Factor Trivial Name(s) Pathway Characteristic/Functions

Prekallikrein
Fletcher factor Intrinsic Functions with HMWK and factor XII
(PK)
Co-factor in kallikrein and factor XII activation,
High molecular contact activation cofactor
necessary in factor XIIa activation of XI, precursor for
weight kininogen Fitzgerald Intrinsic
bradykinin (a potent vasodilator and inducer of smooth
(HMWK) Flaujeac Williams factor
muscle contraction
I Fibrinogen Both Forms clot (fibrin)
Contains N-term. gla segment;
II Prothrombin Both Its active form (IIa) activates I, V, VII, XIII, protein C,
platelets
tissue thromboplastin (tissue factor and
III Extrinsic Co-factor of VIIa (formerly known as factor III)
phospholipid
Required for coagulation factors to bind to phospholipid
IV ionized calcium Both
(formerly known as factor IV)
Proaccelerin Protein cofactor
V Labile factor, accelerator (Ac-) globulin Both Co-factor of X with which it forms the
Activated Protein C Resistance (APC) prothrombinasecomplex
VI (same as Va) Accelerin Both This is Va, redundant to Factor V
Proconvertin
Stable factor Endopeptidase with gla residues
VII Extrinsic
serum prothrombin conversion accelerator (SPCA), Activates IX, X
cothromboplastin
Antihemophiliac factor A Protein cofactor
VIII Intrinsic
Antihemophilic globulin (AHG) Co-factor of IX with which it forms the tenasecomplex
Christmas Factor
Endopeptidase with gla residues
IX antihemophilic factor B Intrinsic
Activates X: forms tenasecomplex with factor VIII
plasma thromboplastin component (PTC)
Endopeptidase with gla residues
X Stuart-Prower Factor Both Activates II: froms prothrombinasecomplex with factor
V
XI Plasma thromboplastin antecedent (PTA) Intrinsic Activates XII, IX and prekallikrein
Endopeptidase
XII Hageman Factor Intrinsic
Activates prekallikrein and fibrinolysis
Protransglutaminase Transpeptidase
XIII Both
Fibrin stabilizing factor (FSF), fibrinoligase Crosslinks fibrin

Fibrinogen Activity
Factor I cleaved by thrombin to form fibrin clot
Zymogens of Serine
Activities
Proteases
Factor II activated on surface of activated platelets by prothrombinase complex
Factor X activated on surface of activated platelets by tenase complex and by factor VIIa in presence of tissue factor and Ca2+
Factor VII activated by thrombin in presence of Ca2+
Factor IX activated by factor XIa in presence of Ca2+
Factor XI activated by factor XIIa
Factor XII binds to exposed collagen at site of vessel wall injury, activated by high-MW kininogen and kallikrein
Cofactors Activities
Factor III (tissue factor) a subendothelial cell-surface glycoprotein that acts as a cofactor for factor VII
Factor V activated by thrombin; factor Va is a cofactor in the activation of prothrombin by factor X
Factor VIII Activated by thrombin; factor VIIIa is a cofactor in the activation of factor X by factor Ixa
Transglutaminase Activity
Factor XIII activated by thrombin in presence of Ca2+; stabilizes fibrin clot by covalent cross-linking
Regulatory/Other Proteins Activities
Binds to VIII, mediates platelet adhesion
associated with subendothelial connective tissue; serves as a bridge between platelet glycoprotein GPIb/IX and
von Willebrand factor
collagen

Inactivates Va and VIIIa

Protein C
activated to protein Ca by thrombin bound to thrombomodulin; then degrades factors VIIIa and Va
Cofactor for activated protein C (APC, inactive when bound to C4b-binding protein)
Protein S
acts as a cofactor of protein C; both proteins contain gla residues
Thrombomodulin protein on the surface of endothelial cells; binds thrombin, which then activates protein C
Inhibits IIa, Xa, and other proteases;
Antithrombin III
most important coagulation inhibitor, controls activities of thrombin, and factors IXa, Xa, XIa and XIIa
Prekallikrein Activates XII and prekallikrein; cleaves HMWK
High molecular weight
Supports reciprocal activation of XII, XI, and prekallikrein
kininogen(HMWK)
Heparin cofactor II Inhibits IIa, cofactor for heparin and dermatan sulfate("minor antithrombin")
Protein Z Mediates thrombin adhesion to phospholipids and stimulates degradation of factor X by ZPI
Protein Z-related protease
Degrades factors X (in presence of protein Z) and XI (independently)
inhibitor (ZPI)
Plasminogen Converts to plasmin, lyses fibrin and other proteins
Alpha 2-antiplasmin Inhibits plasmin