Factor VII (formerly known as
proconvertin) is one of the central
proteins in the
coagulation cascade. It is an
enzyme () of the
serine protease class.
Physiology
The main role of factor VII (FVII) is to initiate the process of coagulation in conjunction with
tissue factor (TF). Tissue factor is found on the outside of blood vessels - normally not exposed to the bloodstream. Upon vessel injury, tissue factor is exposed to the blood and circulating factor VII. Once bound to TF, FVII is activated to FVIIa by different proteases, among which are thrombin (factor IIa), factor Xa, IXa, XIIa, 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. Factor VII deficiency presents as a hemophilia-like bleeding disorder. It is treated with recombinant factor VIIa (NovoSeven).
Therapeutic use
Recombinant human factor VIIa (NovoSeven, eptacog alfa [activated], ATC code B02BD08) has been introduced for use in uncontrollable
bleeding in
hemophilia patients (with
Factor VIII or
IX deficiency) who have developed inhibitors against replacement coagulation factor.
It is being increasingly used in uncontrollable hemorrhage. The first report of its use was in an
Israeli soldier with uncontrollable bleeding in 1999. The rationale for its use in hemorrhage is, that it will only induce coagulation in those sites where tissue factor (TF) is also present. Still, O'Connell
et al. report an increased risk of
deep vein thrombosis,
pulmonary embolism and
myocardial infarction in association with the use of rhFVIIa.
According to a 2005 study, recombinant human factor VII improves outcomes in acute
intracerebral hemorrhage.
Interactions
Factor VII has been shown to
interact with
Tissue factor.