0| 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 |
|
| XIV. Von Willebrand factor or vWF | Binds to VIII, mediates platelet adhesion | Von Willebrand disease
|
| XV. Prekallikrein(Fletcher factor) | Activates XII and prekallikrein; cleaves HMWK | Prekallikrein/Fletcher Factor deficiency |
| XVI. High Molecular weight Kininogen or HMWK (Fitzgerald factor) | Supports reciprocal activation of XII, XI, and prekallikrein | Kininogen deficiency |
| XVII. Fibronectin | Mediates cell adhesion | Gomerulopathy with fibronectin deposits |
| XVIII. Antithrombin III | Inhibits IIa, Xa, and other proteases | Antithrombin III deficiency |
| XIX. Protein C | Inactivates Va and VIIIa | Protein C Deficiency |
| XX. Protein S | Cofactor for activated protein C | Protein S deficiency |
| XXI. Protein Z | Mediates thrombin adhesion to phospholipids and stimulates degradation of factor X by ZPI | Protein Z deficiency |
| XXII. Plasminogen | Converts to plasmin, lyses fibrin and other proteins | Plasminogen deficiency, type I (ligneous conjunctivitis) |
| XXIII. Alpha 2 antiplasmin | Inhibits plasmin | Antiplasmin deficiency |
| XXIV. Tissue plasminogen activator or tPA | Activates plasminogen | Thrombophilia
|
| XXV. Urokinase | Activates plasminogen | Quebec platelet disorder |
| XXVI. Plasminogen Activator Inhibitor 1 (PAI1) | Inactivates tPA & urokinase (endothelial PAI) | Plasminogen activator inhibitor-1 deficiency |
| XXVII. Plasminogen Activator Inhibitor 2 or PAI2 | Inactivates tPA & urokinase |
|
| XXVIII. Cancer procoagulant | Pathological factor X activation | Thrombophilia |
