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Taylor, S., Dirir, O., Zamanian, M. et al. (2 more authors) (2018) The role of neutrophils and neutrophil elastase in pulmonary arterial hypertension. Frontiers in Medicine, 5. 217. ISSN 2296-858X
Abstract
Pulmonary arterial hypertension (PAH) is a severe vasculopathy characterized by the presence of fibrotic lesions in the arterial wall and the loss of small distal pulmonary arteries. The vasculopathy is accompanied by perivascular inflammation and increased protease levels, with neutrophil elastase notably implicated in aberrant vascular remodeling. However, the source of elevated elastase levels in PAH remains unclear. A major source of neutrophil elastase is the neutrophil, an understudied cell population in PAH. The principal function of neutrophils is to destroy invading pathogens by means of phagocytosis and NET formation, but proteases, chemokines, and cytokines implicated in PAH can be released by and/or prime and activate neutrophils. This review focuses on the contribution of inflammation to the development and progression of the disease, highlighting studies implicating neutrophils, neutrophil elastase, and other neutrophil proteases in PAH. The roles of cytokines, chemokines, and neutrophil elastase in the disease are discussed and we describe new insight into the role neutrophils potentially play in the pathogenesis of PAH.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2018 Taylor, Dirir, Zamanian, Rabinovitch and Thompson. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY - http://creativecommons.org/licenses/by/4.0/). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
Keywords: | neutrophils; neutrophil elastase; pulmonary hypertension; pulmonary arterial hypertension; vascular remodeling |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 10 Oct 2018 13:28 |
Last Modified: | 19 Apr 2024 09:05 |
Status: | Published |
Publisher: | Frontiers Media |
Refereed: | Yes |
Identification Number: | 10.3389/fmed.2018.00217 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:136800 |
Available Versions of this Item
- The role of neutrophils and neutrophil elastase in pulmonary arterial hypertension. (deposited 10 Oct 2018 13:28) [Currently Displayed]