Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.
about
Therapeutic rescue of misfolded mutants: validation of primary high throughput screens for identification of pharmacoperone drugsPharmacoperone Identification for Therapeutic Rescue of Misfolded Mutant ProteinsGlycopeptide-preferring polypeptide GalNAc transferase 10 (ppGalNAc T10), involved in mucin-type O-glycosylation, has a unique GalNAc-O-Ser/Thr-binding site in its catalytic domain not found in ppGalNAc T1 or T2Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiencyThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyThe primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domainChemical chaperones improve protein secretion and rescue mutant factor VIII in mice with hemophilia ATrafficking and quality control of the gonadotropin releasing hormone receptor in health and diseaseIdentifying protein stabilizing ligands using GroEL.A phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptorTransitioning pharmacoperones to therapeutic use: in vivo proof-of-principle and design of high throughput screens.Chaperoning G protein-coupled receptors: from cell biology to therapeuticsMultispectral image analysis of binary encoded microspheres for highly multiplexed suspension arrays.Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells.The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.Pharmacological chaperones for misfolded gonadotropin-releasing hormone receptorsAssay strategies for identification of therapeutic leads that target protein traffickingEffects of cellular, chemical, and pharmacological chaperones on the rescue of a trafficking-defective mutant of the ATP-binding cassette transporter proteins ABCB1/ABCB4.Side chain and backbone contributions of Phe508 to CFTR folding.Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR traffickingMechanism-based corrector combination restores ΔF508-CFTR folding and function.Chemical chaperone and inhibitor discovery: potential treatments for protein conformational diseases.Application of metabolomics approaches to the study of respiratory diseases.BMP type II receptor as a therapeutic target in pulmonary arterial hypertension.Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.Benzbromarone stabilizes ΔF508 CFTR at the cell surface.Chemical conjugation of DeltaF508-CFTR corrector deoxyspergualin to transporter human serum albumin enhances its ability to rescue Cl- channel functions.Biochemical and biophysical approaches to probe CFTR structure.Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1.Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.Determination of transport stoichiometry for two cation-coupled myo-inositol cotransporters: SMIT2 and HMIT.Failure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: potential for rescue.Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.Novel down-regulatory mechanism of the surface expression of the vasopressin V2 receptor by an alternative splice receptor variant.
P2860
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P2860
Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.
description
2003 nî lūn-bûn
@nan
2003 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
Organic solutes rescue the fun ...... embrane conductance regulator.
@ast
Organic solutes rescue the fun ...... embrane conductance regulator.
@en
type
label
Organic solutes rescue the fun ...... embrane conductance regulator.
@ast
Organic solutes rescue the fun ...... embrane conductance regulator.
@en
prefLabel
Organic solutes rescue the fun ...... embrane conductance regulator.
@ast
Organic solutes rescue the fun ...... embrane conductance regulator.
@en
P2093
P2860
P356
P1476
Organic solutes rescue the fun ...... membrane conductance regulator
@en
P2093
Hongwen Yue
Philip J Thomas
Sandra E Guggino
Steve W Leung
Xi-Tao Wang
Xue-Mei Zhang
P2860
P304
51232-51242
P356
10.1074/JBC.M309076200
P407
P577
2003-10-07T00:00:00Z