出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/10/27 05:56:09」(JST)
| Coagulation factor VII (serum prothrombin conversion accelerator) | |||||||||||||
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Anchoring of coagulation factor VIIa (PDB 1dan[1]) to the membrane through its Gla domain |
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| Identifiers | |||||||||||||
| Symbols | F7; SPCA | ||||||||||||
| External IDs | OMIM: 613878 MGI: 109325 HomoloGene: 7710 ChEMBL: 3991 GeneCards: F7 Gene | ||||||||||||
| EC number | 3.4.21.21 | ||||||||||||
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| RNA expression pattern | |||||||||||||
| More reference expression data | |||||||||||||
| Orthologs | |||||||||||||
| Species | Human | Mouse | |||||||||||
| Entrez | 2155 | 14068 | |||||||||||
| Ensembl | ENSG00000057593 | ENSMUSG00000031443 | |||||||||||
| UniProt | P08709 | P70375 | |||||||||||
| RefSeq (mRNA) | NM_000131.4 | NM_010172.3 | |||||||||||
| RefSeq (protein) | NP_000122.1 | NP_034302.2 | |||||||||||
| Location (UCSC) | Chr 13: 113.76 – 113.77 Mb |
Chr 8: 13.03 – 13.04 Mb |
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| PubMed search | [1] | [2] | |||||||||||
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Factor VII (formerly known as proconvertin) is one of the proteins that causes blood to clot in the coagulation cascade. It is an enzyme (EC 3.4.21.21) of the serine protease class. A recombinant form of human factor VIIa (NovoSeven, eptacog alfa [activated]) has U.S. Food and Drug Administration approval for uncontrolled bleeding in hemophilia patients. It is sometimes used unlicensed in severe uncontrollable bleeding, although there have been safety concerns. A Biosimilar form of recombinant activated factor VII (AryoSeven) is manufacturing by AryoGen Biopharma and since 2012 is available in the market.
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The main role of factor VII (FVII) is to initiate the process of coagulation in conjunction with tissue factor (TF/factor III). 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 decreases hepatic synthesis of FVII.
The gene for factor VII is located on chromosome 13 (13q34).
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 or AryoSeven).
Recombinant factor VIIa is used for people with hemophilia (with Factor VIII or IX deficiency) who have developed inhibitors against replacement coagulation factor.
It has also been used in the setting of uncontrollable hemorrhage,[2] but its role in this setting is controversial with insufficient evidence to support its use outside of clinical trials.[3] The first report of its use in hemorrhage was in an Israeli soldier with uncontrollable bleeding in 1999.[4] Risks of its use include an increase in arterial thrombosis.[3]
Recombinant human factor VII while initially looking promising in intracerebral hemorrhage falled to show benefit following further study and this is no longer recommended.[5][6]
Factor VII has been shown to interact with Tissue factor.[7][8]
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