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The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals
Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the blood. AAT deficiency (AATD) is an autosomal codominant condition affecting an estimated 3.4 million individuals worldwide. The clinical disease associated with AATD can present in a number of ways including...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Dove Medical Press
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5875399/ https://www.ncbi.nlm.nih.gov/pubmed/29618937 http://dx.doi.org/10.2147/JIR.S156405 |
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author | Dunlea, Danielle M Fee, Laura T McEnery, Thomas McElvaney, Noel G Reeves, Emer P |
author_facet | Dunlea, Danielle M Fee, Laura T McEnery, Thomas McElvaney, Noel G Reeves, Emer P |
author_sort | Dunlea, Danielle M |
collection | PubMed |
description | Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the blood. AAT deficiency (AATD) is an autosomal codominant condition affecting an estimated 3.4 million individuals worldwide. The clinical disease associated with AATD can present in a number of ways including COPD, liver disease, panniculitis and antineutrophil cytoplasmic antibody vasculitis. AATD is the only proven genetic risk factor for the development of COPD, and deficient individuals who smoke are disposed to more aggressive disease. Principally, AAT is a serine protease inhibitor; however, over the past number of years, the assessment of AAT as simply an antiprotease has evolved, and it is now recognized that AAT has significant anti-inflammatory properties affecting a wide range of cells, including the circulating neutrophil. |
format | Online Article Text |
id | pubmed-5875399 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Dove Medical Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-58753992018-04-04 The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals Dunlea, Danielle M Fee, Laura T McEnery, Thomas McElvaney, Noel G Reeves, Emer P J Inflamm Res Review Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the blood. AAT deficiency (AATD) is an autosomal codominant condition affecting an estimated 3.4 million individuals worldwide. The clinical disease associated with AATD can present in a number of ways including COPD, liver disease, panniculitis and antineutrophil cytoplasmic antibody vasculitis. AATD is the only proven genetic risk factor for the development of COPD, and deficient individuals who smoke are disposed to more aggressive disease. Principally, AAT is a serine protease inhibitor; however, over the past number of years, the assessment of AAT as simply an antiprotease has evolved, and it is now recognized that AAT has significant anti-inflammatory properties affecting a wide range of cells, including the circulating neutrophil. Dove Medical Press 2018-03-26 /pmc/articles/PMC5875399/ /pubmed/29618937 http://dx.doi.org/10.2147/JIR.S156405 Text en © 2018 Dunlea et al. This work is published by Dove Medical Press Limited, and licensed under a Creative Commons Attribution License The full terms of the License are available at http://creativecommons.org/licenses/by/4.0/. The license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Review Dunlea, Danielle M Fee, Laura T McEnery, Thomas McElvaney, Noel G Reeves, Emer P The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title_full | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title_fullStr | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title_full_unstemmed | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title_short | The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
title_sort | impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5875399/ https://www.ncbi.nlm.nih.gov/pubmed/29618937 http://dx.doi.org/10.2147/JIR.S156405 |
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