Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin
about
Molecular 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 chaperoneERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein CRegulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisNovel adiponectin variants identified in type 2 diabetic patients reveal multimerization and secretion defectsCystic fibrosis: exploiting its genetic basis in the hunt for new therapiesUbiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity.Applications of proteomic technologies for understanding the premature proteolysis of CFTR.A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein.Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.Transmembrane segments prevent surface expression of sodium channel Nav1.8 and promote calnexin-dependent channel degradationVX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.Synthesis, Processing, and Function of N-glycans in N-glycoproteins.Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanismDetection of phospho-sites generated by protein kinase CK2 in CFTR: mechanistic aspects of Thr1471 phosphorylationERp29 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.Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulatorEndoplasmic reticulum stress decreases intracellular thyroid hormone activation via an eIF2a-mediated decrease in type 2 deiodinase synthesis.Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation.IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsinEndoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.A novel approach to recovery of function of mutant proteins by slowing down translation.In silico search for modifier genes associated with pancreatic and liver disease in Cystic FibrosisGlucose-regulated protein 94 triage of mutant myocilin through endoplasmic reticulum-associated degradation subverts a more efficient autophagic clearance mechanism.Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.Cystic fibrosis transmembrane conductance regulator controls lung proteasomal degradation and nuclear factor-kappaB activity in conditions of oxidative stress.Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells.The protective and destructive roles played by molecular chaperones during ERAD (endoplasmic-reticulum-associated degradation).Consequences of individual N-glycan deletions and of proteasomal inhibition on secretion of active BACE.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 MSD2Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR traffickingN-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic.Identification of GABA(C) receptor protein homeostasis network components from three tandem mass spectrometry proteomics approaches.Glycoprotein folding and quality-control mechanisms in protein-folding diseases
P2860
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P2860
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin
description
2005 nî lūn-bûn
@nan
2005 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Most F508del-CFTR is targeted ...... and independently of calnexin
@ast
Most F508del-CFTR is targeted ...... and independently of calnexin
@en
type
label
Most F508del-CFTR is targeted ...... and independently of calnexin
@ast
Most F508del-CFTR is targeted ...... and independently of calnexin
@en
prefLabel
Most F508del-CFTR is targeted ...... and independently of calnexin
@ast
Most F508del-CFTR is targeted ...... and independently of calnexin
@en
P2860
P1476
Most F508del-CFTR is targeted ...... and independently of calnexin
@en
P2093
Carlos M Farinha
Margarida D Amaral
P2860
P304
P356
10.1128/MCB.25.12.5242-5252.2005
P407
P577
2005-06-01T00:00:00Z