Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.
about
Mechanisms of CFTR Folding at the Endoplasmic ReticulumMolecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisHook2 contributes to aggresome formationOS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERADEndoplasmic reticulum protein quality control is determined by cooperative interactions between Hsp/c70 protein and the CHIP E3 ligaseRNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)Endothelin-converting enzyme-like 1 (ECEL1) is present both in the plasma membrane and in 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 Hsp70Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channelsDigitoxin mimics gene therapy with CFTR and suppresses hypersecretion of IL-8 from cystic fibrosis lung epithelial cellsDefective cellular trafficking of missense NPR-B mutants is the major mechanism underlying acromesomelic dysplasia-type MaroteauxSelective destruction of abnormal proteins by ubiquitin-mediated protein quality control degradationThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyPreviously unknown role for the ubiquitin ligase Ubr1 in endoplasmic reticulum-associated protein degradation.Aggresomes: a cellular response to misfolded proteinsPhenanthrolines--a new class of CFTR chloride channel openersMyotonia congenita mutation enhances the degradation of human CLC-1 chloride channelsSteviol reduces MDCK Cyst formation and growth by inhibiting CFTR channel activity and promoting proteasome-mediated CFTR degradationMechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic FibrosisIncreasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule TherapeuticsAugmentation of CFTR maturation by S-nitrosoglutathione reductase.Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.S-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface.Concentration-dependent effects of endogenous S-nitrosoglutathione on gene regulation by specificity proteins Sp3 and Sp1.Activation of chloride transport in CF airway epithelial cell lines and primary CF nasal epithelial cells by S-nitrosoglutathione.Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients.The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa.Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutantsThe 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.Novel pharmacologic therapies for cystic fibrosisFunctional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation.Folding 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 glycoproteinStructural features of the kringle domain determine the intracellular degradation of under-gamma-carboxylated prothrombin: studies of chimeric rat/human prothrombin.Mutational analysis of threonine 402 adjacent to the GXXXG dimerization motif in transmembrane segment 1 of ABCG2.Role for the ubiquitin-proteasome system in the vacuolar degradation of Ste6p, the a-factor transporter in Saccharomyces cerevisiaeUbiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.
P2860
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P2860
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.
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
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-hant
name
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@en
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@nl
type
label
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@en
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@nl
prefLabel
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@en
Intracellular turnover of cyst ...... wild-type and mutant proteins.
@nl
P1476
Intracellular turnover of cyst ...... wild-type and mutant proteins
@en
P2093
P304
25710-25718
P407
P577
1994-10-01T00:00:00Z