Sulfatide-dependent autoactivation of human blood coagulation Factor XII (Hageman Factor). 1983

G Tans, and J Rosing, and J H Griffin

When purified human blood coagulation Factor XII (Hageman factor) is incubated with sulfatides at 37 degrees C, activation of Factor XII occurs as judged by the appearance of amidolytic activity towards the chromogenic substrate H-D-Pro-Phe-Arg-p-nitroanilide. Polyacrylamide gel electrophoresis studies using 125I-Factor XII as a marker show that the appearance of amidolytic activity correlates with Factor XII cleavage, that activation goes to completion and that virtually all Factor XIIa formed is present as the two-chain, 80,000 Mr form, alpha-Factor XIIa. Rigorous analysis of kinetic data establishes that, between 0.02 and greater than 90% of the reaction, the activation of Factor XII is described by a mechanism of autoactivation of Factor XII by Factor XIIa. The rate of autoactivation increases with increasing Factor XII concentrations at constant sulfatide levels but decreases with increasing sulfatide concentrations at constant levels of Factor XII. These findings suggest that the concentrations of Factor XII and Factor XIIa bound to the sulfatide surface determine the rate of autoactivation. Soybean trypsin inhibitor, Trasylol, and anti-prekallikrein antibodies have no influence on the rate of sulfatide-dependent autoactivation of Factor XII. Benzamidine inhibits autoactivation with an inhibitor constant, Ki, of 1.9 mM which is similar to the Ki of 1.5 mM for the enzyme, alpha-Factor XIIa. Thus, sulfatide-dependent activation of purified Factor XII is not due to contaminating proteases and is described by a second order mechanism of autoactivation due to the action of surface-bound Factor XIIa on surface-bound Factor XII.

UI MeSH Term Description Entries
D007610 Kallikreins Proteolytic enzymes from the serine endopeptidase family found in normal blood and urine. Specifically, Kallikreins are potent vasodilators and hypotensives and increase vascular permeability and affect smooth muscle. They act as infertility agents in men. Three forms are recognized, PLASMA KALLIKREIN (EC 3.4.21.34), TISSUE KALLIKREIN (EC 3.4.21.35), and PROSTATE-SPECIFIC ANTIGEN (EC 3.4.21.77). Kallikrein,Kininogenase,Callicrein,Dilminal,Kallidinogenase,Kalliginogenase,Kallikrein A,Kallikrein B',Kallikrein Light Chain,Kinin-Forming Enzyme,Padutin,alpha-Kallikrein,beta-Kallikrein,beta-Kallikrein B,Enzyme, Kinin-Forming,Kinin Forming Enzyme,Light Chain, Kallikrein,alpha Kallikrein,beta Kallikrein,beta Kallikrein B
D007700 Kinetics The rate dynamics in chemical or physical systems.
D004789 Enzyme Activation Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme. Activation, Enzyme,Activations, Enzyme,Enzyme Activations
D005174 Factor XII Stable blood coagulation factor activated by contact with the subendothelial surface of an injured vessel. Along with prekallikrein, it serves as the contact factor that initiates the intrinsic pathway of blood coagulation. Kallikrein activates factor XII to XIIa. Deficiency of factor XII, also called the Hageman trait, leads to increased incidence of thromboembolic disease. Mutations in the gene for factor XII that appear to increase factor XII amidolytic activity are associated with HEREDITARY ANGIOEDEMA TYPE III. Coagulation Factor XII,Hageman Factor,Factor 12,Factor Twelve
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006863 Hydrogen-Ion Concentration The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH pH,Concentration, Hydrogen-Ion,Concentrations, Hydrogen-Ion,Hydrogen Ion Concentration,Hydrogen-Ion Concentrations
D001550 Benzamidines Amidines substituted with a benzene group. Benzamidine and its derivatives are known as peptidase inhibitors.
D013433 Sulfoglycosphingolipids GLYCOSPHINGOLIPIDS with a sulfate group esterified to one of the sugar groups. Sulfatide,Sulfatides,Sulfatoglycosphingolipids

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