coagulation factor VIII; antihemophilic factor; AHF; procoagulant component; contains: factor VIIIa heavy chain, 200 kD & 92 isoforms; factor VIII B chain; factor VIIIa light chain (F8, F8C)
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Function
- coagulation factor, intrinsic pathway
- zymogen activated by thrombin to factor VIIIa
- factor VIII, along with Ca+2 & phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa
- in the circulating blood, it is mainly bound to vWF
- sulfation on Tyr-1699 is essential for binding vWF
- vWF binding is essential for stabilization of F8 in circulation
Structure
- domain F5/8 type C 2 is responsible for phospholipid- binding & essential for factor VIII activity
- belongs to the multicopper oxidase family
- contains 3 F5/8 type A domains
- contains 2 F5/8 type C domains
- contains 6 plastocyanin-like domains
- subunits contain domains homologous to factor V & to ceruloplasmin
Compartment
Expression
- synthesized by vascular endothelium & released into the bloodstream
Pathology
deficiency associated with hemophilia A
Pharmacology
Laboratory
More general terms
More specific terms
- antihemophilic factor (coagulation factor VIII, AHF-M, Hemofil, Humate-P, Monoclate-P)
- recombinant factor VIII Fc fusion protein; recombinant antihemophilic factor Fc fusion protein; recombinant antihemophilic factor Fc-VWF-XTEN fusion protein-ehtl (Eloctate, Altuviiio)
- recombinant factor VIII; turoctocog alfa; (Factorate, Recombinate, Xyntha, Kogenate FS, Kovaltry, NovoEight)
- Refacto
Additional terms
- coagulation cascade
- coagulation factor VIII alloantibody
- coagulation factor VIII autoantibody (acquired hemophilia)
- coagulation factor VIIIa
- factor VIII intron 22 protein (CpG island protein)
- hemophilia A; factor VIII deficiency
- von Willebrand factor; vWF; ristocetin cofactor; factor VIII related antigen; contains: von Willebrand antigen 2 (VWF, F8VWF)
Component of
References
- ↑ Walker FJ, Fay PJ. Regulation of blood coagulation by the protein C system. FASEB J. 1992 May;6(8):2561-7. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/1317308
- ↑ Andrews BS. Is the WKS motif the tissue-factor binding site for coagulation factor VII? Trends Biochem Sci. 1991 Jan;16(1):31-6. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2053135
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 18, American College of Physicians, Philadelphia 1998, 2018.
- ↑ Wikipedia: Factor VIII http://en.wikipedia.org/wiki/Factor_VIII
- ↑ UniProt http://www.uniprot.org/uniprot/P00451.html
- ↑ HAMsters; Note: factor VIII mutation db http://hadb.org.uk/WebPages/main/main.htm
- ↑ GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/F8
- ↑ SeattleSNPs http://pga.gs.washington.edu/data/f8/