3

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24 August 2017

David N Sheppard

series ordinal

4

1 reference

24 August 2017

1 June 2011

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Acta Pharmacologica Sinica

24 August 2017

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24 August 2017

32

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24 August 2017

693-701

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24 August 2017

6

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https://scigraph.springernature.com/pub.10.1038/aps.2011.71

24 August 2017

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cites work

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A unified view of cystic fibrosis transmembrane conductance regulator (CFTR) gating: combining the allosterism of a ligand-gated channel with the enzymatic activity of an ATP-binding cassette (ABC) transporter

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Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

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Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening

6 September 2017

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Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

6 September 2017

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Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation

6 September 2017

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Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

6 September 2017

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6 September 2017

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Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR

6 September 2017

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G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects

6 September 2017

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Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

6 September 2017

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Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms

6 September 2017

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Structure of a bacterial multidrug ABC transporter

6 September 2017

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The ABC protein turned chloride channel whose failure causes cystic fibrosis

6 September 2017

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Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations.

6 September 2017

1 reference

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Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

6 September 2017

1 reference

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Cystic fibrosis since 1938.

6 September 2017

1 reference

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6 September 2017

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Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

6 September 2017

1 reference

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Voltage-dependent Gating of the Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel

6 September 2017

1 reference

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Prolonged nonhydrolytic interaction of nucleotide with CFTR's NH2-terminal nucleotide binding domain and its role in channel gating

6 September 2017

1 reference

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On the mechanism of MgATP-dependent gating of CFTR Cl- channels

6 September 2017

1 reference

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ATP binding to the motor domain from an ABC transporter drives formation of a nucleotide sandwich dimer

6 September 2017

1 reference

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The E. coli BtuCD structure: a framework for ABC transporter architecture and mechanism

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Structure and function of the CFTR chloride channel

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Phosphatase inhibitors activate normal and defective CFTR chloride channels

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Regulation of the gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR.

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Delta F508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Molecular models of the open and closed states of the whole human CFTR protein

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Nucleoside triphosphates are required to open the CFTR chloride channel.

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

A chemical corrector modifies the channel function of F508del-CFTR.

23 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Cystic fibrosis transmembrane conductance regulator: using differential reactivity toward channel-permeant and channel-impermeant thiol-reactive probes to test a molecular model for the pore

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Atomic model of human cystic fibrosis transmembrane conductance regulator: membrane-spanning domains and coupling interfaces

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure.

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Genistein potentiates wild-type and delta F508-CFTR channel activity

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4009972

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

1 September 2018

1 reference

Crossref

https://api.crossref.org/works/10.1038%2FAPS.2011.71

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1038%2FAPS.2011.71

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1038%2FAPS.2011.71

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

21 January 2018

1 reference

12 December 2020

Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium

1 reference

12 December 2020

Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators. Interaction between activation and inhibition

1 reference

12 December 2020

Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel

1 reference

12 December 2020

Identifiers

DOI

10.1038/APS.2011.71

1 reference

24 August 2017

1 reference

24 August 2017

1 reference