Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
about
Folding efficiency is rate-limiting in dopamine D4 receptor biogenesisThe 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-16The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulatorMisfolding diverts CFTR from recycling to degradation: quality control at early endosomesThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyIn vivo action of the HRD ubiquitin ligase complex: mechanisms of endoplasmic reticulum quality control and sterol regulation.Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesMolecular etiology of primary hyperoxaluria type 1: new directions for treatmentPathophysiology of gene-targeted mouse models for cystic fibrosisMissense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicingCorrection of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effectsThe primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domainStabilization of the tertiary structure of the cholera toxin A1 subunit inhibits toxin dislocation and cellular intoxicationIdentification and glycerol-induced correction of misfolding mutations in the X-linked mental retardation gene CASKNative polycystin 2 functions as a plasma membrane Ca2+-permeable cation channel in renal epitheliaSLC30A10 is a cell surface-localized manganese efflux transporter, and parkinsonism-causing mutations block its intracellular trafficking and efflux activityThe cystic fibrosis transmembrane conductance regulator (CFTR) and its stabilityRestoration of the majority of the visual spectrum by using modified Volvox channelrhodopsin-1.Proper folding and endoplasmic reticulum to golgi transport of tyrosinase are induced by its substrates, DOPA and tyrosine.Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.Hypoxia increases corneal cell expression of CFTR leading to increased Pseudomonas aeruginosa binding, internalization, and initiation of inflammationAdditive effect of multiple pharmacological chaperones on maturation of CFTR processing mutantsCalnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.Novel pharmacologic therapies for cystic fibrosisRole of tyrosine 33 residue for the stabilization of the tetrameric structure of human cytidine deaminase.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesPharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Antagonists to the rescuePhosphorylation of CFTR by PKA promotes binding of the regulatory domainDeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.Clinical and pharmacological experience with LJP-394.Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.Endoplasmic reticulum quality control is involved in the mechanism of endoglin-mediated hereditary haemorrhagic telangiectasia.CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation
P2860
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P2860
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
description
1996 nî lūn-bûn
@nan
1996年の論文
@ja
1996年学术文章
@wuu
1996年学术文章
@zh-cn
1996年学术文章
@zh-hans
1996年学术文章
@zh-my
1996年学术文章
@zh-sg
1996年學術文章
@yue
1996年學術文章
@zh
1996年學術文章
@zh-hant
name
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@en
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@nl
type
label
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@en
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@nl
prefLabel
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@en
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@nl
P2093
P356
P1476
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
@en
P2093
P304
P356
10.1074/JBC.271.2.635
P407
P577
1996-01-01T00:00:00Z