In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
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Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisCorrectors (specific therapies for class II CFTR mutations) for cystic fibrosisEndoplasmic reticulum protein quality control is determined by cooperative interactions between Hsp/c70 protein and the CHIP E3 ligaseDisease-associated mutations affect intracellular traffic and paracellular Mg2+ transport function of Claudin-16Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channelsChloride channels as drug targets.Clinical and experimental applications of sodium phenylbutyrateReduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosisThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyExpression and in vivo rescue of human ABCC6 disease-causing mutants in mouse liverCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesA trafficking-deficient mutant of KCC3 reveals dominant-negative effects on K-Cl cotransport functionNT5E mutations that cause human disease are associated with intracellular mistrafficking of NT5E proteinFunctional Rescue of Trafficking-Impaired ABCB4 Mutants by Chemical ChaperonesRestoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell modelSLC30A10 is a cell surface-localized manganese efflux transporter, and parkinsonism-causing mutations block its intracellular trafficking and efflux activityAugmentation of CFTR maturation by S-nitrosoglutathione reductase.Cryptdin-3 induces novel apical conductance(s) in Cl- secretory, including cystic fibrosis, epithelia.Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.Identification of rab20 as a potential regulator of connexin 43 trafficking.Novel pharmacologic therapies for cystic fibrosisFunctional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation.Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesER retention is imposed by COPII protein sorting and attenuated by 4-phenylbutyrate.DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.CFTR Modulators for the Treatment of Cystic Fibrosis.Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.Clinical and pharmacological experience with LJP-394.Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosisApplications of proteomic technologies for understanding the premature proteolysis of CFTR.Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulatorPharmacological treatment of the ion transport defect in cystic fibrosis.Role of endoplasmic reticulum stress in acrolein-induced endothelial activationObesity-linked variants of melanocortin-4 receptor are misfolded in the endoplasmic reticulum and can be rescued to the cell surface by a chemical chaperone.Histone deacetylase: a potential therapeutic target for fibrotic disordersRescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.ERp29 regulates epithelial sodium channel functional expression by promoting channel cleavage.Regulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells.
P2860
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P2860
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
description
1997 nî lūn-bûn
@nan
1997年の論文
@ja
1997年学术文章
@wuu
1997年学术文章
@zh-cn
1997年学术文章
@zh-hans
1997年学术文章
@zh-my
1997年学术文章
@zh-sg
1997年學術文章
@yue
1997年學術文章
@zh
1997年學術文章
@zh-hant
name
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@en
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@nl
type
label
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@en
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@nl
prefLabel
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@en
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@nl
P2093
P2860
P356
P1476
In vitro pharmacologic restora ...... ls containing delta F508-CFTR.
@en
P2093
P L Zeitlin
R C Rubenstein
P2860
P304
P356
10.1172/JCI119788
P407
P577
1997-11-01T00:00:00Z