Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2
about
Mechanisms of CFTR Folding at the Endoplasmic ReticulumRNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyThe endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508Development of CFTR StructureInterplay between ER exit code and domain conformation in CFTR misprocessing and rescue.Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis.Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTRApplications of proteomic technologies for understanding the premature proteolysis of CFTR.A chaperone trap contributes to the onset of cystic fibrosis.VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.Emergent properties of proteostasis in managing cystic fibrosis.ERp29 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.Increased folding and channel activity of a rare cystic fibrosis mutant with CFTR modulators.Alteration of CFTR transmembrane span integration by disease-causing mutations.4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cellsFK506 binding protein 8 peptidylprolyl isomerase activity manages a late stage of cystic fibrosis transmembrane conductance regulator (CFTR) folding and stabilityMolecular modelling and molecular dynamics of CFTR.Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.Unravelling druggable signalling networks that control F508del-CFTR proteostasisCFTR: folding, misfolding and correcting the ΔF508 conformational defect.Cooperative assembly and misfolding of CFTR domains in vivo.N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic.Mechanism-based corrector combination restores ΔF508-CFTR folding and function.Mechanisms for rescue of correctable folding defects in CFTRDelta F508.Combating cystic fibrosis: in search for CF transmembrane conductance regulator (CFTR) modulators.Functional Rescue of F508del-CFTR Using Small Molecule CorrectorsStabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.Biochemical and biophysical approaches to probe CFTR structure.Analysis of CFTR folding and degradation in transiently transfected cells.Ligand-driven vectorial folding of ribosome-bound human CFTR NBD1Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.Fixing cystic fibrosis by correcting CFTR domain assembly.Synonymous codon usage affects the expression of wild type and F508del CFTR.Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice.A common mechanism for CFTR potentiators.CFTR F508del binds components of the ERAD machinery for ubiquitination and degradationStructural mechanisms of CFTR function and dysfunction.
P2860
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P2860
Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Assembly and misassembly of cy ...... anning domain (MSD) 1 and MSD2
@en
type
label
Assembly and misassembly of cy ...... anning domain (MSD) 1 and MSD2
@en
prefLabel
Assembly and misassembly of cy ...... anning domain (MSD) 1 and MSD2
@en
P2093
P2860
P356
P1476
Assembly and misassembly of cy ...... anning domain (MSD) 1 and MSD2
@en
P2093
Diane E Grove
Douglas M Cyr
Liling Chen
Meredith F N Rosser
P2860
P304
P356
10.1091/MBC.E08-04-0357
P577
2008-08-20T00:00:00Z