Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice (Q28255797)

2

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Jack R Harkema

3

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Wanda K O'Neal

4

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Richard C Boucher

Amiloride-sensitive epithelial Na+ channel is made of three homologous subunits

5

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language of work or name

English

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publication date

May 2004

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published in

Nature Medicine

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volume

10

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issue

5

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page(s)

487-93

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cites work

1 reference

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Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

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Loss of function of axonemal dynein Mdnah5 causes primary ciliary dyskinesia and hydrocephalus

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Identification of the cystic fibrosis gene: genetic analysis

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Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

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Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

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Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.

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CFTR as a cAMP-dependent regulator of sodium channels

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Wild type but not deltaF508 CFTR inhibits Na+ conductance when coexpressed in Xenopus oocytes

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The amiloride-inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways.

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Mucus clearance as a primary innate defense mechanism for mammalian airways

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The genesis of cystic fibrosis lung disease

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Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na(+)] and pH but elevated viscosity

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Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease

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Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa

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An animal model for cystic fibrosis made by gene targeting

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Pathophysiology of gene-targeted mouse models for cystic fibrosis

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Cell-specific expression of a Clara cell secretory protein-human growth hormone gene in the bronchiolar epithelium of transgenic mice

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Disruption of disulfide bonds exhibits differential effects on trafficking of regulated secretory proteins

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Regulation of intracellular calcium in human esophageal smooth muscles

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Mucociliary transport determined by in vivo microdialysis in the airways of normal and CF mice.

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Airway remodeling in asthma

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Human airway ion transport. Part two.

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Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

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Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation

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Mucociliary clearance in the airways

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An ultrastructural morphometric analysis of elastase-treated hamster bronchi shows discharge followed by progressive accumulation of secretory granules

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Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line

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Epidermal growth factor system regulates mucin production in airways

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Particulate matter induces cytokine expression in human bronchial epithelial cells

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Lung inflammation early in cystic fibrosis: bugs are indicted, but the defense is guilty.

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The CF salt controversy: in vivo observations and therapeutic approaches.

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Mutation of the mouse hepatocyte nuclear factor/forkhead homologue 4 gene results in an absence of cilia and random left-right asymmetry

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Ciliogenesis and left-right axis defects in forkhead factor HFH-4-null mice

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Hydrocephalus, situs inversus, chronic sinusitis, and male infertility in DNA polymerase lambda-deficient mice: possible implication for the pathogenesis of immotile cilia syndrome

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Physical and functional properties of airway secretions in cystic fibrosis--therapeutic approaches

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Criteria for mucus transport in the airways by two-phase gas-liquid flow mechanism

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Aldosterone-mediated regulation of ENaC alpha, beta, and gamma subunit proteins in rat kidney

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Differential subcellular localization of ENaC subunits in mouse kidney in response to high- and low-Na diets

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Subunit composition determines the single channel kinetics of the epithelial sodium channel

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Association of hypertension with T594M mutation in beta subunit of epithelial sodium channels in black people resident in London

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Targeting expression of a transgene to the airway surface epithelium using a ciliated cell-specific promoter

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Alterations in airway ion transport in NKCC1-deficient mice

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Requirement for IL-13 independently of IL-4 in experimental asthma

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Identifiers

DOI

10.1038/NM1028

1 reference

Consolidated OpenCitations Corpus – April 2017

release_5eksxv4xu5bnzh36p6uxg5v3aa

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/5eksxv4xu5bnzh36p6uxg5v3aa

24 November 2022

mapped directly with Wikidata item

1 reference

Consolidated OpenCitations Corpus – April 2017

PubMed publication ID

15077107

1 reference

Consolidated OpenCitations Corpus – April 2017