Sunday, February 19, 2012

COAGULATION FACTORS AND RELATED SUBSTANCES


Number and/or name
Function
Associated genetic disorders
Forms clot (fibrin)
Its active form (IIa) activates I, V, VII, VIII, XI, XIII, protein C, platelets
Co-factor of VIIa (formerly known as factor III)
Required for coagulation factors to bind to phospholipid (formerly known as factor IV)
V (Proaccelerin, labile factor)
Co-factor of X with which it forms the prothrombinase complex
VI
Unassigned – old name of Factor Va
VII (Stable factor, proconvertin)
Activates IX, X
congenital proconvertin/factor VII deficiency
VIII (Antihemophilic factor A)
Co-factor of IX with which it forms the tenase complex
IX (Antihemophilic factor B or Christmas factor)
Activates X: forms tenase complex with factor VIII
X (Stuart-Power factor)
Activates II: forms prothrombinase complex with factor V
Congenital Factor X deficiency
XI (plasma thromboplastin antecedent)
Activates IX
XII (Hageman factor)
Activates factor XI, VII and prekallikrein
XIII (fibrin-stabilizing factor)
Congenital Factor XIIIa/b deficiency
Binds to VIII, mediates platelet adhesion
Prekallikrein (Fletcher factor)
Activates XII and prekallikrein; cleaves HMWK
Prekallikrein/Fletcher Factor deficiency
High-molecular-weight kininogen (HMWK) (Fitzgerald factor)
Supports reciprocal activation of XII, XI, and prekallikrein
Kininogen deficiency
Mediates cell adhesion
Glomerulopathy with fibronectin deposits
Inhibits IIa, Xa, and other proteases
Inhibits IIa, cofactor for heparin and dermatan sulfate ("minor antithrombin")
Heparin cofactor II deficiency
Inactivates Va and VIIIa
Cofactor for activated protein C (APC, inactive when bound to C4b-binding protein)
Mediates thrombin adhesion to phospholipids and stimulates degradation of factor X by ZPI
Degrades factors X (in presence of protein Z) and XI (independently)
Converts to plasmin, lyses fibrin and other proteins
Plasminogen deficiency, type I (ligneous conjunctivitis)
Inhibits plasmin
Antiplasmin deficiency
Activates plasminogen
Activates plasminogen
Inactivates tPA & urokinase (endothelial PAI)
Plasminogen activator inhibitor-1 deficiency
Inactivates tPA & urokinase (placental PAI)
Pathological factor X activator linked to thrombosis in cancer

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