Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression.
about
Clinical and experimental applications of sodium phenylbutyrateGlycosylation and the cystic fibrosis transmembrane conductance regulatorBinding of serum response factor to cystic fibrosis transmembrane conductance regulator CArG-like elements, as a new potential CFTR transcriptional regulation pathway.NT5E mutations that cause human disease are associated with intracellular mistrafficking of NT5E proteinImpact of heterogeneity within cultured cells on bacterial invasion: analysis of Pseudomonas aeruginosa and Salmonella enterica serovar typhi entry into MDCK cells by using a green fluorescent protein-labelled cystic fibrosis transmembrane conductanNovel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.Novel pharmacologic therapies for cystic fibrosisFunctional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation.Effects of cystic fibrosis and congenital bilateral absence of the vas deferens-associated mutations on cystic fibrosis transmembrane conductance regulator-mediated regulation of separate channelsIntracellular transport, assembly, and degradation of wild-type and disease-linked mutant gap junction proteins.Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosisMislocalization of fukutin protein by disease-causing missense mutations can be rescued with treatments directed at folding ameliorationMetagenomic evidence for taxonomic dysbiosis and functional imbalance in the gastrointestinal tracts of children with cystic fibrosis.A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator.Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolismAutophagy failure in Alzheimer's disease and the role of defective lysosomal acidificationIn vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue.Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels.Trafficking of GFP-tagged DeltaF508-CFTR to the plasma membrane in a polarized epithelial cell line.Induction of HSP70 promotes DeltaF508 CFTR trafficking.A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.Abnormal CFTR Affects Glucagon Production by Islet α Cells in Cystic Fibrosis and Polycystic Ovarian Syndrome.
P2860
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P2860
Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression.
description
1995 nî lūn-bûn
@nan
1995 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1995 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1995年の論文
@ja
1995年論文
@yue
1995年論文
@zh-hant
1995年論文
@zh-hk
1995年論文
@zh-mo
1995年論文
@zh-tw
1995年论文
@wuu
name
Functional activation of the c ...... a F508-CFTR by overexpression.
@ast
Functional activation of the c ...... a F508-CFTR by overexpression.
@en
Functional activation of the c ...... a F508-CFTR by overexpression.
@nl
type
label
Functional activation of the c ...... a F508-CFTR by overexpression.
@ast
Functional activation of the c ...... a F508-CFTR by overexpression.
@en
Functional activation of the c ...... a F508-CFTR by overexpression.
@nl
prefLabel
Functional activation of the c ...... a F508-CFTR by overexpression.
@ast
Functional activation of the c ...... a F508-CFTR by overexpression.
@en
Functional activation of the c ...... a F508-CFTR by overexpression.
@nl
P2093
P1476
Functional activation of the c ...... a F508-CFTR by overexpression.
@en
P2093
Jefferson DM
Marshall J
Schiavi SC
P304
P356
10.1152/AJPLUNG.1995.268.4.L615
P407
P433
P577
1995-04-01T00:00:00Z