(Q41660557)

6 October 2017

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title

Genetic and immunologic aspects of cystic fibrosis (English)

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6 October 2017

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Hilman BC

1

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6 October 2017

1 November 1997

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Annals of Allergy, Asthma & Immunology

6 October 2017

1 reference

6 October 2017

79

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6 October 2017

5

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6 October 2017

379-90; quiz 391-4

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Heterogeneity in the severity of cystic fibrosis and the role of CFTR gene mutations

6 October 2017

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis locus defined by a genetically linked polymorphic DNA marker.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Identification of the cystic fibrosis gene: chromosome walking and jumping

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Identification of the cystic fibrosis gene: genetic analysis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Chloride impermeability in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Missing Cl conductance in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis transmembrane conductance regulator and obstructive azoospermia

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

The cystic fibrosis transmembrane conductance regulator gene

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Functions of the cystic fibrosis transmembrane conductance regulator protein

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis: molecular biology and therapeutic implications

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Phosphorylation-dependent block of cystic fibrosis transmembrane conductance regulator chloride channel by exogenous R domain protein

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

1 reference

7 January 2021

Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Pancreatitis in young children with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

CFTR as a cAMP-dependent regulator of sodium channels

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Relative expression of the human epithelial Na+ channel subunits in normal and cystic fibrosis airways

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

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%2FS1081-1206%2810%2963031-1

7 January 2021

The molecular basis for disease variability in cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

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%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis: genotypic and phenotypic variations

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

A Novel Mutation in the Cystic Fibrosis Gene in Patients with Pulmonary Disease but Normal Sweat Chloride Concentrations

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Mild cystic fibrosis and normal or borderline sweat test in patients with the 3849 + 10 kb C-->T mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis 3849+10kb C > T mutation associated with severe pulmonary disease and male fertility

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Congenital bilateral absence of the vas deferens. A primarily genital form of cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

A mutation in CFTR produces different phenotypes depending on chromosomal background

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Expression of an abundant alternatively spliced form of the cystic fibrosis transmembrane conductance regulator (CFTR) gene is not associated with a cAMP-activated chloride conductance.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Sweat testing for the diagnosis of cystic fibrosis: practical considerations

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Human airway ion transport. Part one.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Relative ion permeability of normal and cystic fibrosis nasal epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Cystic fibrosis gene and protein expression during fetal lung development.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Inflammatory cytokines in cystic fibrosis lungs

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Proteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Neutrophil elastase cleaves C3bi on opsonized pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatch

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

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%2FS1081-1206%2810%2963031-1

7 January 2021

Transient Pulmonary Infiltrations in Cystic Fibrosis due to Allergic Aspergillosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Variability in parameters of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Allergic bronchopulmonary aspergillosis complicating cystic fibrosis in childhood

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Allergic bronchopulmonary aspergillosis in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Prevalence of allergic bronchopulmonary aspergillosis and atopy in adult patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Computerized tomography in the evaluation of allergic bronchopulmonary aspergillosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Identification of allergic bronchopulmonary aspergillosis in cystic fibrosis patients by recombinant Aspergillus fumigatus I/a-specific serology

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Skin test reactivity to recombinant Aspergillus fumigatus allergen I/a in patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Response to aerosol salbutamol, SCH 1000, and placebo in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Reversible airway obstruction in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Lung function in cystic fibrosis: acute effect of salbutamol.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Bronchodilators increase airway instability in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Bronchodilator responsiveness in infants and young children with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Longitudinal response of pulmonary function to bronchodilators in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Short-term and long-term effects of albuterol aerosol therapy in cystic fibrosis: a preliminary report

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Regular inhaled beta-agonist treatment in bronchial asthma.

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

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%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

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%2FS1081-1206%2810%2963031-1

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS1081-1206%2810%2963031-1

7 January 2021

10.1016/S1081-1206(10)63031-1

Identifiers

DOI

1 reference

6 October 2017

1 reference

PubMed ID

9396969