cystic fibrosis/protease

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Pulmonary proteases in the cystic fibrosis lung induce interleukin 8 expression from bronchial epithelial cells via a heme/meprin/epidermal growth factor receptor/Toll-like receptor pathway.

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A high intrapulmonary protease burden is characteristic of cystic fibrosis (CF), and the resulting dysregulation of the protease/anti-protease balance has serious implications for inflammation in the CF lung. Because of this inflammation, micro-bleeds can occur releasing hemoglobin into the lung.

Proteases of Pseudomonas aeruginosa in patients with cystic fibrosis.

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Radioimmunoassays were used to determine titers of antibody to alkaline protease (AP) and elastase (Ela) produced by Pseudomonas aeruginosa in sera and bronchial secretions, in vitro production of AP and Ela by P. aeruginosa isolates, and occurrence of these enzymes in bronchial secretions from

Production of elastase, exotoxin A, and alkaline protease in sputa during pulmonary exacerbation of cystic fibrosis in patients chronically infected by Pseudomonas aeruginosa.

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Secretion of Pseudomonas aeruginosa elastase, exotoxin A, and alkaline protease in sputum during bronchopulmonary exacerbations was examined in 18 cystic fibrosis patients chronically infected with this microorganism. The patients were studied during one or several exacerbation periods necessitating

Detection of proteases of Pseudomonas aeruginosa in immune complexes isolated from sputum of cystic fibrosis patients.

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Sera and sputa of 12 cystic fibrosis patients suffering from chronic Pseudomonas aeruginosa lung infections were assayed for immune complexes using the Raji cell assay. Whereas all sera were negative, 33% of the sputa were positive for immune complexes. Sera and sputa of these patients were also

Cystic fibrosis: protease activity in saliva evaluated with chromogenic substrates.

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The protease activities in saliva from individuals with cystic fibrosis (CF) were studied using four different chromogenic substrates. In the CF-group a significantly decreased protease activity in the range 50-70% was found, compared to an age- and sex-matched control group, but with considerable

Secretory leukocyte protease inhibitor in cystic fibrosis.

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Cystic fibrosis (CF) is a genetic disorder that leads to a defect in chloride ion transport and results in pancreatic and pulmonary insufficiency. The pulmonary disease is characterized by bacterial colonization and inflammation with excessive levels of neutrophils and neutrophil elastase within the

Cystic fibrosis sputum induces a secretory response from airway gland serous cells that can be prevented by neutrophil protease inhibitors.

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High activities of the neutrophil proteases, elastase and cathepsin G, are found in the sputum of patients with cystic fibrosis (CF). Because both proteases have been shown to be potent secretagogues for airway submucosal glands, and because hypersecretion is a characteristic feature of CF, the

Reaction of 4-methylumbelliferylguanidinobenzoate with proteases in plasma of patients with cystic fibrosis.

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Protease activity, assayed using 4-methylumbelliferylguanidinobenzoate, an active site titrant of certain proteases, is significantly deficient in plasma of patients with cystic fibrosis. The deficiency can be demonstrated with both chloroform-ellagic acid activated plasma in which the proteases can

Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis.

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Efficient clearance of mucus and inhaled pathogens from the lung is dependent on an optimal airway surface liquid (ASL) volume, which is maintained by the regulated transport of sodium and chloride across the airway epithelium. Accumulating evidence suggests that impaired mucus clearance in cystic

The role of bacterial proteases in the pathogenesis of cystic fibrosis.

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Among the roles of mediators damaging the respiratory epithelium in patients with cystic fibrosis (CF) during the course of chronic, purulent bronchitis, that of neutrophil proteases is well established. The role of bacterial proteases is less well known. Among all pathogens colonizing the airways

Protease phenotypes of Pseudomonas aeruginosa isolated from patients with cystic fibrosis.

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The protease phenotypes expressed by isolates of Pseudomonas aeruginosa from cystic fibrosis (CF) patients were evaluated. The majority of isolates tested produced elastase (65%) or alkaline protease (64%) or both. The mucoid phenotype expressed by many CF isolates of P. aeruginosa did not

Protease binding by alpha 2 macroglobulin in cystic fibrosis.

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The interaction of alpha 2 macroglobulin (alpha 2M) with exogenous proteases has been reported by others to be abnormal in cystic fibrosis (CF). We have re-examined these claims. Four parameters were considered:(1) the molar protease binding of alpha 2M; (2) the interaction of bovine cationic

Prenatal diagnosis of cystic fibrosis by methylumbelliferylguanidinobenzoate protease titration in amniotic fluid.

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Amniotic fluids were obtained from 19 mothers who had previously given birth to a child with cystic fibrosis. Measurement of methylumbelliferylguanidinobenzoate (MUGB) reactive proteases suggested that all 19 would have unaffected babies. Amongst the first 10 cases to come to term there were 5

Impaired Airway Epithelial Barrier Integrity in Response to Stenotrophomonas maltophilia Proteases, Novel Insights Using Cystic Fibrosis Bronchial Epithelial Cell Secretomics.

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Stenotrophomonas maltophilia is a Gram-negative opportunistic pathogen that can chronically colonize the lungs of people with cystic fibrosis (CF) and is associated with lethal pulmonary hemorrhage in immunocompromised patients. Its secreted virulence factors include the extracellular serine

Anomalous alpha 2-macroglobulin-protease complexes in cystic fibrosis: decreased uptake of the complexes by fibroblasts in culture.

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Immunochemical and functional properties of control and Cystic Fibrosis (CF) alpha 2-Macroglobulin (alpha 2M) are compared. Crossed immunoelectrophoresis and Ouchterlony double diffusion revealed no qualitative differences between the two alpha 2-M preparations. Trypsin-esterase activity assayed

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