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 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 alpha1-antitrypsin.
Gooptu
Bibek
B
School of Crystallography, Birkbeck College, University of London, London WC1E 7HX, England, UK.
Lomas
David A
DA
D000818:Animals; D004646:Emphysema / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D005342:Fibrinolysis / Q000235:genetics; D006801:Humans; D007249:Inflammation / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008954:Models, Biological; D009154:Mutation; D011108:Polymers; D000515:alpha 1-Antitrypsin / Q000235:genetics / Q000378:metabolism*
D011108:Polymers; D000515:alpha 1-Antitrypsin