The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
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Mechanisms of CFTR Folding at the Endoplasmic ReticulumMolecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisDeletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperonePerturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasomeThe Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesisThe human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP.Disease-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 channelsThe cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulatorThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyDistinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast.CFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisInteraction of Hsp90 with 20S proteasome: thermodynamic and kinetic characterizationAggresomes: a cellular response to misfolded proteinsInvolvement of the Cdc42 pathway in CFTR post-translational turnover and in its plasma membrane stability in airway epithelial cellsRescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementationActivation of chloride transport in CF airway epithelial cell lines and primary CF nasal epithelial cells by S-nitrosoglutathione.Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.Novel pharmacologic therapies for cystic fibrosisSyntaxin 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 glycoproteinInterplay between ER exit code and domain conformation in CFTR misprocessing and rescue.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesComplement yourself: Transcomplementation rescues partially folded mutant proteins.A mild variant of cystic fibrosis.Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexinDeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Clinical and pharmacological experience with LJP-394.Applications of proteomic technologies for understanding the premature proteolysis of CFTR.Pharmacological treatment of the ion transport defect in cystic fibrosis.Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator.Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.Glycoprotein folding in the endoplasmic reticulum.Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.Dexamethasone regulates CFTR expression in Calu-3 cells with the involvement of chaperones HSP70 and HSP90.Degradation of unassembled Vph1p reveals novel aspects of the yeast ER quality control system.Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosisERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.
P2860
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P2860
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
description
1993 nî lūn-bûn
@nan
1993年の論文
@ja
1993年論文
@yue
1993年論文
@zh-hant
1993年論文
@zh-hk
1993年論文
@zh-mo
1993年論文
@zh-tw
1993年论文
@wuu
1993年论文
@zh
1993年论文
@zh-cn
name
The common variant of cystic f ...... Golgi nonlysosomal compartment
@ast
The common variant of cystic f ...... Golgi nonlysosomal compartment
@en
type
label
The common variant of cystic f ...... Golgi nonlysosomal compartment
@ast
The common variant of cystic f ...... Golgi nonlysosomal compartment
@en
prefLabel
The common variant of cystic f ...... Golgi nonlysosomal compartment
@ast
The common variant of cystic f ...... Golgi nonlysosomal compartment
@en
P2093
P2860
P356
P1476
The common variant of cystic f ...... Golgi nonlysosomal compartment
@en
P2093
P2860
P304
P356
10.1073/PNAS.90.20.9480
P407
P577
1993-10-01T00:00:00Z