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20-September-2008 09:55:53 - Factor VIII Coagulation factor VIII, procoagulant component hemophilia A PDB rendering based on 1d7p. Available structures: 1d7p, 1iqd Identifiers Symbols F8; AHF; DXS1253E; F8 protein; F8B; F8C; FVIII; HEMA External IDs OMIM: 306700 MGI: 88383 HomoloGene: 49153 Gene ontology Molecular function: copper ion binding calcium ion binding oxidoreductase activity Cellular component: extracellular region Biological process: acute-phase response cell adhesion blood coagulation RNA expression pattern More reference expression data Orthologs Human Mouse Entrez 2157 14069 Ensembl ENSG00000185010 ENSMUSG00000031196 Uniprot P00451 Q684Q7 Refseq NM_000132 mRNA NP_000123 protein NM_007977 mRNA NP_032003 protein Location Chr X: 153.72 - 153.9 Mb Chr X: 71.43 - 71.64 Mb Pubmed search 1 2 Factor VIII FVIII is an essential clotting factor. A damaged FVIII causes Hemophilia A, a bleeding disorder. This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder.1 Contents 1 Genetics 2 Physiology 3 Therapeutic use 4 See Also 5 References 6 Further reading 7 External links Genetics The gene for Factor VIII is located on the X chromosome Xq28. The gene for factor VIII presents an interesting primary structure, as another gene is embedded in one of its introns.2. Physiology FVIII is a glycoprotein procofactor synthesized and released into the bloodstream by the endothelium. In the circulating blood, it is mainly bound to von Willebrand factor vWF, also known as Factor VIII-related antigen to form a stable complex. Upon activation by thrombin, Factor IIa, it dissociates from the complex to interact with Factor IXa the coagulation cascade. It is a cofactor to Factor IXa in the activation of Factor X, which, in turn, with its cofactor Factor Va, activates more thrombin. Thrombin cleaves fibrinogen into fibrin which polymerizes and crosslinks using Factor XIII into a blood clot. No longer protected by vWF, activated FVIII is proteolytically inactivated in the process most prominently by activated Protein C and Factor IXa and quickly cleared from the blood stream. Factor VIII is synthesized predominantly in the vascular endothelium and is not affected by liver disease. In fact, levels usually are elevated in such instances.3 Therapeutic use FVIII concentrated from donated blood plasma Aafact, or alternatively recombinant FVIII can be given to hemophiliacs to restore hemostasis. Thus, FVIII is also known as Anti-hemophilic factor. The transfer of a plasma byproduct into the blood stream of a patient with hemophilia often led to the transmission of diseases such as HIV and hepatitis B and C before purification methods were improved. Antibody formation to Factor VIII can also be a major concern for patients receiving therapy against bleeding; the incidence of these inhibitors is dependent of various factors, including the Factor VIII product itself.4 In the early 1990s, pharmaceutical companies began to produce recombinant synthesized factor products, which now prevent nearly all forms of disease transmission during replacement therapy. In particular, some pharmaceutical companies such as Bayer sparked controversy by continuing to sell contaminated factor VIII after new heat-treated versions were available.5 See Also Ryan White References ^ Entrez Gene: F8 coagulation factor VIII, procoagulant component hemophilia A. ^ Levinson B, Kenwrick S, Lakich D, Hammonds G, Gitschier J 1990. A transcribed gene in an intron of the human factor VIII gene. Genomics 7 1: 1-11. doi:10.1016/0888-75439090512-S. PMID 2110545. ^ R. Rubin, L. Leopold 1998. Hematologic Pathophysiology. Madison, Conn: Fence Creek Publishing. ISBN 1-889325-04-X. ^ = Overview of Factor VIII Inhibitors. ^ 2 Paths of Bayer Drug in 80's: Riskier One Steered Overseas. Further reading Gitschier J 1991. The molecular basis of hemophilia A. Ann. N. Y. Acad. Sci. 614: 89-96. doi:10.1111/j.1749-6632.1991.tb43694.x. PMID 1902642. White GC, Shoemaker CB 1989. Factor VIII gene and hemophilia A. Blood 73 1: 1-12. PMID 2491949. Antonarakis SE, Kazazian HH, Tuddenham EG 1995. Molecular etiology of factor VIII deficiency in hemophilia A. Hum. Mutat. 5 1: 1-22. doi:10.1002/humu.1380050102. PMID 7728145. Lenting PJ, van Mourik JA, Mertens K 1999. The life cycle of coagulation factor VIII in view of its structure and function. Blood 92 11: 3983-96. PMID 9834200. Saenko EL, Ananyeva N, Kouiavskaia D, et al. 2003. Molecular defects in coagulation Factor VIII and their impact on Factor VIII function. Vox Sang. 83 2: 89-96. PMID 12201837. Lollar P 2003. Molecular characterization of the immune response to factor VIII. Vox Sang. 83 Suppl 1: 403-8. PMID 12617176. Fay PJ 2004. Activation of factor VIII and mechanisms of cofactor action. Blood Rev. 18 1: 1-15. doi:10.1016/S0268-960X0300025-0. PMID 14684146. Lavigne-Lissalde G, Schved JF, Granier C, Villard S 2005. Anti-factor VIII antibodies: a 2005 update. Thromb. Haemost. 94 4: 760-9. doi:10.1160/TH05-02-0118. PMID 16270627. Fang H, Wang L, Wang H 2007. The protein structure and effect of factor VIII. Thromb. Res. 119 1: 1-13. doi:10.1016/j.thromres.2005.12.015. PMID 16487577. External links The Coagulation Factor VIII Protein MeSH Factor+VIII v d e Proteins: coagulation Coagulation factors primary hemostasis: vWF intrinsic pathway: HMWK/Bradykinin - Kallikrein - Hageman / FXII - FXI - FIX - FVIII extrinsic pathway: Tissue factor / FIII - FVII common pathway: FX - FV - Prothrombin / FII, Fibrin / FI - FXIII Coagulation inhibitors Antithrombin inhibits II, IX, X, XI, XII - Protein C inhibits V, VIII/Protein S cofactor for protein C - Protein Z inhibits X - ZPI inhibits X, XI - TFPI inhibits III Fibrinolysis Plasmin - tPA/urokinase - PAI-1/2 - α2-AP - α2-macroglobulin - TAFI Retrieved from http://en..org/wiki/Factor_VIII Categories: Genes on chromosome X | Human proteins | Acute phase proteins | Recombinant proteins | Coagulation system | Genes associated with genetic disorders | Cofactors Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Français Polski Português This page was last modified on 17 August 2008, at 21:54
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