Sas G, Petö I, Bánhegyi D, Blaskó G, Domján G
Thromb Haemost. 1980 Jun 18;43(2):133-6.
We investigated two thrombophilic families with the "classical" type of antithrombin III deficiency, i.e., with a low antithrombin III level measured both by immunochemical and functional methods. We obtained different antithrombin III patterns in the plasma of the affected members of the two families with the modified two dimensional immunoelectrophoresis method (heparin in agarose). In one family, the electrophoretic mobility of the antithrombin III is identical with that of normal antithrombin III. In the other, the antithrombin III displayed a decreased electrophoretic mobility in the heparinized agarose gel. The relatively low affinity of this antithrombin III to heparin could be directly proved by the heparin-agarose affinity chromatography, too. These two different antithrombin III patterns were observed by other investigators at different families as well. On the basis of our simultaneous observations of these two families we propose a classification of the inherited congenital antithrombin III deficiencies.
我们研究了两个患有“经典”型抗凝血酶III缺乏症的血栓形成倾向家族，即通过免疫化学和功能方法测得抗凝血酶III水平均较低。我们采用改良的二维免疫电泳法（琼脂糖中的肝素）在这两个家族受影响成员的血浆中获得了不同的抗凝血酶III图谱。在一个家族中，抗凝血酶III的电泳迁移率与正常抗凝血酶III相同。在另一个家族中，抗凝血酶III在肝素化琼脂糖凝胶中显示出降低的电泳迁移率。这种抗凝血酶III与肝素相对较低的亲和力也可通过肝素-琼脂糖亲和层析直接证明。其他研究者在不同家族中也观察到了这两种不同的抗凝血酶III图谱。基于我们对这两个家族的同步观察，我们提出了遗传性先天性抗凝血酶III缺乏症的分类。
