List of Coagulation Factors and Related substances

Number and/or name	Function	Associated genetic disorders
I (Fibrinogen)	Forms clot (fibrin)	Congenital Afibrogenmia Familial Renal Amyloidosis
II (Prothrombin)	Thrombin or IIa activates I, V, VII, VIII, XI, XIII, Protein C and platelets	Thrombophilia
III Tissue factor	Co-factor of VIIa
IV Calcium	Required for coagulation factors to bind to phospholipid (formerly known as factor IV)	Hypocalcemia is not associated with clotting problem because it has other more serious effects.
V(Proaccelerin)	Co-factor of X with which it forms the Prothrombinase complex	Activated protein C resistance
VI	Unassigned – old name of Factor Va
VII (stable factor, proconvertin)	Activates IX, X	congenital proconvertin/factor VII deficiency
VIII (Anti Hemophilic Factor A)	Co-factor of IX with which it forms the TENASE complex	Haemophilia A
IX (Antihemophilic factor B or Christmas factor)	Activates X: forms TENASE complex with factor VIII	Haemophilia B
X(Stuart-Prower factor)	Forms Prothrombinase complex with factor V and converts Prothrombin or II into Thrombin or IIa.	Congenital Factor X deficiency
XI(Plasma Thromboplastin Antecedent)	Activates IX	Haemophilia C
XII (Hageman factor)	Activates factor XI, VII and prekallikrein	Hereditary Angioedema type III
XIII (Fibrin-Stabilizing Factor)	Congenital Factor XIIIa/b deficiency
Von Willebrand factor or vWF	Binds to VIII, mediates platelet adhesion	Von Willebrand disease
Prekallikrein(Fletcher factor)	Activates XII and prekallikrein; cleaves HMWK	Prekallikrein/Fletcher Factor deficiency
High Molecular weight Kininogen or HMWK  (Fitzgerald factor)	Supports reciprocal activation of XII, XI, and prekallikrein	Kininogen deficiency
Fibronectin	Mediates cell adhesion	Gomerulopathy with fibronectin deposits
Antithrombin III	Inhibits IIa, Xa, and other proteases	Antithrombin III deficiency
Protein C	Inactivates Va and VIIIa	Protein C Deficiency
Protein S	Cofactor for activated protein C	Protein S deficiency
Protein Z	Mediates thrombin adhesion to phospholipids and stimulates degradation of factor X by ZPI	Protein Z deficiency
Plasminogen	Converts to plasmin, lyses fibrin and other proteins	Plasminogen deficiency, type I (ligneous conjunctivitis)
Alpha 2 antiplasmin	Inhibits plasmin	Antiplasmin deficiency
Tissue plasminogen activator or tPA	Activates plasminogen	Thrombophilia
Urokinase	Activates plasminogen	Quebec platelet disorder
Plasminogen Activator Inhibitor 1 (PAI1)	Inactivates tPA & urokinase (endothelial PAI)	Plasminogen activator inhibitor-1 deficiency
Plasminogen Activator Inhibitor 2 or PAI2	Inactivates tPA & urokinase
Cancer procoagulant	Pathological factor X activation	Thrombophilia

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