TY - JOUR
T1 - Polymers and inflammation
T2 - Disease mechanisms of the serpinopathies
AU - Gooptu, Bibekbrata
AU - Lomas, D.A.
N1 - MEDLINE® is the source for the MeSH terms of this document.
PY - 2008/7/7
Y1 - 2008/7/7
N2 - 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 def ned 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 -antitrypsin.
AB - 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 def ned 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 -antitrypsin.
UR - http://www.scopus.com/inward/record.url?partnerID=yv4JPVwI&eid=2-s2.0-46949107890&md5=439e329193b52535f9973c228aac075e
U2 - 10.1084/jem.20072080
DO - 10.1084/jem.20072080
M3 - Article
AN - SCOPUS:46949107890
SN - 0022-1007
VL - 205
SP - 1529
EP - 1534
JO - Journal of Experimental Medicine
JF - Journal of Experimental Medicine
IS - 7
ER -