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Encyclopedia > Proconvertin

Factor VII (old name proconvertin) is one of the central proteins in the coagulation cascade. It is an enzyme (EC 3.4.21.21 (http://www.expasy.org/cgi-bin/nicezyme.pl?3.4.21.21)) of the serine protease class.

Contents

Physiology

The main role of factor VII (FVII) is to initiate the process of coagulation in conjunction with tissue factor (TF) after release of the latter from damaged tissues. Once bound to TF, FVII is activated to FVIIa by different proteases, among which are thrombin (factor IIa), activated factor X and the FVIIa-TF complex itself. The most important substrates for FVIIa-TF are Factor X and Factor IX.


The action of the factor is impeded by tissue factor pathway inhibitor (TFPI), which is released almost immediately after initiation of coagulation. Factor VII is vitamin K dependent; it is produced in the liver. Use of warfarin or similar anticoagulants impairs its function.


Genetics

The gene for factor VII is located on chromosome 13 (13q34).


Role in disease

Deficiency is rare (congenital proconvertin deficiency) and inherits recessively.


Therapeutic use

Recombinant human factor VIIa (NovoSeven®, ATC code B02BD05) has been introduced for use in uncontrollable bleeding in hemophilia patients who have developed inhibitors against replacement coagulation factor.


It is being increasingly used in uncontrollable hemorrhage. The rationale for its use in hemorrhage is, that it will only induce coagulation in those sites where tissue factor (TF) is also present. Indeed, there is no report on significantly increased thrombosis risk in rhFVIIa use.


Reference

  • Roberts HR, Monroe DM, White GC. The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood 2004;104:3858-64. PMID 15328151.

External links

  • OMIM 227500 (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=227500)

  Results from FactBites:
 
Factor VII - Wikipedia, the free encyclopedia (383 words)
Factor VII (old name proconvertin) is one of the central proteins in the coagulation cascade.
The gene for factor VII is located on chromosome 13 (13q34).
Deficiency is rare (congenital proconvertin deficiency) and inherits recessively.
  More results at FactBites »

 
 

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