Scharrer I, Hach-Wunderle V, Wohl R C, Sinio L, Boreisha I, Robbins K C
Dept. of Internal Medicine, University Hospital, Frankfurt/Main, FRG.
Haemostasis. 1988;18 Suppl 1:77-86. doi: 10.1159/000215841.
A new abnormal plasminogen, Frankfurt I, has been identified in the plasma of a 42 year-old male patients who had recurring thromboses, thrombophlebitis and pulmonary embolism since his age of 29. Reduced functional and also slightly reduced antigen plasminogen concentrations were found in both the proposituts and his mother. Plasmin generation rates carried out by Streptokinase and Urokinase were also abnormal. The plasmin generated was very unstable in the absence of stabilizing ligands and/or substrates. Crossed immunoelectrophoresis of the purified Frankfurt I revealed a peak with normal size and shape, but displaced with respect to normal Glu-plasminogen toward the anode. Isoelectric focusing followed by zymography on an agarose-fibrin plate proved this observation but did not indicate a separation of the normal from the abnormal plasminogen molecular species, also, fewer bands were found in the abnormal plasminogen isozyme pattern. Kinetic studies of Frankfurt I Glu-plasminogen and plasmin showed that most of the functional abnormality is related to absence of active sites in half of the molecules.
在一名42岁男性患者的血浆中发现了一种新的异常纤溶酶原，即法兰克福I型。该患者自29岁起就反复出现血栓形成、血栓性静脉炎和肺栓塞。在患者及其母亲体内均发现功能性纤溶酶原浓度降低，抗原纤溶酶原浓度也略有降低。由链激酶和尿激酶进行的纤溶酶生成率也异常。在没有稳定配体和/或底物的情况下，生成的纤溶酶非常不稳定。对纯化的法兰克福I型进行交叉免疫电泳显示，有一个峰，其大小和形状正常，但相对于正常的谷氨酸纤溶酶原向阳极偏移。在琼脂糖-纤维蛋白平板上进行等电聚焦后再进行酶谱分析证实了这一观察结果，但未显示正常和异常纤溶酶原分子种类的分离，而且在异常纤溶酶原同工酶图谱中发现的条带较少。对法兰克福I型谷氨酸纤溶酶原和纤溶酶的动力学研究表明，大多数功能异常与一半分子中活性位点的缺失有关。
