Airway disease phenotypes in animal models of cystic fibrosis. (Q54243366)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29609604%20AND%20SRC:MED&resulttype=core&format=json

22 March 2020

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Airway disease phenotypes in animal models of cystic fibrosis (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29609604%20AND%20SRC:MED&resulttype=core&format=json

22 March 2020

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13 March 2019

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28 January 2021

biomedical investigative technique

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19 September 2019

1 reference

3 December 2020

1 reference

6 February 2021

1 reference

8 April 2021

1

1 reference

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22 March 2020

Martin Donnelley

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22 March 2020

David W Parsons

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22 March 2020

author name string

Alexandra McCarron

1

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http://europepmc.org/abstract/PMC/5879563

26 May 2018

Martin Donnelley

2

1 reference

http://europepmc.org/abstract/PMC/5879563

26 May 2018

David Parsons

3

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http://europepmc.org/abstract/PMC/5879563

26 May 2018

publication date

2 April 2018

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13 March 2019

21 March 2019

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19 September 2019

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22 March 2020

3 December 2020

28 January 2021

6 February 2021

8 April 2021

10 references

13 March 2019

21 March 2019

22 April 2019

20 June 2019

19 September 2019

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29609604%20AND%20SRC:MED&resulttype=core&format=json

22 March 2020

3 December 2020

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8 April 2021

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22 March 2020

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Creative Commons Attribution 4.0 International

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Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs

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The rabbit as a model for studying lung disease and stem cell therapy.

26 May 2018

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26 May 2018

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26 May 2018

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26 May 2018

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26 May 2018

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Sodium channels and cystic fibrosis

26 May 2018

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26 May 2018

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Processing and function of CFTR-DeltaF508 are species-dependent

26 May 2018

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Evidence for airway surface dehydration as the initiating event in CF airway disease

26 May 2018

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Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

26 May 2018

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Bioelectric properties of chloride channels in human, pig, ferret, and mouse airway epithelia

26 May 2018

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26 May 2018

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

26 May 2018

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

26 May 2018

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

26 May 2018

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Cystic fibrosis since 1938.

26 May 2018

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Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia

26 May 2018

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The "Goldilocks effect" in cystic fibrosis: identification of a lung phenotype in the cftr knockout and heterozygous mouse

26 May 2018

1 reference

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

26 May 2018

1 reference

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

26 May 2018

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

26 May 2018

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An overview of the pathogenesis of cystic fibrosis lung disease

26 May 2018

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Mucus clearance as a primary innate defense mechanism for mammalian airways

26 May 2018

1 reference

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Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis

26 May 2018

1 reference

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Mouse models of cystic fibrosis

26 May 2018

1 reference

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Enhanced susceptibility to pulmonary infection with Burkholderia cepacia in Cftr(-/-) mice

26 May 2018

1 reference

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

26 May 2018

1 reference

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Pathophysiology of gene-targeted mouse models for cystic fibrosis

26 May 2018

1 reference

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

26 May 2018

1 reference

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

26 May 2018

1 reference

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Lung disease in mice with cystic fibrosis

26 May 2018

1 reference

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

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Towards an ovine model of cystic fibrosis

26 May 2018

1 reference

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

26 May 2018

1 reference

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Developmental expression patterns of CFTR in ferret tracheal surface airway and submucosal gland epithelia.

26 May 2018

1 reference

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

26 May 2018

1 reference

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Defective fluid transport by cystic fibrosis airway epithelia

26 May 2018

1 reference

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The immune system of the respiratory tract in pigs

26 May 2018

1 reference

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A mouse model for the cystic fibrosis delta F508 mutation.

26 May 2018

1 reference

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

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

The cells of the tracheobronchial epithelium of the mouse: a quantitative light and electron microscope study

26 May 2018

1 reference

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

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Quantitation of the secretory cells of the ferret tracheobronchial tree.

26 May 2018

1 reference

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An animal model for cystic fibrosis made by gene targeting

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

New features in the development of the submucosal gland of the respiratory tract

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

A rat model of chronic respiratory infection with Pseudomonas aeruginosa

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Environmental Burkholderia cenocepacia Strain Enhances Fitness by Serial Passages during Long-Term Chronic Airways Infection in Mice.

26 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Airway surface liquid pH is not acidic in children with cystic fibrosis.

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Lower airway inflammation in infants with cystic fibrosis detected by newborn screening.

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Ventilatory responses to hypercapnia and hypoxia in conscious cystic fibrosis knockout mice Cftr-/-.

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

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

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5879563

Submucosal glands are the predominant site of CFTR expression in the human bronchus

30 November 2018

1 reference

12 December 2020

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

1 reference

12 December 2020

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

1 reference

12 December 2020

Mucociliary clearance in patients with cystic fibrosis and in normal subjects

1 reference

12 December 2020

Phenotypic abnormalities in long-term surviving cystic fibrosis mice

1 reference

12 December 2020

Impaired ability of Cftr knockout mice to control lung infection with Pseudomonas aeruginosa.

1 reference

12 December 2020

Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients

1 reference

12 December 2020

Functional genomics and rat models

1 reference

12 December 2020

Upregulated expression of ENaC in human CF nasal epithelium

1 reference

12 December 2020

Inhaled phosphodiesterase type 5 inhibitors restore chloride transport in cystic fibrosis mice

1 reference

12 December 2020

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

1 reference

12 December 2020

Cystic Fibrosis Mice Develop Spontaneous Chronic Bordetella Airway Infections

1 reference

12 December 2020

10.1186/S12931-018-0750-Y

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29609604%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

22 March 2020

1 reference

9 September 2019

https://scigraph.springernature.com/pub.10.1186/s12931-018-0750-y

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29609604%20AND%20SRC:MED&resulttype=core&format=json

22 March 2020

1 reference