Cystic fibrosis: a multiple exocrinopathy caused by dysfunctions in a multifunctional transport protein (Q47801804)

5 February 2018

review article

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title

Cystic fibrosis: a multiple exocrinopathy caused by dysfunctions in a multifunctional transport protein (English)

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5 February 2018

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Schwiebert EM

1

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5 February 2018

Benos DJ

series ordinal

2

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5 February 2018

Fuller CM

series ordinal

3

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5 February 2018

language of work or name

English

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publication date

1 June 1998

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5 February 2018

American Journal of Medicine

published in

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5 February 2018

volume

104

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5 February 2018

issue

6

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5 February 2018

page(s)

576-590

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5 February 2018

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

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Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters

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Identification of mutations in the putative ATP-binding domain of the adrenoleukodystrophy gene

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CFTR is a conductance regulator as well as a chloride channel

7 January 2021

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

7 January 2021

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Permeability properties of cell membranes and tight junctions of normal and cystic fibrosis sweat ducts

7 January 2021

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Both the wild type and a functional isoform of CFTR are expressed in kidney

7 January 2021

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

7 January 2021

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Generation of cAMP-Activated Chloride Currents by Expression of CFTR

7 January 2021

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

7 January 2021

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

7 January 2021

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Nucleoside triphosphates are required to open the CFTR chloride channel.

7 January 2021

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Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium

7 January 2021

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Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells

7 January 2021

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Cl- channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase

7 January 2021

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Ca(2+)-dependent Cl- channels in undifferentiated human colonic cells (HT-29). I. Single-channel properties

7 January 2021

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Molecular physiology of anion channels

7 January 2021

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Molecular physiology of voltage-gated chloride channels

7 January 2021

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Chloride channels: an emerging molecular picture

7 January 2021

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Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR

7 January 2021

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

7 January 2021

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

7 January 2021

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7 January 2021

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7 January 2021

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Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis

7 January 2021

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7 January 2021

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7 January 2021

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

7 January 2021

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Effect of uridine 5'-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis

7 January 2021

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

7 January 2021

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Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

7 January 2021

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Epithelial sodium channel related to proteins involved in neurodegeneration

7 January 2021

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Amiloride-sensitive epithelial Na+ channel is made of three homologous subunits

7 January 2021

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Membrane topology of the epithelial sodium channel in intact cells

7 January 2021

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Increased expression and activity of sodium channels in alveolar type II cells of hyperoxic rats

7 January 2021

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Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: a patch-clamp study

7 January 2021

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Cystic fibrosis affects chloride and sodium channels in human airway epithelia

7 January 2021

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Expression of the epithelial Na+ channel in the developing rat lung

7 January 2021

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Epithelial Na Channels: Function and Diversity

7 January 2021

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cGMP stimulates sodium and chloride currents in rat tracheal airway epithelia.

7 January 2021

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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

7 January 2021

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CFTR as a cAMP-dependent regulator of sodium channels

7 January 2021

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Regulation of epithelial sodium channels by short actin filaments.

7 January 2021

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Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator

7 January 2021

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

7 January 2021

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

7 January 2021

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Defective acidification of intracellular organelles in cystic fibrosis

7 January 2021

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Regulation of plasma membrane recycling by CFTR.

7 January 2021

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Regulated endocytosis in a chloride secretory epithelial cell line

7 January 2021

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Heterotrimeric G proteins, vesicle trafficking, and CFTR Cl- channels

7 January 2021

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Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface

7 January 2021

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Effects of the ?F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR

7 January 2021

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Defective protein folding as a basis of human disease

7 January 2021

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A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein

7 January 2021

1 reference

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

7 January 2021

1 reference

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Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis

7 January 2021

1 reference

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Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents

7 January 2021

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Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator

7 January 2021

1 reference

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Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

7 January 2021

1 reference

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Association of less common cystic fibrosis mutations with a mild phenotype

7 January 2021

1 reference

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Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D

7 January 2021

1 reference

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Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes

7 January 2021

1 reference

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Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Multi-ion pore behaviour in the CFTR chloride channel

7 January 2021

1 reference

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Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity.

7 January 2021

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Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel

7 January 2021

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Effect of Deleting the R Domain on CFTR-Generated Chloride Channels

7 January 2021

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Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis

7 January 2021

1 reference

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Reduction in viscosity of cystic fibrosis sputum in vitro by gelsolin

7 January 2021

1 reference

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Effect of high-dose ibuprofen in patients with cystic fibrosis

7 January 2021

1 reference

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

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

A sensitive defense: salt and cystic fibrosis.

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Human airway epithelia express a beta-defensin

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

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

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Degradation of CFTR by the ubiquitin-proteasome pathway

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis

7 January 2021

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Gene therapy for cystic fibrosis: challenges and future directions

7 January 2021

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7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

Defective acidification of the biosynthetic pathway in cystic fibrosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0002-9343%2898%2900119-3

inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1016/S0002-9343(98)00119-3

1 reference

5 February 2018

1 reference

5 February 2018