C1-Inhibitor

C1-inhibitor (C1-INH, SERPING1) is a heavily glycosylated plasma serpin that is the master regulator of the classical complement pathway and the contact system. It irreversibly inactivates the activated complement proteases C1r and C1s, and also neutralizes plasma kallikrein, coagulation factors XIIa and XIa and, to a lesser extent, thrombin and plasmin, thereby controlling bradykinin generation, coagulation and inflammation simultaneously. Its unusually high carbohydrate content affects secretion and half-life. Heterozygous deficiency causes hereditary angioedema with recurrent swelling attacks, and both plasma-derived and recombinant C1-INH are approved therapies. Recombinant protein is also used to probe complement-kallikrein crosstalk in vitro.