AUTHOR=Sanrattana Wariya , Maas Coen , de Maat Steven 

TITLE=SERPINs—From Trap to Treatment

JOURNAL=Frontiers in Medicine

VOLUME=6

YEAR=2019

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2019.00025

DOI=10.3389/fmed.2019.00025

ISSN=2296-858X

ABSTRACT=<p>Excessive enzyme activity often has pathological consequences. This for example is the case in thrombosis and hereditary angioedema, where serine proteases of the coagulation system and kallikrein-kinin system are excessively active. Serine proteases are controlled by SERPINs (<underline>se</underline>rine <underline>p</underline>rotease <underline>in</underline>hibitors). We here describe the basic biochemical mechanisms behind SERPIN activity and identify key determinants that influence their function. We explore the clinical phenotypes of several SERPIN deficiencies and review studies where SERPINs are being used beyond replacement therapy. Excitingly, rare human SERPIN mutations have led us and others to believe that it is possible to refine SERPINs toward desired behavior for the treatment of enzyme-driven pathology.</p>