Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function. - Wikidata - awgldk - TriplyDB

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

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

http://www.wikidata.org/entity/Q42321930

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Mechanisms of CFTR Folding at the Endoplasmic Reticulum New and emerging targeted therapies for cystic fibrosis Recent developments in the use of differential scanning fluorometry in protein and small molecule discovery and characterization Pharmacological chaperoning: a primer on mechanism and pharmacology ABCG2: the molecular mechanisms of urate secretion and gout A functional CFTR assay using primary cystic fibrosis intestinal organoids Allosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTR Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.New insights into interactions between the nucleotide-binding domain of CFTR and keratin 8.Development of CFTR Structure Using a second-order differential model to fit data without baselines in protein isothermal chemical denaturation Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.Alteration of protein function by a silent polymorphism linked to tRNA abundance.Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.Stabilizing rescued surface-localized δf508 CFTR by potentiation of its interaction with Na(+)/H(+) exchanger regulatory factor 1.Decoding F508del misfolding in cystic fibrosis.Use of kinase inhibitors to correct ΔF508-CFTR function.A chaperone trap contributes to the onset of cystic fibrosis.VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.Pharmacological Correctors of Mutant CFTR Mistrafficking.Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitors CFTR and lung homeostasis.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain.Pharmacological correction of misfolding of ABC proteins.Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.RNA Interference Screen to Identify Kinases That Suppress Rescue of ΔF508-CFTR.F508del-cystic fibrosis transmembrane regulator correctors for treatment of cystic fibrosis: a patent review.Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activity Molecular modelling and molecular dynamics of CFTR.Cystic fibrosis lung environment and Pseudomonas aeruginosa infection.Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.Hallmarks of therapeutic management of the cystic fibrosis functional landscape.Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator.Molecular basis for the binding and modulation of V-ATPase by a bacterial effector protein.

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P2860

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

http://www.wikidata.org/entity/Q42321930

description

2012 nî lūn-bûn

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2012年の論文

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2012年論文

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2012年論文

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2012年論文

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2012年論文

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2012年論文

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2012年论文

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2012年论文

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2012年论文

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name

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@en

Correction of both NBD1 energe ...... 508 CFTR folding and function.

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type

label

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@en

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@nl

prefLabel

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@en

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@nl

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J.CELL.2011.11.024

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P1476

Correction of both NBD1 energe ...... 508 CFTR folding and function.

@en

P2093

Ariel Roldan

http://www.w3.org/2001/XMLSchema#string

Cory M Mulvihill

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Florian Bossard

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Haijin Xu

http://www.w3.org/2001/XMLSchema#string

Kai Du

http://www.w3.org/2001/XMLSchema#string

Lars Konermann

http://www.w3.org/2001/XMLSchema#string

Miklos Bagdany

http://www.w3.org/2001/XMLSchema#string

Salvatore di Bernardo

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Tsukasa Okiyoneda

http://www.w3.org/2001/XMLSchema#string

Wael M Rabeh

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P2860

Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis

Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes

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

Structure of a eukaryotic CLC transporter defines an intermediate state in the transport cycle

Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding

The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain

The use of differential scanning fluorimetry to detect ligand interactions that promote protein stability

The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

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

Evaluation of fluorescence-based thermal shift assays for hit identification in drug discovery.

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150-163

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10.1016/J.CELL.2011.11.024

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1-2

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148

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Gergely L Lukacs

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221766726

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3431169

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