Mouse models of cystic fibrosis: phenotypic analysis and research applications (Q37887172)

24 August 2017

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title

Mouse models of cystic fibrosis: phenotypic analysis and research applications (English)

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24 August 2017

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6

Lhousseine Touqui

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24 August 2017

Martina Wilke

1

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24 August 2017

Ruvalic M Buijs-Offerman

2

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24 August 2017

Jamil Aarbiou

3

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24 August 2017

William H Colledge

4

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24 August 2017

David N Sheppard

5

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24 August 2017

Alice Bot

7

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24 August 2017

Huub Jorna

8

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24 August 2017

Hugo R de Jonge

9

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24 August 2017

Bob J Scholte

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

published in

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24 August 2017

volume

10 Suppl 2

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24 August 2017

page(s)

S152-71

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Pathophysiology of gene-targeted mouse models for cystic fibrosis

24 August 2017

cites work

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Animal models of cystic fibrosis

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Cystic fibrosis mouse models

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The porcine lung as a potential model for cystic fibrosis

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Pathology of gastrointestinal organs in a porcine model of cystic fibrosis

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A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo

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Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.

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Generation of a conditional null allele for Cftr in mice

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

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

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

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

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7 January 2021

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

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7 January 2021

Abnormal enamel development in a cystic fibrosis transgenic mouse model.

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7 January 2021

Sex differences in mouse models of asthma

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7 January 2021

Phenotypic abnormalities in long-term surviving cystic fibrosis mice

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7 January 2021

Impact of nutrition on phenotype in CFTR-deficient mice

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7 January 2021

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

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7 January 2021

Fat absorption in cystic fibrosis mice is impeded by defective lipolysis and post-lipolytic events

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7 January 2021

Expression of Intestinal and Lung Alkaline Sphingomyelinase and Neutral Ceramidase in Cystic Fibrosis F508del Transgenic Mice

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7 January 2021

The phenotypic consequences of CFTR mutations

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7 January 2021

CFTR pharmacology and its role in intestinal fluid secretion

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7 January 2021

Murine CFTR channel and its role in regulatory volume decrease of small intestine crypts

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7 January 2021

Ion transport in the small intestine

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7 January 2021

Electrolyte transport in the mammalian colon: mechanisms and implications for disease

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7 January 2021

Differential expression of calcium-activated chloride channels (CLCA) gene family members in the small intestine of cystic fibrosis mouse models.

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7 January 2021

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7 January 2021

Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion

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7 January 2021

Increased levels of mucins in the cystic fibrosis mouse small intestine, and modulator effects of the Muc1 mucin expression.

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7 January 2021

Spontaneous rescue from cystic fibrosis in a mouse model

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7 January 2021

Salivary secretion assay for drug efficacy for cystic fibrosis in mice

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Stimulation of salivary secretion in vivo by CFTR potentiators in Cftr+/+ and Cftr−/− mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Cystic fibrosis is associated with a defect in apical receptor-mediated endocytosis in mouse and human kidney

1 reference

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7 January 2021

CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis

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7 January 2021

Hepatobiliary complications of cystic fibrosis

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7 January 2021

Cystic fibrosis transmembrane conductance regulator mediates the cyclic adenosine monophosphate-induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium

1 reference

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7 January 2021

CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice

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7 January 2021

Evidence that CFTR is expressed in rat tracheal smooth muscle cells and contributes to bronchodilation.

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7 January 2021

Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries

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7 January 2021

Role of the cystic fibrosis transmembrane conductance channel in human airway smooth muscle

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7 January 2021

CFTR regulates phagosome acidification in macrophages and alters bactericidal activity

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages

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7 January 2021

Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis

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7 January 2021

Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles

1 reference

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7 January 2021

Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis

1 reference

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7 January 2021

Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator-/- mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Inflammation of the cystic fibrosis mouse small intestine

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Influence of the cystic fibrosis transmembrane conductance regulator on expression of lipid metabolism-related genes in dendritic cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Functional expression of cystic fibrosis transmembrane conductance regulator in mouse chondrocytes.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl- secretory channel in mouse airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Loss of TMEM16A causes a defect in epithelial Ca2+-dependent chloride transport

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Studies on expression and function of the TMEM16A calcium-activated chloride channel

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Severe osteopenia in CFTR-null mice

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Murine model for cystic fibrosis bone disease demonstrates osteopenia and sex-related differences in bone formation

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The prevalence of osteoporosis, osteopenia, and fractures among adults with cystic fibrosis: a systematic literature review with meta-analysis

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7 January 2021

Update on cystic fibrosis-related bone disease: a special focus on children

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7 January 2021

Nasal abnormalities in cystic fibrosis mice independent of infection and inflammation

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7 January 2021

Olfactory epithelia exhibit progressive functional and morphological defects in CF mice

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7 January 2021

Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers

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7 January 2021

Cystic fibrosis: a disease of vulnerability to airway surface dehydration.

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7 January 2021

Morituri te salutant? Olfactory signal transduction and the role of phosphoinositides

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7 January 2021

Phosphatidyl-inositide signalling proteins in a novel class of sensory cells in the mammalian olfactory epithelium

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7 January 2021

Substance P stimulates CFTR-dependent fluid secretion by mouse tracheal submucosal glands

1 reference

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7 January 2021

Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice

1 reference

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7 January 2021

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

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7 January 2021

Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice

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7 January 2021

Chloride secretion in the trachea of null cystic fibrosis mice: the effects of transfection with pTrial10-CFTR2.

1 reference

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7 January 2021

Expression cloning of TMEM16A as a calcium-activated chloride channel subunit

1 reference

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7 January 2021

TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

TMEM16A confers receptor-activated calcium-dependent chloride conductance

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Distribution of ion transport mRNAs throughout murine nose and lung

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Mucin is produced by clara cells in the proximal airways of antigen-challenged mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

SPDEF regulates goblet cell hyperplasia in the airway epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Regulated airway goblet cell mucin secretion.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Clara cell impact in air-side activation of CFTR in small pulmonary airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

CFTR-like chloride channels in non-ciliated bronchiolar epithelial (Clara) cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Cystic fibrosis lung disease starts in the small airways: can we treat it more effectively?

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Alveolar epithelium: role in lung fluid balance and acute lung injury

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

G551D Cystic Fibrosis Mice Exhibit Abnormal Regulation of Inflammation in Lungs and Macrophages

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Lung disease in mice with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

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%2960020-9

7 January 2021

Fenretinide corrects newly found ceramide deficiency in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primary-cultured epithelial cells from cystic fibrosis mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Response to acute lung infection with mucoid Pseudomonas aeruginosa in cystic fibrosis mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pseudomonas aeruginosaMicroevolution during Cystic Fibrosis Lung Infection Establishes Clones with Adapted Virulence

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Novel experimental Pseudomonas aeruginosa lung infection model mimicking long-term host-pathogen interactions in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Hypersusceptibility of cystic fibrosis mice to chronic Pseudomonas aeruginosa oropharyngeal colonization and lung infection

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Resistance to Pseudomonas aeruginosa chronic lung infection requires cystic fibrosis transmembrane conductance regulator-modulated interleukin-1 (IL-1) release and signaling through the IL-1 receptor

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pseudomonas aeruginosa LPS or flagellin are sufficient to activate TLR-dependent signaling in murine alveolar macrophages and airway epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Role of Toll-like receptor 5 in the innate immune response to acute P. aeruginosa pneumonia

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The relationship of chronic mucin secretion to airway disease in normal and CFTR-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Critical role of cytosolic phospholipase A2{alpha} in bronchial mucus hypersecretion in CFTR-deficient mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(-/-) mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Fatty acids in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Cystic fibrosis fatty acid imbalance is linked to ceramide deficiency and corrected by fenretinide.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Fenretinide prevents the development of osteoporosis in Cftr-KO mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Proteomic analysis of naphthalene-induced airway epithelial injury and repair in a cystic fibrosis mouse model.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Eicosanoid release is increased by membrane destabilization and CFTR inhibition in Calu-3 cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Acid sphingomyelinase inhibitors normalize pulmonary ceramide and inflammation in cystic fibrosis

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Therapeutic efficacy and safety of amitriptyline in patients with cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pharmacological therapy for cystic fibrosis: from bench to bedside

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Structure and function of the CFTR chloride channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Regulation of murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in Chinese hamster ovary cells.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Diversity of the basic defect of homozygous CFTR mutation genotypes in humans

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Update on gene modifiers in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Modifier genetics: cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Modifier genes in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Amelioration of intestinal disease severity in cystic fibrosis mice is associated with improved chloride secretory capacity

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Very mild disease phenotype of congenic CftrTgH(neoim)Hgu cystic fibrosis mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Mapping of genetic factors influencing the weight of cystic fibrosis knockout mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Potential genetic modifiers of the cystic fibrosis intestinal inflammatory phenotype on mouse chromosomes 1, 9, and 10

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

An animal model for cystic fibrosis made by gene targeting

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Production of a severe cystic fibrosis mutation in mice by gene targeting

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Severe phenotype in mice with termination mutation in exon 2 of cystic fibrosis gene

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

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%2960020-9

7 January 2021

A mouse model for the delta F508 allele of cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Targeted replacement of normal and mutant CFTR sequences in human airway epithelial cells using DNA fragments

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Genotype and phenotype in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Lung infections associated with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Physiology and pathology of tracheobronchial glands

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Angiogenesis in paediatric airway disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pediatric surgical issues in meconium disease and cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Gastrointestinal outcomes and confounders in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Talniflumate increases survival in a cystic fibrosis mouse model of distal intestinal obstructive syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Mouse as a model of growth retardation in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Liver disease in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Hepatobiliary disease in patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Ultrasound evaluation of liver disease in cystic fibrosis as part of an annual assessment clinic: a 9-year review

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Decreased peroxisome proliferator activated receptor alpha is associated with bile duct injury in cystic fibrosis transmembrane conductance regulator-/- mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Renal involvement in cystic fibrosis: diseases spectrum and clinical relevance

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Evidence for cystic fibrosis transmembrane conductance regulator-dependent sodium reabsorption in kidney, using Cftr(tm2cam) mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Restoration by intratracheal gene transfer of bicarbonate secretion in cystic fibrosis mouse gallbladder.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Mechanisms of bicarbonate secretion in the pancreatic duct

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Ducts isolated from the pancreas of CFTR-null mice secrete fluid

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

The flow rate and composition of human labial gland saliva

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Secretion of ions and pharmacological responsiveness in the mouse paw sweat gland

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Potential difference measurements of ocular surface Na+ absorption analyzed using an electrokinetic model

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Aquaporins and CFTR in ocular epithelial fluid transport

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

Pulmonary neuroendocrine cells, airway innervation, and smooth muscle are altered in Cftr null mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960020-9

7 January 2021

10.1016/S1569-1993(11)60020-9

Identifiers

DOI

1 reference

24 August 2017

1 reference