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抗凝血酶Ⅲ(AT-Ⅲ)是一种分子量约为65,000的α_2球蛋白,对凡以丝氨酸为活性中心的蛋白酶均有抑制作用。除能以进行性的方式灭活循环中的凝血酶外,对血液中其它凝血因子,如Ⅻ_a、Ⅺ_a、Ⅹ_a、Ⅸ_a以及血浆素、激肽释放酶等亦有抑制作用。肝素作为一种催化剂加速AT-Ⅲ的抑制速度。因此,AT-Ⅲ对维持血液凝固功能的正常状态具重要意义。自1965年Egeberg首先报道家族性AT-Ⅲ缺乏症以来,欧洲和北美陆续报道,至今已发现20多个遗传性AT-Ⅲ缺陷的家系。家族性AT-Ⅲ缺陷是严重的,常伴有高血栓发生率可导致猝死。本文简要复习有关文献,以期对此遗传性疾病有所认识。
Antithrombin III (AT-III) is an α 2 -globulin with a molecular weight of about 65,000, which inhibits all serine-active proteases. In addition to being capable of inactivating circulating thrombin in a progressive manner, other blood clotting factors, such as Ⅻ_a, Ⅺ_a, Ⅹ_a, Ⅸ_a, and plasma kallikrein, as well as kallikrein, are also inhibited. Heparin acts as a catalyst to accelerate the rate of AT-III inhibition. Therefore, AT-III is important for maintaining the normal state of blood coagulation. Since Egeberg first reported familial AT-III deficiency in 1965, Europe and North America reported successively that so far more than 20 families with inherited AT-III deficiency have been found. Familial AT-III deficiency is serious, often accompanied by a high incidence of thrombosis can lead to sudden death. This article briefly reviews the literature, with a view to understanding this genetic disease.