In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium
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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 Hsp70Pathophysiology of gene-targeted mouse models for cystic fibrosisINO-4995 therapeutic efficacy is enhanced with repeat dosing in cystic fibrosis knockout mice and human epithelia.Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6.DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Pharmacotherapy of the ion transport defect in cystic fibrosis.Marked repression of CFTR mRNA in the transgenic Cftr(tm1kth) mouse model.Processing and function of CFTR-DeltaF508 are species-dependent.Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.Role of oxygen availability in CFTR expression and function.Generation of a conditional null allele for Cftr in mice.Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells.Mis-trafficking of bicarbonate transporters: implications to human diseases.Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency.In vivo alterations of IFN regulatory factor-1 and PIAS1 protein levels in cystic fibrosis epithelium.Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.A(2) adenosine receptors regulate CFTR through PKA and PLA(2).What happens to deltaF508 in vivo?Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression.Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein.PDE5 Inhibitors as Potential Tools in the Treatment of Cystic Fibrosis.Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue.A cyclic nucleotide PDE5 inhibitor corrects defective mucin secretion in submandibular cells containing antibody directed against the cystic fibrosis transmembrane conductance regulator protein.Examining basal chloride transport using the nasal potential difference response in a murine model.Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation.CFTR involvement in nasal potential differences in mice and pigs studied using a thiazolidinone CFTR inhibitor.Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice.Defective respiratory amiloride-sensitive sodium transport predisposes to pulmonary oedema and delays its resolution in mice.Pharmacology of CFTR chloride channel activity.
P2860
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P2860
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium
description
1997 nî lūn-bûn
@nan
1997年の論文
@ja
1997年論文
@yue
1997年論文
@zh-hant
1997年論文
@zh-hk
1997年論文
@zh-mo
1997年論文
@zh-tw
1997年论文
@wuu
1997年论文
@zh
1997年论文
@zh-cn
name
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@ast
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@en
type
label
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@ast
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@en
prefLabel
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@ast
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@en
P2093
P2860
P356
P1476
In vivo activation of the cyst ...... 508 in murine nasal epithelium
@en
P2093
P2860
P304
P356
10.1073/PNAS.94.6.2604
P407
P577
1997-03-01T00:00:00Z