Figure 8: Potential pro (A) and anticoagulatory (B) effects of C1-esterase inhibitor (C1–INH) function.

. C1–INH can potentially elicit procoagulatory effects (A), by inhibiting fibrinolysis or increasing platelet activation, but also anticoagulatory effects (B), by inhibiting thrombin formation, platelet activation, or the function of complement pathway. Dotted arrows indicate indirect actions and continuous arrows indicate direct actions. Red Xs indicate inhibition by C1–INH. Ca=calcium; coagulation factors Va, VIIIa, IX/IXa, X/Xa, XI/XIa, XII/XIIa, XIII/XIIIa; HKa=cleaved kinin-free kininogen; HMWK=high-molecular weight kininogen; MASP=mannose-binding lectin-associated serine protease; NO=nitric oxide; PGI2=prostaglandin I2; PL=phospholipids; pro-uPA=pro-urokinase-type plasminogen activator; TF:VIIa=TF complex with factor VIIa; tPA=tissue plasminogen activator; TXA2=thromboxane A2; uPA=urokinase-type plasminogen activator.