Exploring the role of C1-inhibitor in thrombotic diseases

Potential mechanisms linking SERPING1 insufficiency/C1INH deficiency with the risk of HAE-C1INH and arterial and venous thrombosis.SERPING1 insufficiency leads to C1INH deficiency.C1INH is a multifunctional serine protease inhibitor of constituents of the KKS (FXII and kallikrein), contact pathway of coagulation (FXII and FXI), the classical pathway (C1r and C1s) and lectin pathway (MASP-1 and MASP-2) of the complement system, and fibrinolysis (activation of plasminogen).C1INH deficiency secondary to SERPING1 insufficiency would lead to the dysregulation of the activity of those plasma cascade systems.Although HAE-C1INH is predominantly a disorder of the KKS, thrombotic diseases secondary to C1INH deficiency may result from the dysregulation of the contact pathway of coagulation, complement system, and KKS, along with the interplay among them.