Coagulation proteins


See also Individual agents

General information

The major coagulation and fibrinolytic proteins are listed in Table 1 .

Table 1
Proteins involved in coagulation and fibrinolysis
Protein (synonym) Function
Coagulation proteins
Factor I (fibrinogen) Activated to fibrin
Factor II a (prothrombin) Activated to thrombin
Factor III (tissue thromboplastin) Extrinsic pathway
Factor IV (calcium) Co-factor
Factor V (Ac-globulin; proaccelerin) Converts prothrombin to thrombin
Factor VI (unassigned)
Factor VII a (proconvertin) Extrinsic pathway
Factor VIII (antihemophilic factor) Intrinsic pathway
Factor IX a (Christmas factor) Intrinsic pathway
Factor X a (Stuart-Prower factor) Extrinsic pathway
Factor XI (plasma thromboplastin antecedent) Intrinsic pathway
Factor XII (Hageman factor) Intrinsic pathway
Factor XIII (fibrin stabilizing factor) Stabilizes fibrin
Von Willebrand factor (factor VIII-related antigen; vWF)
High molecular weight kininogen (Fitzgerald factor) Intrinsic pathway
Prekallikrein (Fletcher factor) Intrinsic pathway
Fibrinolytic proteins
Plasminogen Converted to fibrinolysin
Prourokinase Precursor of urokinase
Tissue plasminogen activator Activates plasminogen
Antithrombin III Inhibits thrombin, IXa, Xa, XIa, XIIa
Protein C a Inhibits factors Va, VIIIa
Protein S a Co-factor for protein C
a 2 -Antiplasmin Inhibits binding of fibrinolysin

a Vitamin K-dependent factor; the intrinsic and extrinsic pathways converge with the activation of factor X to Xa by factor VIII and IX.

Coagulation protein concentrates became available in the 1970s, a significant step in the prevention and management of bleeding. The factors that are currently available are factor VII, factor VIIa, factor VIII, factor IX, factor XI, and factor XIII. There is also a factor VIII inhibitor bypassing factor, activated prothrombin complex concentrate. Fresh frozen plasma and prothrombin complex concentrate, which contain mixtures of coagulation proteins, are also available.

Two major classes of complications in the use of these proteins have emerged [ ]. First, transfusion-related infections with various blood-borne viruses, such as hepatitis B and C and human immunodeficiency virus (HIV). Secondly, alloimmune antibodies (inhibitors) against the deficient coagulation factors.

Organs and systems

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