Protein Z Deficiency

Because protein Z is involved in the inactivation of coagulation factor Xa, a deficiency or dysfunction of protein Z predisposes the affected for venous thrombosis (especially patients with other bleeding disorders).

Because protein Z is involved in the inactivation of coagulation factor Xa, a deficiency or dysfunction of protein Z predisposes the affected for venous thrombosis (especially patients with other bleeding disorders).

(See also Thrombotic diseases at a glance.) Because protein Z is involved in the inactivation of coagulation factor Xa, a deficiency or dysfunction of protein Z predisposes sufferers of venous thromboembolism (especially patients with other bleeding disorders). Protein Z is a vitamin K-dependent protein that acts as a cofactor in the down-regulation of blood coagulation via the formation of a complex with the protein Z-dependent protease inhibitor (ZPI). The complex inactivate mainly factor Xa on phospholipid surfaces. The consequences of protein Z or ZPI defect or autoantibodies against protein Z in the pathophysiology of thrombosis and miscarriages are not fully understood. However, each of these disorders increases the risk of thrombosis in patients who have a further hereditary coagulation disorder (z. B. factor V Leiden). In specialized regional laboratories protein Z, protein Z-ZPI- and autoantibody values ??can be determined by means of plasma electrophoresis, immunoblot and ELISA. It is not yet known whether a therapy or prophylaxis with anticoagulants in protein Z or ZPI deficiency is indicated.

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