Liu Y, Lyons R M, McDonagh J
Department of Pathology, Beth Israel Hospital, Boston, MA 02215.
Thromb Haemost. 1988 Feb 25;59(1):49-53.
An abnormal plasminogen (San Antonio) has been isolated from a patient with axillary vein thrombosis. A decreased level of fibrinolytic activity was detected in both plasma and a purified system. The molecular abnormalities were investigated with both functional and immunological tests. Slightly decreased antigen concentration was noted in plasma. By crossed immunoelectrophoresis, the patient and his two children had a second small arc and the primary arc migrated more cathodically. A distinct isozyme was detected in the abnormal plasminogen. Functionally, this abnormal plasminogen is characterized by failure to enhance maximal conversion to plasmin, especially by plasminogen activators, which are enhanced by fibrin or fibrin degradation products. The proband and his children are heterozygous for this abnormal plasminogen.
从一名患有腋静脉血栓形成的患者中分离出了一种异常的纤溶酶原(圣安东尼奥型)。在血浆和纯化系统中均检测到纤溶活性水平降低。通过功能和免疫测试对分子异常进行了研究。血浆中抗原浓度略有降低。通过交叉免疫电泳发现,该患者及其两个孩子有第二条小弧,且主弧向阴极迁移得更多。在异常纤溶酶原中检测到一种独特的同工酶。在功能上,这种异常纤溶酶原的特征是不能增强向纤溶酶的最大转化率,尤其是不能被纤溶酶原激活剂增强,而纤溶酶原激活剂会被纤维蛋白或纤维蛋白降解产物增强。先证者及其孩子均为这种异常纤溶酶原的杂合子。