about
Endoplasmic reticulum retention is a common defect associated with tyrosinase-negative albinismBAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP.The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulatorA surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activationMisfolding diverts CFTR from recycling to degradation: quality control at early endosomesThe PEST sequence does not contribute to the stability of the cystic fibrosis transmembrane conductance regulatorIslet-intrinsic effects of CFTR mutationNPGPx (GPx7): a novel oxidative stress sensor/transmitter with multiple roles in redox homeostasisProtein folding and quality control in the ERMolecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosisLighting the fires within: the cell biology of autoinflammatory diseasesCrystal structure of a class I α1,2-mannosidase involved in N-glycan processing and endoplasmic reticulum quality controlIdentification of human Kir2.2 (KCNJ12) gene encoding functional inward rectifier potassium channel in both mammalian cells and Xenopus oocytesAugmentation 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.Intracellular aggregation of polypeptides with expanded polyglutamine domain is stimulated by stress-activated kinase MEKK1.Activation of chloride transport in CF airway epithelial cell lines and primary CF nasal epithelial cells by S-nitrosoglutathione.ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy.Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTRNovel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.The calpain, caspase 12, caspase 3 cascade leading to apoptosis is altered in F508del-CFTR expressing cellsModulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial culturesTLR-4-mediated innate immunity is reduced in cystic fibrosis airway cells.Elevated miR-155 promotes inflammation in cystic fibrosis by driving hyperexpression of interleukin-8Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.Identification of a trafficking determinant localized to the Kv1 potassium channel poreRestoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTRThe cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.Salicylic acid signaling controls the maturation and localization of the arabidopsis defense protein ACCELERATED CELL DEATH6Transmembrane segments prevent surface expression of sodium channel Nav1.8 and promote calnexin-dependent channel degradationUse of kinase inhibitors to correct ΔF508-CFTR function.The CFTR frameshift mutation 3905insT and its effect at transcript and protein level.CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners.Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulatorFrom the cradle to the grave: molecular chaperones that may choose between folding and degradation.Beta-2-adrenergic receptor polymorphisms in cystic fibrosisMAPK signaling pathways regulate IL-8 mRNA stability and IL-8 protein expression in cystic fibrosis lung epithelial cell lines.Calpain inhibition promotes the rescue of F(508)del-CFTR in PBMC from cystic fibrosis patients.Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis
P2860
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P2860
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年学术文章
@wuu
1999年学术文章
@zh-cn
1999年学术文章
@zh-hans
1999年学术文章
@zh-my
1999年学术文章
@zh-sg
1999年學術文章
@yue
1999年學術文章
@zh
1999年學術文章
@zh-hant
name
Biosynthesis and degradation of CFTR.
@en
Biosynthesis and degradation of CFTR.
@nl
type
label
Biosynthesis and degradation of CFTR.
@en
Biosynthesis and degradation of CFTR.
@nl
prefLabel
Biosynthesis and degradation of CFTR.
@en
Biosynthesis and degradation of CFTR.
@nl
P1476
Biosynthesis and degradation of CFTR.
@en
P2093
R R Kopito
P304
P356
10.1152/PHYSREV.1999.79.1.S167
P433
P577
1999-01-01T00:00:00Z