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Clotting Factors

Dr Paparaj Subbanna
23/12/2017 0 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

 

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