Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
about
Correcting the cystic fibrosis disease mutant, A455E CFTRComputational design of a PDZ domain peptide inhibitor that rescues CFTR activityIvacaftor for patients with cystic fibrosisDeletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperoneChloride channels as drug targets.Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770New and emerging targeted therapies for cystic fibrosisTargeted therapies to improve CFTR function in cystic fibrosisEmerging novel concept of chaperone therapies for protein misfolding diseasesThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyA synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cellsRequirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved SequencesCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisRescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementationSphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ActivityThe cystic fibrosis transmembrane conductance regulator (CFTR) and its stabilitySmall-molecule structure correctors target abnormal protein structure and function: structure corrector rescue of apolipoprotein E4-associated neuropathologyHarnessing public domain data to discover and validate therapeutic targets.Small molecule correctors of F508del-CFTR discovered by structure-based virtual screeningAdditive effect of multiple pharmacological chaperones on maturation of CFTR processing mutantsThe role of the UPS in cystic fibrosis.Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.Activation of the CFTR Cl- channel by trimethoxyflavone in vitro and in vivoInterplay between ER exit code and domain conformation in CFTR misprocessing and rescue.Clinical and molecular characterization of S1118F-CFTR.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesModulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial culturesSynergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.New horizons in the treatment of cystic fibrosis.Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator.Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.Decoding F508del misfolding in cystic fibrosis.DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.CFTR Modulators for the Treatment of Cystic Fibrosis.Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling.Is CFTR-delF508 really absent from the apical membrane of the airway epithelium?Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosisCystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.Applications of proteomic technologies for understanding the premature proteolysis of CFTR.
P2860
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P2860
Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Rescue of DeltaF508-CFTR traff ...... y cultures by small molecules.
@en
type
label
Rescue of DeltaF508-CFTR traff ...... y cultures by small molecules.
@en
prefLabel
Rescue of DeltaF508-CFTR traff ...... y cultures by small molecules.
@en
P2093
P1476
Rescue of DeltaF508-CFTR traff ...... y cultures by small molecules.
@en
P2093
Anna Hazlewood
Ashvani Singh
Eric Olson
Fredrick Van Goor
James Rader
Jeffrey H Stack
Jesús González
John Joubran
Kimberly S Straley
P304
P356
10.1152/AJPLUNG.00169.2005
P577
2006-01-27T00:00:00Z