C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation.
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The 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 Hsp70Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinaseMisfolding diverts CFTR from recycling to degradation: quality control at early endosomesMolecular analysis using DHPLC of cystic fibrosis: increase of the mutation detection rate among the affected population in Central ItalyNew and emerging targeted therapies for cystic fibrosisCystic fibrosis chronic rhinosinusitis: a comprehensive reviewCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesA novel frameshift mutation in exon 23 of ATP7A (MNK) results in occipital horn syndrome and not in Menkes diseaseLiver disease in cystic fibrosis: an updateTracking of quantum dot-labeled CFTR shows near immobilization by C-terminal PDZ interactionsSingle-particle tracking of membrane protein diffusion in a potential: simulation, detection, and application to confined diffusion of CFTR Cl- channels.Clinical and molecular characterization of S1118F-CFTR.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesCystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosisThe CFTR frameshift mutation 3905insT and its effect at transcript and protein level.Genetics of acute and chronic pancreatitis: An update.Towards the pharmacogenomics of cystic fibrosis.Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epitheliaOrphan missense mutations in the cystic fibrosis transmembrane conductance regulator: A three-step biological approach to establishing a correlation between genotype and phenotypeThe phenotypic consequences of CFTR mutations.Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients.Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR.Emerging drug treatments for cystic fibrosis.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Toward the pharmacogenomics of cystic fibrosis--an update.Endocytic trafficking of CFTR in health and disease.COOH-terminal truncations promote proteasome-dependent degradation of mature cystic fibrosis transmembrane conductance regulator from post-Golgi compartments.Keratin K18 increases cystic fibrosis transmembrane conductance regulator (CFTR) surface expression by binding to its C-terminal hydrophobic patch.Reduced PDZ interactions of rescued ΔF508CFTR increases its cell surface mobility.From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.Novel CFTR Mutations in Two Iranian Families with Severe Cystic Fibrosis.Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane.Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors.Disease-modifying genes and monogenic disorders: experience in cystic fibrosis.Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.Pharmacogenetics of cystic fibrosis treatment.On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator.Ezrin controls the macromolecular complexes formed between an adapter protein Na+/H+ exchanger regulatory factor and the cystic fibrosis transmembrane conductance regulator.CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies.The role of the C terminus and Na+/H+ exchanger regulatory factor in the functional expression of cystic fibrosis transmembrane conductance regulator in nonpolarized cells and epithelia.
P2860
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P2860
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
1999年论文
@zh
1999年论文
@zh-cn
name
C-terminal truncations destabi ...... is. A novel class of mutation.
@en
type
label
C-terminal truncations destabi ...... is. A novel class of mutation.
@en
prefLabel
C-terminal truncations destabi ...... is. A novel class of mutation.
@en
P2093
P2860
P356
P1476
C-terminal truncations destabi ...... is. A novel class of mutation.
@en
P2093
Benharouga M
Lechardeur D
P2860
P304
21873-21877
P356
10.1074/JBC.274.31.21873
P407
P577
1999-07-01T00:00:00Z