Factor XII Quick Facts
- Molecular mass: 80 000 D
- Synthesis: Liver
- Half-life: 40 – 50 hours
- Plasma concentration: 15 – 47mg/l
- Normal range: 50 – 150% or 0.5 – 1.5U/ml
Biochemistry of Factor XII
Factor XIIa is the central enzyme of the contact activation phase of the intrinsic coagulation system. Binding of the proenzyme factor XII to negatively charged surfaces results in its activation to factor XIIa, which in turn activates prekallikrein to kallikrein. In a positive recoupling mechanism, kallikrein again activates factor XII. The coagulatory effect of factor XIIa is the activation of factor XI. In addition to the procoagulant effect of factor XIIa, the formation of kallikrein simultaneously results in both an activation of the fibrinolytic system and the kallikrein-kinin system. Factor XII is also known as Hageman Factor.
Clinical significance of Factor XII
Hereditary factor XII deficiency is very rare. Acquired deficiencies may occur in connection with liver disease, increased consumption during DIC, lupus anticoagulants, nephrotic syndrome and rejection reactions. Increased factor XII concentrations are observed during pregnancy and in women taking oral contraceptives.
Clinical or Research use of Factor XII
- Suspected inherited factor XII deficiency
- Suspected factor XII inhibitors
- Differential diagnosis in subjects with lupus anticoagulants
- Suspected disorders of the kallikrein-kinin system