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Int J Biochem Cell Biol. 2008;40(6-7):1238-45. doi: 10.1016/j.biocel.2008.03.003. Epub 2008 Mar 14.

Proteases and cystic fibrosis.

Author information

1
Department of Pediatrics, Duke University Medical Center, Box 2994, Durham, NC 27710, USA. voyno001@mc.duke.edu

Abstract

Cystic fibrosis is the most common, inherited fatal disease in Caucasians. The major cause of morbidity and mortality is chronic lung disease due to infection and inflammation in the airways leading to bronchiectasis and respiratory failure. The signature pathologic features of CF lung disease including abnormal mucus obstructing airways, chronic infection with Staphylococcus aureus, Pseudomonas aeruginosa and other gram negative bacteria, and a robust neutrophil-dominant airway inflammation, are exacerbated by unopposed proteases present at high concentrations in the ASL. There is strong evidence that proteases, particularly neutrophil elastase, contribute to the pathology of CF by impairing mucociliary clearance, interfering with innate immune functions, and perpetuating neutrophilic inflammation. The mechanisms employed by proteases to impact airway function in CF will be reviewed.

PMID:
18395488
PMCID:
PMC2431113
DOI:
10.1016/j.biocel.2008.03.003
[Indexed for MEDLINE]
Free PMC Article

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