Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
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Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisAn aquaporin-2 water channel mutant which causes autosomal dominant nephrogenic diabetes insipidus is retained in the Golgi complexIdentification of mutations in the putative ATP-binding domain of the adrenoleukodystrophy geneMolecular pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts: mutations in MLC1 cause folding defectsGlycosylation and the cystic fibrosis transmembrane conductance regulatorRoad to ruin: targeting proteins for degradation in the endoplasmic reticulumThe Evolution of Cystic Fibrosis CareThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyDefining the glycan destruction signal for endoplasmic reticulum-associated degradationCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesABCG2: the molecular mechanisms of urate secretion and goutPharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutantsIdentification of human Kir2.2 (KCNJ12) gene encoding functional inward rectifier potassium channel in both mammalian cells and Xenopus oocytesPhenanthrolines--a new class of CFTR chloride channel openersDCPIB is a novel selective blocker of I(Cl,swell) and prevents swelling-induced shortening of guinea-pig atrial action potential durationPseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cellsCharacterization of Dent's disease mutations of CLC-5 reveals a correlation between functional and cell biological consequences and protein structure.Concentration-dependent effects of endogenous S-nitrosoglutathione on gene regulation by specificity proteins Sp3 and Sp1.Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.4'-Methyl-4,5'-bithiazole-based correctors of defective delta F508-CFTR cellular processing.Syntaxin 1A inhibits CFTR chloride channels by means of domain-specific protein-protein interactionsCystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoproteinGenetic variation and clinical heterogeneity in cystic fibrosisCystic fibrosis: exploiting its genetic basis in the hunt for new therapiesStructure and function of the cystic fibrosis transmembrane conductance regulator.Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR)DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Nonsense mutation R1162X of the cystic fibrosis transmembrane conductance regulator gene does not reduce messenger RNA expression in nasal epithelial tissue.Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.Cystic 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.Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes.Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents.Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype.Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells.Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the poreRegulation of recombinant cardiac cystic fibrosis transmembrane conductance regulator chloride channels by protein kinase C.Water channels encoded by mutant aquaporin-2 genes in nephrogenic diabetes insipidus are impaired in their cellular routing.Regulation of male fertility by CFTR and implications in male infertility.
P2860
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P2860
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
description
1991 nî lūn-bûn
@nan
1991年の論文
@ja
1991年学术文章
@wuu
1991年学术文章
@zh
1991年学术文章
@zh-cn
1991年学术文章
@zh-hans
1991年学术文章
@zh-my
1991年学术文章
@zh-sg
1991年學術文章
@yue
1991年學術文章
@zh-hant
name
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@en
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@nl
type
label
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@en
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@nl
prefLabel
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@en
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@nl
P2093
P356
P1433
P1476
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
@en
P2093
Collins FS
Frizzell RA
Wilkinson DJ
Worrell RT
P304
P356
10.1126/SCIENCE.1722350
P407
P577
1991-12-01T00:00:00Z