(Q57952421)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

review article

1 reference

title

Therapies directed at the basic defect in cystic fibrosis (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

1 reference

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

author name string

Pamela Leslie Zeitlin

series ordinal

1

0 references

publication date

1 September 1998

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

Clinics in Chest Medicine

5 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

volume

19

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

issue

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

page(s)

515-25, vi-vii

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

In vivo model of adeno-associated virus vector persistence and rescue

5 December 2019

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Evidence of lack of toxicity of sodium phenylacetate and sodium benzoate in treating urea cycle enzymopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Aerosol administration of a recombinant adenovirus expressing CFTR to cystic fibrosis patients: a phase I clinical trial

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Magainins: a new family of membrane-active host defense peptides

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Gene therapy for cystic fibrosis using E1-deleted adenovirus: a phase I trial in the nasal cavity. The University of North Carolina at Chapel Hill.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Correcting temperature-sensitive protein folding defects

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Restoration of nitrogen homeostasis in a man with ornithine transcarbamylase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Amino Acid Acylation: A Mechanism of Nitrogen Excretion in Inborn Errors of Urea Synthesis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Nucleotide transport through the cystic fibrosis transmembrane conductance regulator

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

CPX, a selective A1-adenosine-receptor antagonist, regulates intracellular pH in cystic fibrosis cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis: molecular biology and therapeutic implications

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The cystic fibrosis transmembrane conductance regulator and ATP.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Increased fetal hemoglobin in patients receiving sodium 4-phenylbutyrate

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The human cytosolic molecular chaperones hsp90, hsp70 (hsc70) and hdj-1 have distinct roles in recognition of a non-native protein and protein refolding

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Passive smoking in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Human beta-defensin-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Genistein potentiates wild-type and delta F508-CFTR channel activity

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Alternate stimulation of apical CFTR by genistein in epithelia

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Role of actin in regulation of epithelial sodium channels by CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Elemental composition of human airway surface fluid in healthy and diseased airways

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508).

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Extracellular ATP and UTP induce chloride secretion in nasal epithelia of cystic fibrosis patients and normal subjects in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis, lung infections, and a human tracheal antimicrobial peptide (hTAP)

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Passive smoking and lung function in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Induction by antitumoral drugs of proteins that functionally complement CFTR: a novel therapy for cystic fibrosis?

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The bactericidal activity of magainins against Pseudomonas aeruginosa and Enterococcus faecium.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Human airway epithelia express a beta-defensin

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Disposition of phenylbutyrate and its metabolites, phenylacetate and phenylacetylglutamine.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

CFTR chloride channel activation by genistein: the role of serine/threonine protein phosphatases

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Gene therapy for cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Extended therapy with intravenous arginine butyrate in patients with beta-hemoglobinopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Passive smoking and impaired lung function in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Degradation of CFTR by the ubiquitin-proteasome pathway

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Direct action of genistein on CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis gene therapy using an adenovirus vector: in vivo safety and efficacy in nasal epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Modulation of CFTR chloride channels by calyculin A and genistein

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Butyrate induces expression of transfected human fetal and endogenous mouse embryonic globin genes in GM 979 erythroleukemia cells

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

Cystic fibrosis: genotypic and phenotypic variations

1 reference

https://api.crossref.org/works/10.1016%2FS0272-5231%2805%2970097-6

7 January 2021

10.1016/S0272-5231(05)70097-6

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:9759553%20AND%20SRC:MED&resulttype=core&format=json

5 December 2019

1 reference