(Q46380953)

21 December 2017

review article

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title

Physiological basis of cystic fibrosis: a historical perspective (English)

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21 December 2017

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P M Quinton

1

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21 December 2017

publication date

1 January 1999

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21 December 2017

published in

Physiological Reviews

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21 December 2017

volume

79

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21 December 2017

page(s)

S3-S22

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21 December 2017

issue

1 Suppl

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21 December 2017

Serum neutral alpha-D-glucosidase from patients with cystic fibrosis and chronic pulmonary disease

cites work

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Nucleoside triphosphates are required to open the CFTR chloride channel.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Coupling of CFTR Cl- channel gating to an ATP hydrolysis cycle

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

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.S3

The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Maintenance of contractility in dissociated smooth muscle: low-density cultures in a defined medium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Functional CFTR in endosomal compartment of CFTR-expressing fibroblasts and T84 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Flow rate and inorganic components of submandibular saliva in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Human airway ion transport. Part two.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Distinguishing homozygous and heterozygous cystic fibrosis fibroblasts from normal cells by differences in sodium transport.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Sweat gland function in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Separate Cl- conductances activated by cAMP and Ca2+ in Cl(-)-secreting epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis and pregnancy. A national survey

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Immunocytochemical localization of the cystic fibrosis gene product CFTR

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis of the pancreas. A study in cell culture

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Low sweat electrolytes in a patient with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Fate of orally ingested enzymes in pancreatic insufficiency. Comparison of two dosage schedules

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis of the pancreas: structural changes in peripheral airways

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Aminoglycoside clearance in patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Two types of chloride channel on duct cells cultured from human fetal pancreas

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Gastrointestinal pH, motility/transit and permeability in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Expression and characterization of the cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Congenital steatorrhoea due to pancreatic defect

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Mutations in the putative pore-forming domain of CFTR do not change anion selectivity of the cAMP activated Cl- conductance.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis of the pancreas. A generalised disturbance of water and electrolyte movement in exocrine tissues.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Lung disease in mice with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Identification of the cystic fibrosis gene: genetic analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Bacterial content and ionic composition of sputum in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Increased DNA levels in bronchoalveolar lavage fluid obtained from infants with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Properties and regulation of chloride channels in cystic fibrosis and normal airway cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

The role of mucous glycoproteins in the rheologic properties of cystic fibrosis sputum

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Effect of menses, estrogens and hemolysis on a serum-lectin-like factor in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Viscoelasticity of anionic polymers and their mucociliary transport on the frog palate.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Enhancement of the viscosity of mucin by serum albumin

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Studies on the Mechanism of Inhibition of Sodium Transport in Cystic Fibrosis of the Pancreas

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Altered chloride metabolism in cultured cystic fibrosis skin fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Observations on cystic fibrosis of the pancreas. V. Developmental changes in the male genital system

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Sex ratio and heterozygote advantage in cystic fibrosis families

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Suggestion of an abnormal anion exchange mechanism in sweat glands of cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Chloride impermeability in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Deficiency of Arginine Esterase in Cystic Fibrosis of the Pancreas: Demonstration of the Proteolytic Nature of the Activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Hydrolytic and nonhydrolytic interactions in the ATP regulation of CFTR Cl- conductance

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Interactions between glycoconjugates from human respiratory airways and Pseudomonas aeruginosa

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Intracellular free calcium levels in mononuclear cells of patients with cystic fibrosis and normal controls

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Micropuncture studies of the sweat formation in cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Evidence against defective trans-Golgi acidification in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Aerosolized recombinant human DNase I for the treatment of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

The mechanism of sweat-secretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Assessment of glomerular filtration rate and effective renal plasma flow in cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Decreased renal clearance of sodium in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Effect of secretin and graded doses of CCK-PZ on pancreatic secretion in man.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis gene expression is not correlated with rectifying Cl- channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

An apical-membrane chloride channel in human tracheal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Research on cystic fibrosis: a journey from the Heart House

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Induction of alkaline phosphatase in cultured human fibroblasts. Comparison of normal cells and those from patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Studies on Cystic Fibrosis Using Isoelectric Focusing. II. Demonstration of Deficient Proteolytic Cleavage of α2-Macroglobulin in Cystic Fibrosis Plasma

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Cystic fibrosis ciliary dyskinesia substances and pulmonary disease. Effects of ciliary dyskinesia substances on neutrophil movement in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Pharmacokinetics of Methicillin in Patients with Cystic Fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Comparison of gastrointestinal pH in cystic fibrosis and healthy subjects

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

Duodenal pH in cystic fibrosis and its relationship to fat malabsorption

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

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.S3

Control of mucus hydration as a Donnan equilibrium process

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FPHYSREV.1999.79.1.S3

10.1152/PHYSREV.1999.79.1.S3

21 January 2018

Identifiers

DOI

0 references

PubMed ID

9922374

1 reference

21 December 2017