Combating cystic fibrosis: in search for CF transmembrane conductance regulator (CFTR) modulators (Q37833017)

24 August 2017

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title

Combating cystic fibrosis: in search for CF transmembrane conductance regulator (CFTR) modulators (English)

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24 August 2017

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transmembrane protein

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author name string

Efrat Noy

series ordinal

1

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PubMed ID

21275046

24 August 2017

Hanoch Senderowitz

series ordinal

2

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24 August 2017

14 January 2011

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24 August 2017

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24 August 2017

6

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24 August 2017

243-251

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24 August 2017

2

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The ABC protein turned chloride channel whose failure causes cystic fibrosis

24 August 2017

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Subunit interactions in ABC transporters: towards a functional architecture

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

ABC transporter architecture and mechanism: implications from the crystal structures of BtuCD and BtuF.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

ABC transporter architecture and regulatory roles of accessory domains

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Multidrug transporters in prokaryotic and eukaryotic cells: physiological functions and transport mechanisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Cystic fibrosis since 1938.

21 January 2018

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https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

CFTR function and prospects for therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Emerging treatments in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Altered protein folding may be the molecular basis of most cases of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Defective protein folding as a basis of human disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

G protein-coupled receptor trafficking in health and disease: lessons learned to prepare for therapeutic mutant rescue in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein

21 January 2018

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https://api.crossref.org/works/10.1002%2FCMDC.201000488

Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR).

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Correctors of protein trafficking defects identified by a novel high-throughput screening assay

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

PTC124 targets genetic disorders caused by nonsense mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Repairing faulty genes by aminoglycosides: development of new derivatives of geneticin (G418) with enhanced suppression of diseases-causing nonsense mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structure-based drug screening and ligand-based drug screening with machine learning.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Enhancing drug discovery through in silico screening: strategies to increase true positives retrieval rates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

G Protein-Coupled Receptors: target-based in silico screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structure of P-glycoprotein reveals a molecular basis for poly-specific drug binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structure of a bacterial multidrug ABC transporter

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Flexibility in the ABC transporter MsbA: Alternating access with a twist

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Building an understanding of cystic fibrosis on the foundation of ABC transporter structures

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Domain interdependence in the biosynthetic assembly of CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Side chain and backbone contributions of Phe508 to CFTR folding

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Dynasore, a cell-permeable inhibitor of dynamin

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

4'-Methyl-4,5'-bithiazole-based correctors of defective delta F508-CFTR cellular processing

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

Structure and function of the CFTR chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

7 January 2021

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

7 January 2021

Prevention of platelet glycoprotein IIb/IIIa activation by 3,4-methylenedioxy-beta-nitrostyrene, a novel tyrosine kinase inhibitor

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

7 January 2021

Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

7 January 2021

Arrest of CFTRDeltaF508 folding

1 reference

https://api.crossref.org/works/10.1002%2FCMDC.201000488

7 January 2021

Identifiers

DOI

10.1002/CMDC.201000488

1 reference

24 August 2017

1 reference

PubMed ID

21275046