SERPINs-From Trap to Treatment

Frontiers in Medicine
Wariya SanrattanaSteven de Maat

Abstract

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 (serine protease inhibitors). 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.

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Citations

Apr 5, 2020·Journal of Clinical Medicine·Jordan R YaronAlexandra R Lucas
Jan 23, 2021·International Journal of Molecular Sciences·Mohamed Amine JmelMichalis Kotsyfakis
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Nov 7, 2021·Proceedings of the National Academy of Sciences of the United States of America·Wariya SanrattanaSteven de Maat
Sep 24, 2021·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·Tinsley Claire Douglas, Sari S Hannila

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Methods Mentioned

BETA
glycosylation
Replacement

Software Mentioned

PyMol

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