CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
about
Mechanisms of CFTR Folding at the Endoplasmic ReticulumRNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)The Contribution of the Airway Epithelial Cell to Host DefenseDNAJs: more than substrate delivery to HSPAExpanding proteostasis by membrane trafficking networksModulation of the maladaptive stress response to manage diseases of protein foldingDer1 promotes movement of misfolded proteins through the endoplasmic reticulum membrane.CFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisScreening the expression of ABCB6 in erythrocytes reveals an unexpectedly high frequency of Lan mutations in healthy individualsPotentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel HydroxypyrazolinesMechanistic 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 TherapeuticsThe silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunctionCodon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa.Rare mutations in renal sodium and potassium transporter genes exhibit impaired transport function.Mutations in MCT8 in patients with Allan-Herndon-Dudley-syndrome affecting its cellular distributionDisruption of interleukin-1β autocrine signaling rescues complex I activity and improves ROS levels in immortalized epithelial cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.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.In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.Using pharmacological chaperones to restore proteostasisScreening for F508del as a first step in the molecular diagnosis of cystic fibrosis.Electrophysiological evidence for the presence of cystic fibrosis transmembrane conductance regulator (CFTR) in mouse sperm.Constrained bithiazoles: small molecule correctors of defective ΔF508-CFTR protein trafficking.Functional rescue of Kallmann syndrome-associated prokineticin receptor 2 (PKR2) mutants deficient in traffickingMeta-analyses of 4 CFTR variants associated with the risk of the congenital bilateral absence of the vas deferens.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.Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.CFTR inhibitors.Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonistRoscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanismTargeting protein-protein interactions to rescue Δf508-cftr: a novel corrector approach to treat cystic fibrosis.Pharmacological correction of misfolding of ABC proteins.Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulatorFull-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.A stable human-cell system overexpressing cystic fibrosis transmembrane conductance regulator recombinant protein at the cell surfacePersonalized biochemistry and biophysics.
P2860
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P2860
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@ast
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@en
type
label
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@ast
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@en
prefLabel
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@ast
CFTR: folding, misfolding and correcting the ΔF508 conformational defect.
@en
P2860
P1476
CFTR: folding, misfolding and correcting the ΔF508 conformational defect
@en
P2093
A S Verkman
P2860
P356
10.1016/J.MOLMED.2011.10.003
P577
2011-12-03T00:00:00Z