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Mechanisms of CFTR Folding at the Endoplasmic ReticulumMolecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisUpdate in Cystic Fibrosis 2014Epithelial sodium channels (ENaC) are uniformly distributed on motile cilia in the oviduct and the respiratory airwaysEndoplasmic reticulum protein quality control is determined by cooperative interactions between Hsp/c70 protein and the CHIP E3 ligaseSignature motifs identify an Acinetobacter Cif virulence factor with epoxide hydrolase activityReduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosisNew and emerging targeted therapies for cystic fibrosisInhaled protein/peptide-based therapies for respiratory diseaseRegulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.Chemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyThe Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): THREE-DIMENSIONAL STRUCTURE AND LOCALIZATION OF A CHANNEL GATEStructural insights into PDZ-mediated interaction of NHERF2 and LPA2, a cellular event implicated in CFTR channel regulationAdvances in the molecular detection of ABC transporters involved in multidrug resistance in cancerA functional CFTR assay using primary cystic fibrosis intestinal organoidsDefective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosisCarbachol-induced MUC17 endocytosis is concomitant with NHE3 internalization and CFTR membrane recruitment in enterocytesIdentification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plantsPseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial CellsPotentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel HydroxypyrazolinesAugmentation of CFTR maturation by S-nitrosoglutathione reductase.The cystic fibrosis transmembrane conductance regulator (CFTR) and its stabilityS-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface.LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.Development of CFTR StructureSmall-molecule structure correctors target abnormal protein structure and function: structure corrector rescue of apolipoprotein E4-associated neuropathologyHsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy.Cdc42 controls spindle orientation to position the apical surface during epithelial morphogenesis.Small molecule correctors of F508del-CFTR discovered by structure-based virtual screeningDeletion of CFTR translation start site reveals functional isoforms of the protein in CF patients.Functional regulation of cystic fibrosis transmembrane conductance regulator-containing macromolecular complexes: a small-molecule inhibitor approachA novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway.Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotypeHsp40 chaperones promote degradation of the HERG potassium channel.Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis.Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.Serum- and glucocorticoid-induced protein kinase 1 (SGK1) increases the cystic fibrosis transmembrane conductance regulator (CFTR) in airway epithelial cells by phosphorylating Shank2E protein.Chloride channels: often enigmatic, rarely predictable.
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description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
gotara zanistî
@ku-latn
scientific article published on January 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
name
CFTR function and prospects for therapy.
@en
CFTR function and prospects for therapy.
@nl
type
label
CFTR function and prospects for therapy.
@en
CFTR function and prospects for therapy.
@nl
prefLabel
CFTR function and prospects for therapy.
@en
CFTR function and prospects for therapy.
@nl
P1476
CFTR function and prospects for therapy.
@en
P2093
John R Riordan
P304
P356
10.1146/ANNUREV.BIOCHEM.75.103004.142532
P577
2008-01-01T00:00:00Z