Plasma protease C1 inhibitor is a member of the serpin family of structurally related proteins. Recombinant Human C1 inhibitor is a highly glycosylated glycoprotein containing 478 amino acid residues (52.8kDa), corresponding to amino acids 23-500 of the C1 inhibitor precursor, and is fully functional in its ability to inhibit the C1 complex. Glycosylated C1 inhibitor migrates at an apparent molecular weight of approximately 80-90kDa by SDS PAGE analysis under reducing conditions.
Plasma protease C1 inhibitor is the primary regulator of the immune complement system. C1 inhibitor is a protease inhibitor th at functions to inhibit the complement system in order to prevent over-activation or spontaneous activation. Inhibition is achieved by binding to and irreversibly inhibiting the C1r and C1s proteases of the C1 complex, which has the effect of shutting down all subsequent downstream events in the complement activation cascade. C1 inhibitor can also inhibit various other proteases, including Kallikrein, Factor XIa, and Factor XIIa. Deficiencies in C1 inhibitor are the primary cause of hereditary angioedema (HAE, hereditary angioneurotic edema), a disease characterized by edema in the respiratory and gastrointestinal tracts. In certain clinical situations, the direct administration of C1 inhibitor can be used to tre at HAE and certain other conditions.
