Factor Viii

From Anvita Health Wiki

Contents 1 More Specific Terms 2 Function 3 Structure 4 Compartment 5 Expression 6 Pathology 7 Pharmacology 8 More General Terms 9 Additional Terms 10 Internet Database 11 References

More Specific Terms

• antihemophilic factor (coagulation factor VIII, AHF-M, Hemofil, Humate-P, Monoclate-P)
• recombinant factor VIII (Factorate, Recombinate, Xyntha)
• Refacto

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

• secreted, extracellular space

Expression

• synthesized by vascular endothelium & released into the bloodstream

Pathology

• deficiency associated with hemophilia A

Pharmacology

• see antihemophilic factor

More General Terms

• coagulation factor
• protein precursor
• glycoprotein
• multisubunit protein

Additional Terms

• coagulation cascade
• coagulation factor VIII alloantibody
• coagulation factor VIII autoantibody
• coagulation factor VIIIa
• factor VIII intron 22 protein (CpG island protein)
• hemophilia A; factor VIII deficiency
• von Willebrand factor; vWF; ristocetin cofactor; contains: von Willebrand antigen 2 (VWF, F8VWF)

Internet Database

Entrez gene: 2157
Kegg: [1]
OMIM: 134500
OMIM: 306700
Prosite: [2]
Prosite: [3]
Prosite: [4]
Prosite: [5]
UniProt: [6]

References

1. Walker FJ, Fay PJ. Regulation of blood coagulation by the protein C system. FASEB J. 1992 May;6(8):2561-7. Review. PMID: [7]
2. 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: [8]
3. Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
4. Wikipedia: Factor VIII [9]
5. UniProt [10]
6. HAMsters; Note: factor VIII mutation db [11]
7. GeneReviews [12]
8. SeattleSNPs [13]

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)