The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung

Matthew S. Twigg, Simon Brockbank, Philip Lowry, S. Peter FitzGerald, Clifford Taggart, Sinéad Weldon

Research output: Contribution to journalReview articlepeer-review

66 Citations (Scopus)
428 Downloads (Pure)

Abstract

Cystic fibrosis (CF) lung disease is an inherited condition with an incidence rate of approximately 1 in 2500 new born babies. CF is characterized as chronic infection of the lung which leads to inflammation of the airway. Sputum from CF patients contains elevated levels of neutrophils and subsequently elevated levels of neutrophil serine proteases. In a healthy individual these proteases aid in the phagocytic process by degrading microbial peptides and are kept in homeostatic balance by cognate antiproteases. Due to the heavy neutrophil burden associated with CF the high concentration of neutrophil derived proteases overwhelms cognate antiproteases. The general effects of this protease/antiprotease imbalance are impaired mucus clearance, increased and self-perpetuating inflammation, and impaired immune responses and tissue. To restore this balance antiproteases have been suggested as potential therapeutics or therapeutic targets. As such a number of both endogenous and synthetic antiproteases have been trialed with mixed success as therapeutics for CF lung disease.

Original languageEnglish
Article number293053
JournalMediators of Inflammation
Volume2015
DOIs
Publication statusPublished - 2015

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