Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences
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Correcting the cystic fibrosis disease mutant, A455E CFTRMechanisms of CFTR Folding at the Endoplasmic ReticulumNew and emerging targeted therapies for cystic fibrosisPharmacological chaperoning: a primer on mechanism and pharmacologyModulation of the maladaptive stress response to manage diseases of protein foldingCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisABCG2: the molecular mechanisms of urate secretion and goutAllosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTRRescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementationMechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic FibrosisIncreasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule TherapeuticsImpact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.Stabilization of Nucleotide Binding Domain Dimers Rescues ABCC6 Mutants Associated with Pseudoxanthoma Elasticum.Development of CFTR StructureEnhancing 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.Complement yourself: Transcomplementation rescues partially folded mutant proteins.Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftorDecoding F508del misfolding in cystic fibrosis.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.Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNAPharmacological Correctors of Mutant CFTR Mistrafficking.Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitorsDiscovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain.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.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.Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease.Pharmacogenomics: mapping monogenic mutations to direct therapyPharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platformThermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activityMolecular modelling and molecular dynamics of CFTR.Cystic fibrosis lung environment and Pseudomonas aeruginosa infection.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.Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.Capturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry.
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P2860
Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences
description
2012 nî lūn-bûn
@nan
2012 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
name
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@ast
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@en
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@nl
type
label
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@ast
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@en
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@nl
prefLabel
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@ast
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@en
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@nl
P2093
P2860
P3181
P1433
P1476
Requirements for Efficient Cor ...... Analyses of Evolved Sequences
@en
P2093
Andrew P Feranchak
André Schmidt
Emmanuel Nuvaga
Philip J Thomas
Robert J Bridges
Tyler Barrett
P2860
P304
P3181
P356
10.1016/J.CELL.2011.11.023
P407
P577
2012-01-20T00:00:00Z