Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
about
Mechanisms of CFTR Folding at the Endoplasmic ReticulumPerturbation 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 Hsp70Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosisDefective cellular trafficking of missense NPR-B mutants is the major mechanism underlying acromesomelic dysplasia-type MaroteauxThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyAggresomes: a cellular response to misfolded proteinsThe molecular basis of oculocutaneous albinism type 1 (OCA1): sorting failure and degradation of mutant tyrosinases results in a lack of pigmentationRibosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression DefectThe endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508Development of CFTR StructureER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy.The function of the intermediate compartment in pre-Golgi trafficking involves its stable connection with the centrosomeDeletion of CFTR translation start site reveals functional isoforms of the protein in CF patients.Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTRThe role of the UPS in cystic fibrosis.Function of SSA subfamily of Hsp70 within and across species varies widely in complementing Saccharomyces cerevisiae cell growth and prion propagation.The calpain, caspase 12, caspase 3 cascade leading to apoptosis is altered in F508del-CFTR expressing cellsFolding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteinsCystic 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.Disruption of interleukin-1β autocrine signaling rescues complex I activity and improves ROS levels in immortalized epithelial cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.COMMD1-mediated ubiquitination regulates CFTR trafficking.Solution NMR studies of periplasmic binding proteins and their interaction partnersSmall-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.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.Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.Histone deacetylase inhibitors influence chemotherapy transport by modulating expression and trafficking of a common polymorphic variant of the ABCG2 efflux transporter.The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.Ion channel associated diseases: overview of molecular mechanismsVX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations.Improvement of chloride transport defect by gonadotropin-releasing hormone (GnRH) in cystic fibrosis epithelial cells.Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice.Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapyRevertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.
P2860
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P2860
Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
description
1994 nî lūn-bûn
@nan
1994年の論文
@ja
1994年学术文章
@wuu
1994年学术文章
@zh-cn
1994年学术文章
@zh-hans
1994年学术文章
@zh-my
1994年学术文章
@zh-sg
1994年學術文章
@yue
1994年學術文章
@zh
1994年學術文章
@zh-hant
name
Conformational maturation of C ...... mic reticulum and requires ATP
@en
type
label
Conformational maturation of C ...... mic reticulum and requires ATP
@en
prefLabel
Conformational maturation of C ...... mic reticulum and requires ATP
@en
P2093
P2860
P1433
P1476
Conformational maturation of C ...... mic reticulum and requires ATP
@en
P2093
P2860
P304
P356
10.1002/J.1460-2075.1994.TB06954.X
P407
P577
1994-12-01T00:00:00Z