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纤溶酶原激活剂抑制物-1(PAI-1)是纤溶系统的重要负调控因子,它与组织型或尿激酶型纤溶酶原激活剂形成1:1的复合物,抑制纤溶酶的产生。遗传性PAI-1缺乏症是一种罕见的常染色体隐性遗传的出血性疾病,国际上仅美国与日本有10余例报
Plasminogen activator inhibitor-1 (PAI-1), an important negative regulator of the fibrinolytic system, forms a 1: 1 complex with tissue-type or urokinase-type plasminogen activator and inhibits fibrinolysis Production of enzymes. Hereditary PAI-1 deficiency is a rare autosomal recessive hereditary hemorrhagic disease, the United States and Japan alone in the world there are more than 10 cases reported