Alpha-1 Antitrypsin

Alpha-1 antitrypsin is a serine protease inhibitor and member of the serpin superfamily.¹ It has a five-stranded A β-sheet and a mobile reactive center loop that acts as a pseudosubstrate for various proteases. Alpha-1 antitrypsin binds to a protease, undergoes proteolytic cleavage, and forms a covalent linkage between a carboxyl group in the reactive loop and the serine hydroxyl of the protease active site, effectively inactivating the enzyme which is then cleared from circulation. The primary targets of alpha-1 antitrypsin are neutrophil elastase and proteinase 3, however, it also inhibits trypsin, kallikreins 7 and 14, and matriptase.² Alpha-1 antitrypsin protects the lower respiratory tract from proteolytic destruction via inhibition of neutrophil elastase and reduced serum levels of alpha-1 antitrypsin have been linked to early-onset liver disease and emphysema.³ Alpha-1 antitrypsin is an acute-phase protein that reduces production of inflammatory cytokines, inhibits apoptosis, blocks leukocyte degranulation and migration, as well as suppresses NF-κB nuclear translocation in monocytes. It delays disease onset in mouse models of inflammatory disease, including collagen-induced arthritis and experimental autoimmune encephalomyelitis (EAE).

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1. Elliott, P.R., Abrahams, J.P., and Lomas, D.A. Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation. J. Mol. Biol. 275(3), 419-425 (1998).

2. Janciauskiene, S.M., Bals, R., Koczulla, R., et al. The discovery of α1-antitrypsin and its role in health and disease. Respir. Med. 105(8), 1129-1139 (2011).

3. Ehlers, M.R. Immune-modulating effects of alpha-1 antitrypsin. Biol. Chem. 395(10), 1187-1193 (2014).