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Mediators of Inflammation
Volume 2015 (2015), Article ID 293053, 10 pages
http://dx.doi.org/10.1155/2015/293053
Review Article

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

1Centre for Infection and Immunity, School of Medicine, Dentistry and Biomedical Sciences, Queen’s University Belfast, Health Sciences Building, 97 Lisburn Road, Belfast BT9 7AE, UK
2Randox Laboratories Limited, 55 Diamond Road, Crumlin, County Antrim BT29 4QY, UK

Received 14 November 2014; Accepted 8 January 2015

Academic Editor: Nades Palaniyar

Copyright © 2015 Matthew S. Twigg et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.