【摘 要】
:
Factor Ⅷ (FⅧ) is a large protein that is essential for normal hemostasis.Activated factor Ⅷ (FⅢa) serves a cofactor function,increasing the catalytic rate of the serine protease factor Ⅸa (FⅨa) by fou
【机 构】
:
Puget Sound Blood Center University of Washington USA
【出 处】
:
2008中国深圳蛋白质和多肽科学大会
论文部分内容阅读
Factor Ⅷ (FⅧ) is a large protein that is essential for normal hemostasis.Activated factor Ⅷ (FⅢa) serves a cofactor function,increasing the catalytic rate of the serine protease factor Ⅸa (FⅨa) by four orders of magnitude.Deficiencies in FⅧ lead to the bleeding disorder hemophilia A,which affects one in 5000 males worldwide.Hemophilia A may be treated by infusions with recombinant or plasma-derived FⅧ.Unfortunately,the FⅧ protein is highly immunogenic,and approximately one quarter of patients who receive these infusions develop an immune response in which anti-FⅧ antibodies block its procoagulant function.Most of the inhibitory IgGs prevent FⅧa binding to phospholipid membranes or FIXa,and they bind primarily to the A2 and C2 domains of FⅧ.
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