Polymers and inflammation: disease mechanisms of the serpinopathies

Members of the serpin (serine proteinase inhibitor) superfamily play a central role in the control of inflammatory, coagulation, and fibrinolytic cascades. Point mutations that cause abnormal conformational transitions in these proteins can trigger disease. Recent work has defined three pathways by...

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Detalles Bibliográficos
Autores principales: Gooptu, Bibek, Lomas, David A.
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2008
Materias:
Commentaries
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2442629/
https://www.ncbi.nlm.nih.gov/pubmed/18591408
http://dx.doi.org/10.1084/jem.20072080
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author Gooptu, Bibek
Lomas, David A.
author_facet Gooptu, Bibek
Lomas, David A.
author_sort Gooptu, Bibek
collection PubMed
description Members of the serpin (serine proteinase inhibitor) superfamily play a central role in the control of inflammatory, coagulation, and fibrinolytic cascades. Point mutations that cause abnormal conformational transitions in these proteins can trigger disease. Recent work has defined three pathways by which these conformers cause tissue damage. Here, we describe how these three mechanisms can be integrated into a new model of the pathogenesis of emphysema caused by mutations in the serpin α(1)-antitrypsin.
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spelling pubmed-24426292009-01-07 Polymers and inflammation: disease mechanisms of the serpinopathies Gooptu, Bibek Lomas, David A. J Exp Med Commentaries Members of the serpin (serine proteinase inhibitor) superfamily play a central role in the control of inflammatory, coagulation, and fibrinolytic cascades. Point mutations that cause abnormal conformational transitions in these proteins can trigger disease. Recent work has defined three pathways by which these conformers cause tissue damage. Here, we describe how these three mechanisms can be integrated into a new model of the pathogenesis of emphysema caused by mutations in the serpin α(1)-antitrypsin. The Rockefeller University Press 2008-07-07 /pmc/articles/PMC2442629/ /pubmed/18591408 http://dx.doi.org/10.1084/jem.20072080 Text en © 2008 Gooptu and Lomas This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.jem.org/misc/terms.shtml). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).
spellingShingle Commentaries
Gooptu, Bibek
Lomas, David A.
Polymers and inflammation: disease mechanisms of the serpinopathies
title Polymers and inflammation: disease mechanisms of the serpinopathies
title_full Polymers and inflammation: disease mechanisms of the serpinopathies
title_fullStr Polymers and inflammation: disease mechanisms of the serpinopathies
title_full_unstemmed Polymers and inflammation: disease mechanisms of the serpinopathies
title_short Polymers and inflammation: disease mechanisms of the serpinopathies
title_sort polymers and inflammation: disease mechanisms of the serpinopathies
topic Commentaries
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2442629/
https://www.ncbi.nlm.nih.gov/pubmed/18591408
http://dx.doi.org/10.1084/jem.20072080
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