Pathophysiology of gene-targeted mouse models for cystic fibrosis (Q28295602)

Generation of cAMP-Activated Chloride Currents by Expression of CFTR

Europe PubMed Central

title

Pathophysiology of gene-targeted mouse models for cystic fibrosis (English)

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B R Grubb

1

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R C Boucher

series ordinal

2

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language of work or name

English

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publication date

January 1999

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published in

Physiological Reviews

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volume

79

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page(s)

S193-214

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issue

1 Suppl

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cites work

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https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Cl- secretion by trachea of CFTR (+/-) and (-/-) fetal mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Enhanced intestinal glucose and alanine transport in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C- and A-dependent mechanisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Transcriptional regulation of endothelial constitutive PGHS-1 expression by phorbol ester

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Ion-transporting activity in the murine colonic epithelium of normal animals and animals with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Deregulated expression and function of CFTR and Cl- secretion after activation of the Ras and Src/PyMT pathways in Caco-2 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Cystic fibrosis in the mouse by targeted insertional mutagenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Small-intestinal abnormalities in cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Regulation of membrane chloride currents in rat bile duct epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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/10.1152%2FPHYSREV.1999.79.1.S193

Functions of the cystic fibrosis transmembrane conductance regulator protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Modification of nasal membrane potential difference with inhaled amiloride and loperamide in the cystic fibrosis (CF) mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Anomalies in ion transport in CF mouse tracheal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Failure of cholinergic stimulation to induce a secretory response from the rectal mucosa in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Expression cloning and cDNA sequencing of the Na+/glucose co-transporter

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Identification of the cystic fibrosis gene: genetic analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Relative ion permeability of normal and cystic fibrosis nasal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Altering genes in animals by gene targeting

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

cAMP- but not Ca(2+)-regulated Cl- conductance in the oviduct is defective in mouse model of cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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/10.1152%2FPHYSREV.1999.79.1.S193

Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Chloride impermeability in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Identification of the cystic fibrosis gene: chromosome walking and jumping

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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/10.1152%2FPHYSREV.1999.79.1.S193

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

An animal model for cystic fibrosis made by gene targeting

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

CFTR as a cAMP-dependent regulator of sodium channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Failure to induce secretion in jejunal biopsies from children with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Regulation of human airway surface liquid

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Abnormal enamel development in a cystic fibrosis transgenic mouse model.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Regulation of chloride channels by protein kinase C in normal and cystic fibrosis airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Accounting for cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S193

10.1152/PHYSREV.1999.79.1.S193

21 January 2018

Identifiers

DOI

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Fatcat ID

release_eh3sm3o7xff3ngeloetvfsyqmi

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OpenCitations bibliographic resource ID

1272411

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