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凝血因子Ⅷ(FⅧ)是一种重要的凝血辅因子,其缺乏将导致血友病A。研究FⅧ结构与功能的关系,将为血友病及血栓病的防治带来新的思路。FⅧ分泌受内质网内驻蛋白调节。成熟的FⅧ分为3个A区、1个B区和2个C区。vWF与FⅧ的a3区和C2区结合,以维持FⅧ稳定及调节其活性。FⅧ被凝血酶激活,变为活化的FⅧa,其分别通过C2区、A3区和a1区与磷脂、FⅨa及因子Ⅹ(FⅩ)结合。活化的蛋白C和FⅩ可灭活FⅧa。
Factor Ⅷ (FⅧ) is an important coagulation cofactor, its lack of will lead to hemophilia A. Studying the relationship between structure and function of F Ⅷ will bring new ideas for the prevention and treatment of hemophilia and thrombosis. FⅧ secretion by the endoplasmic reticulum within the protein regulation. FⅧ is divided into mature three A zone, a B zone and two C zone. vWF binds to a3 region and C2 region of FⅧ to maintain the stability of FⅧ and regulate its activity. FⅧ is activated by thrombin to become activated FVIIIa, which binds to phospholipid, FIXa and factor X (FX) via the C2, A3 and a1 domains, respectively. Activated proteins C and FX can inactivate FVIIIa.