Title: Factor VIII
CAS Registry Number: 9001-27-8
CAS Name: Blood-coagulation factor VIII
Additional Names: antihemophilic globulin; antihemophilic factor A; AHG; AHF
Trademarks: Alphanate (Alpha Ther.); Hemofil-M (Baxter); Humate-P (Centeon); Koate-HP (Bayer); Monoclate-P (Centeon); Nordiocto (Nordisk); Profilate (Alpha Ther.)
Literature References: Crucial, nonenzymatic cofactor in the intrinsic coagulation pathway leading to the localized generation of thrombin. Deficiency results in the bleeding disorder known as hemophilia A or classical hemophilia. Factor VIII is an X-linked gene product synthesized by hepatocytes. Mature, single chain protein contains 2332 amino acid residues; mol wt 265 kDa. Released into the circulation as a set of heterodimers that rapidly interact with von Willebrand factor to form a stable, noncovalent complex; present at extremely low concn in plasma. Converted by thrombin into a heterotrimer (factor VIIIa) that accelerates the activation of factor X by factor IXa. Inactivated by protein C. Identification in plasma: T. Addis, J. Pathol. Bacteriol. 15, 427 (1911). Role in hemostasis: A. J. Patek, Jr., F. H. L.Taylor, J. Clin. Invest. 16, 113 (1937). Isoln and characterization of human factor VIII: E. J. Hershgold et al., J. Lab. Clin. Med. 77, 185 (1971). Purification and properties of bovine: G. A. Vehar, E. W. Davie, Biochemistry 19, 401 (1980). Characterization of the human factor VIII gene: J. Gitschier et al., Nature 312, 326 (1984). Series of articles on cloning, expression, and structure: ibid. 330-347. Review of bioregulation: R. J. Kaufman, Annu. Rev. Med. 43, 325-339 (1992). Review of production methods and clinical use in treatment of factor VIII deficiency: J. C. Gill, Semin. Thromb. Hemostasis 19, 1-12 (1993). Review of biosynthesis, structure and function: P. Lollar, Adv. Exp. Med. Biol. 386, 3-17 (1995); P. J. Lenting et al., Blood 92, 3983-3996 (1998).
Derivative Type: Kogenate
CAS Registry Number: 169149-90-0
Literature References: Recombinant human factor VIII produced in baby hamster kidney cells. Review of manufacturing process: H.-D. Hörlein, Prog. Biotechnol. 9, 731-734 (1994). Clinical trial in treatment of hemophilia: E. Aygören-Pürsün et al., Thromb. Haemostasis 78, 1352 (1997).
Derivative Type: Recombinate
Literature References: Recombinant human factor VIII produced in Chinese hamster ovary cells. Review of manufacturing process: E. Gomperts et al., Transfus. Med. Rev. 6, 247-251 (1992). Clinical trial in treatment of hemophilia: G. C. White II et al., Thromb. Haemostasis 77, 660 (1997).
Therap-Cat: Antihemophilic factor (human).
Keywords: Hemostatic; Antihemophilic Factor. |