(Q36874346)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

review article

1 reference

title

What have we learned from mouse models for cystic fibrosis? (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

0 references

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

author name string

Isabel Carvalho-Oliveira

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

Bob J Scholte

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

publication date

1 July 2007

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

Expert Review of Molecular Diagnostics: new diagnostic technologies are set to revolutionise healthcare

1 January 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

volume

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

issue

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

page(s)

407-417

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

Cystic fibrosis and the National Institutes of Health consensus statement: are obstetrician-gynecologists ready to comply?

1 January 2020

cites work

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Identification of the cystic fibrosis gene: genetic analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Identification of the cystic fibrosis gene: chromosome walking and jumping

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

The phenotypic consequences of CFTR mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Comparative genomic sequence analysis of the human and mouse cystic fibrosis transmembrane conductance regulator genes

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Site-directed mutagenesis by gene targeting in mouse embryo-derived stem cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

An animal model for cystic fibrosis made by gene targeting

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Cystic fibrosis in the mouse by targeted insertional mutagenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Animal models of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Lung disease in mice with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Strategies for identifying modifier genes in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Gene modifiers of lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Neutrophilic inflammation as a major determinant in the progression of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Pathogenesis of lung disease in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Current understanding of the inflammatory process in cystic fibrosis: onset and etiology

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Human airway surface epithelial regeneration is delayed and abnormal in cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Macromolecular interactions and ion transport in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Pharmacological approaches to correcting the ion transport defect in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis andCFTRStop Mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Isoprenoid-mediated control of SMAD3 expression in a cultured model of cystic fibrosis epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Lack of inducible nitric oxide synthase in bronchial epithelium: a possible mechanism of susceptibility to infection in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Current prospects for gene therapy of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Limited restoration of cystic fibrosis lung epithelium in vivo with adult bone marrow-derived cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Mouse models of human non-small-cell lung cancer: raising the bar.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Proteomic biomarker discovery for the monogenic disease cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: search for novel biomarkers of cystic fibrosis lung disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Apical localization of ITPK1 enhances its ability to be a modifier gene product in a murine tracheal cell model of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

RNA interference targeted to multiple P2X receptor subtypes attenuates zinc-induced calcium entry

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

New concepts of the pathogenesis of cystic fibrosis lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Acid in the airways. Focus on "Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis".

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Parasympathetic control of airway submucosal glands: central reflexes and the airway intrinsic nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Murine models of chronic Pseudomonas aeruginosa lung infection

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Pulmonary inflammation induced by Pseudomonas aeruginosa lipopolysaccharide, phospholipase C, and exotoxin A: role of interferon regulatory factor 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Lung pathology in response to repeated exposure to Staphylococcus aureus in congenic residual function cystic fibrosis mice does not increase in response to decreased CFTR levels or increased bacterial load.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

A specific database for providing local and national level of integration of clinical data in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

In vivo measurements of ion transport in long-living CF mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Enhancing the efficiency of introducing precise mutations into the mouse genome by hit and run gene targeting

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Mutations in the beta-subunit of the epithelial Na+ channel in patients with a cystic fibrosis-like syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

New insights into cystic fibrosis: molecular switches that regulate CFTR

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1586%2F14737159.7.4.407

21 January 2018

Identifiers

DOI

10.1586/14737159.7.4.407

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17620048%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

1 reference