The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease (Q37887177)

24 August 2017

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title

The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease (English)

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24 August 2017

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8

Alexander Dalpke

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24 August 2017

Zhe Zhou

1

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24 August 2017

Julia Duerr

2

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24 August 2017

Bjarki Johannesson

3

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24 August 2017

Susanne C Schubert

4

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24 August 2017

Diana Treis

5

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24 August 2017

Maria Harm

6

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24 August 2017

Simon Y Graeber

7

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24 August 2017

Carsten Schultz

9

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24 August 2017

Marcus A Mall

10

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24 August 2017

language of work or name

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1 June 2011

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Journal of Cystic Fibrosis

24 August 2017

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24 August 2017

10 Suppl 2

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24 August 2017

S172-82

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Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening.

24 August 2017

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

An animal model for cystic fibrosis made by gene targeting

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Generation and characterization of a delta F508 cystic fibrosis mouse model.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Pathophysiology of gene-targeted mouse models for cystic fibrosis

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Mouse models of cystic fibrosis: phenotypic analysis and research applications

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Lung disease in mice with cystic fibrosis

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Anomalies in ion transport in CF mouse tracheal epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Mucociliary clearance in patients with cystic fibrosis and in normal subjects

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

CFTR as a cAMP-dependent regulator of sodium channels

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

A mutation in the epithelial sodium channel causing Liddle disease increases channel activity in the Xenopus laevis oocyte expression system

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

The genesis of cystic fibrosis lung disease

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Development of chronic bronchitis and emphysema in beta-epithelial Na+ channel-overexpressing mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Airway and lung pathology due to mucosal surface dehydration in {beta}-epithelial Na+ channel-overexpressing mice: role of TNF-{alpha} and IL-4R{alpha} signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Airway mucus: the good, the bad, the sticky

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Particulate matter induces cytokine expression in human bronchial epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Epidermal growth factor system regulates mucin production in airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Neonatal adaptive immunity comes of age

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Mucus clearance as a primary innate defense mechanism for mammalian airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Real-time polymerase chain reaction for detection and quantification of bacteria in periodontal patients.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

CFTR regulates phagosome acidification in macrophages and alters bactericidal activity

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Genetic modifiers of lung disease in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

The mouse as a model for human biology: a resource guide for complex trait analysis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

A global perspective on newborn screening for cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Airway deposition and clearance and systemic pharmacokinetics of amiloride following aerosolization with an ultrasonic nebulizer to normal airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Persistent activation of an innate immune response translates respiratory viral infection into chronic lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Design, synthesis, and structure-activity relationships of novel 2-substituted pyrazinoylguanidine epithelial sodium channel blockers: drugs for cystic fibrosis and chronic bronchitis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Pharmacological Properties of N-(3,5-Diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine Methanesulfonate (552-02), a Novel Epithelial Sodium Channel Blocker with Potential Clinical Efficacy for Cystic Fibrosis L

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Pharmacological therapy for cystic fibrosis: from bench to bedside

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Finally, mice with CF lung disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960021-0

7 January 2021

10.1016/S1569-1993(11)60021-0

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DOI

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24 August 2017

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