Polymers and inflammation: disease mechanisms of the serpinopathies

被引：63

作者：

Gooptu, Bibek ^{[1
,2
]}

Lomas, David A. ^{[1
]}

机构：

[1] Univ Cambridge, Dept Med, Cambridge Inst Med Res, Cambridge CB2 OXY, England

[2] Univ London, Birkbeck Coll, Sch Crystallog, London WC1E 7HX, England

来源：

JOURNAL OF EXPERIMENTAL MEDICINE | 2008年 / 205卷 / 07期

基金：

英国医学研究理事会; 英国惠康基金;

关键词：

D O I：

10.1084/jem.20072080

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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 alpha(1)-antitrypsin.

引用

页码：1529 / 1534

页数：6

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