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

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 subsequen...

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Autores principales: Twigg, Matthew S., Brockbank, Simon, Lowry, Philip, FitzGerald, S. Peter, Taggart, Clifford, Weldon, Sinéad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4491392/
https://www.ncbi.nlm.nih.gov/pubmed/26185359
http://dx.doi.org/10.1155/2015/293053
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author Twigg, Matthew S.
Brockbank, Simon
Lowry, Philip
FitzGerald, S. Peter
Taggart, Clifford
Weldon, Sinéad
author_facet Twigg, Matthew S.
Brockbank, Simon
Lowry, Philip
FitzGerald, S. Peter
Taggart, Clifford
Weldon, Sinéad
author_sort Twigg, Matthew S.
collection PubMed
description 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.
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spelling pubmed-44913922015-07-16 The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung Twigg, Matthew S. Brockbank, Simon Lowry, Philip FitzGerald, S. Peter Taggart, Clifford Weldon, Sinéad Mediators Inflamm Review Article 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. Hindawi Publishing Corporation 2015 2015-06-21 /pmc/articles/PMC4491392/ /pubmed/26185359 http://dx.doi.org/10.1155/2015/293053 Text en Copyright © 2015 Matthew S. Twigg et al. https://creativecommons.org/licenses/by/3.0/ 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.
spellingShingle Review Article
Twigg, Matthew S.
Brockbank, Simon
Lowry, Philip
FitzGerald, S. Peter
Taggart, Clifford
Weldon, Sinéad
The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title_full The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title_fullStr The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title_full_unstemmed The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title_short The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
title_sort role of serine proteases and antiproteases in the cystic fibrosis lung
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4491392/
https://www.ncbi.nlm.nih.gov/pubmed/26185359
http://dx.doi.org/10.1155/2015/293053
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