C1 inhibitor, a multi-functional serine protease inhibitor

• Published in: Thrombosis and haemostasis Vol. 104; no. 5; p. 886
• Main Authors: Davis, 3rd, Alvin E, Lu, Fengxin, Mejia, Pedro
• Format: Journal Article
• Language: English
• Published: Germany 01.11.2010
• Subjects: Angioedemas, Hereditary - blood; Angioedemas, Hereditary - drug therapy; Angioedemas, Hereditary - immunology; Animals; Anti-Inflammatory Agents - therapeutic use; Capillary Permeability; Complement Activation; Complement C1 Inhibitor Protein - chemistry; Complement C1 Inhibitor Protein - genetics; Complement C1 Inhibitor Protein - metabolism; Complement C1 Inhibitor Protein - therapeutic use; Graft Rejection - blood; Graft Rejection - drug therapy; Graft Rejection - immunology; Hemostasis; Humans; Malaria - blood; Malaria - drug therapy; Malaria - immunology; Protein Conformation; Reperfusion Injury - blood; Reperfusion Injury - drug therapy; Reperfusion Injury - immunology; Sepsis - blood; Sepsis - drug therapy; Sepsis - immunology; Shock, Septic - blood; Shock, Septic - drug therapy; Shock, Septic - immunology; Signal Transduction; Structure-Activity Relationship

C1 inhibitor (C1INH) is a serpin that regulates both complement and contact (kallikrein-kinin) system activation. It consists of a serpin domain that is highly homologous to other serpins and an amino terminal non-serpin mucin-like domain. Deficiency of C1INH results in hereditary angioedema, a dise...