Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression. (Q35743348)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25101887%20AND%20SRC:MED&resulttype=core&format=json

12 March 2020

title

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression (English)

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12 March 2020

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12 March 2020

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12 March 2020

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12 March 2020

1 reference

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12 March 2020

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12 March 2020

Miklos Bagdany

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12 March 2020

Radu G Avramescu

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12 March 2020

Puay-Wah Phuan

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12 March 2020

Walter E Finkbeiner

1 reference

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12 March 2020

Alan S Verkman

1 reference

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12 March 2020

Florence Borot

1 reference

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12 March 2020

Yu-Sheng Wu

series ordinal

1 reference

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12 March 2020

Tamas Hegedus

series ordinal

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12 March 2020

language of work or name

English

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publication date

1 July 2014

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Science Translational Medicine

12 March 2020

published in

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volume

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12 March 2020

issue

246

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page(s)

246ra97

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Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

12 March 2020

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Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.

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Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function

27 July 2018

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Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction.

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

27 July 2018

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Conditionally reprogrammed cells represent a stem-like state of adult epithelial cells

27 July 2018

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Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia

27 July 2018

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Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation

27 July 2018

1 reference

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Ivacaftor potentiation of multiple CFTR channels with gating mutations

27 July 2018

1 reference

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Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences

27 July 2018

1 reference

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Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function

27 July 2018

1 reference

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

27 July 2018

1 reference

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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

27 July 2018

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Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment

27 July 2018

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Use of primary cultures of human bronchial epithelial cells isolated from cystic fibrosis patients for the pre-clinical testing of CFTR modulators

27 July 2018

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MicroRNAs and cystic fibrosis--an epigenetic perspective.

27 July 2018

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

27 July 2018

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Quality control for unfolded proteins at the plasma membrane

27 July 2018

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Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide‐binding domain 1

27 July 2018

1 reference

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The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.

27 July 2018

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Peripheral protein quality control removes unfolded CFTR from the plasma membrane

27 July 2018

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Inhibition of Ca2+-activated Cl- channels by gallotannins as a possible molecular basis for health benefits of red wine and green tea.

27 July 2018

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CFTR-adenylyl cyclase I association responsible for UTP activation of CFTR in well-differentiated primary human bronchial cell cultures

27 July 2018

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

27 July 2018

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AutoDock4 and AutoDockTools4: Automated docking with selective receptor flexibility

27 July 2018

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

27 July 2018

1 reference

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CFTR function and prospects for therapy

27 July 2018

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F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive

27 July 2018

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The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating

27 July 2018

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Towards an in vitro model of cystic fibrosis small airway epithelium: characterisation of the human bronchial epithelial cell line CFBE41o-.

27 July 2018

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27 July 2018

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CFTR gating II: Effects of nucleotide binding on the stability of open states

27 July 2018

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The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

27 July 2018

1 reference

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27 July 2018

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Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508.

27 July 2018

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Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings

27 July 2018

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Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line

27 July 2018

1 reference

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C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation

27 July 2018

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DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis

27 July 2018

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Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype

27 July 2018

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Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

27 July 2018

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Differentiated structure and function of cultures from human tracheal epithelium

27 July 2018

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Cystic fibrosis: molecular biology and therapeutic implications

27 July 2018

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Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.

27 July 2018

1 reference

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VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.

25 September 2018

1 reference

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Corrector VX-809 stabilizes the first transmembrane domain of CFTR.

25 September 2018

1 reference

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A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

25 September 2018

1 reference

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Potent, metabolically stable benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR inhibitors for polycystic kidney disease

25 September 2018

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Endocytic sorting of CFTR variants monitored by single-cell fluorescence ratiometric image analysis (FRIA) in living cells.

25 September 2018

1 reference

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

25 September 2018

1 reference

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Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials

25 September 2018

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

25 September 2018

1 reference

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

25 September 2018

1 reference

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Well-differentiated human airway epithelial cell cultures.

25 September 2018

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

25 September 2018

1 reference

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

25 September 2018

1 reference

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25 September 2018

1 reference

https://pubmed.ncbi.nlm.nih.gov/25101887

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1126/SCITRANSLMED.3008889

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25101887%20AND%20SRC:MED&resulttype=core&format=json

12 March 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25101887%20AND%20SRC:MED&resulttype=core&format=json

12 March 2020

PubMed publication ID

25101887

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25101887%20AND%20SRC:MED&resulttype=core&format=json