Proteases and cystic fibrosis.

Journal Article (Journal Article;Review)

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.

Full Text

Duke Authors

Cited Authors

  • Voynow, JA; Fischer, BM; Zheng, S

Published Date

  • 2008

Published In

Volume / Issue

  • 40 / 6-7

Start / End Page

  • 1238 - 1245

PubMed ID

  • 18395488

Pubmed Central ID

  • PMC2431113

International Standard Serial Number (ISSN)

  • 1357-2725

Digital Object Identifier (DOI)

  • 10.1016/j.biocel.2008.03.003


  • eng

Conference Location

  • Netherlands