Shomura I, Nagao N, Sawada Y
Biochem Med. 1985 Jun;33(3):350-61. doi: 10.1016/0006-2944(85)90010-9.
In our previous research, we found that the level of the plasminogen activity in the plasma from Duchenne-type patients with progressive muscular dystrophy was higher than of the normal boys, though the level of the plasmin inhibitors was lower. Therefore, in the present study, we investigated the differences in the fractions of plasmin inhibitors. The subjects were nine patients (the average age being 17.1 years) who had been diagnosed, by clinical and biochemical tests, as having PMD; serving as controls were normal boys (the average age being 15 years), the patients' mothers, and the mothers of the normal boys. The plasmin inhibitors were separated from plasma using lysine-Sepharose columns according to the method of Urita et al. The determination was performed based on the method of Aoyagi et al. and an immunoreactive assay. The results were as follows: (1) No significant differences were seen between patients with PMD and control subjects with respect to either alpha 1-antichymotrypsin, antithrombin III, and alpha 1-antitrypsin or alpha 2-macroglobulin and inter-alpha-trypsin inhibitors. These results suggested that the low level of plasmin inhibitors in patients was due to the low activity of the C1 inactivator. (2) The patients with PMD showed lower values than the normal boys in the levels of C1 inactivator in plasma; similarly, the mothers of these patients showed lower values than the normal mothers.
在我们之前的研究中，我们发现，进行性肌营养不良的杜氏型患者血浆中的纤溶酶原活性水平高于正常男孩，尽管纤溶酶抑制剂水平较低。因此，在本研究中，我们调查了纤溶酶抑制剂各组分的差异。研究对象为9例经临床和生化检测确诊为进行性肌营养不良的患者（平均年龄17.1岁）；正常男孩（平均年龄15岁）、患者母亲以及正常男孩的母亲作为对照。按照Urita等人的方法，使用赖氨酸-琼脂糖柱从血浆中分离纤溶酶抑制剂。测定基于Aoyagi等人的方法和免疫反应测定法进行。结果如下：（1）在α1-抗糜蛋白酶、抗凝血酶III和α1-抗胰蛋白酶，或α2-巨球蛋白和α-胰蛋白酶抑制剂方面，进行性肌营养不良患者与对照受试者之间未观察到显著差异。这些结果表明，患者纤溶酶抑制剂水平低是由于C1灭活剂活性低所致。（2）进行性肌营养不良患者血浆中C1灭活剂水平低于正常男孩；同样，这些患者的母亲的C1灭活剂水平低于正常母亲。
