两种病理性抗凝血酶III分子的鉴别：抗凝血酶III“Aalborg”和抗凝血酶III“Budapest”

Distinction of two pathologic antithrombin III molecules: antithrombin III "Aalborg' and antithrombin III "Budapest'.

作者信息

Sørensen P J, Sas G, Petó I, Blaskó G, Kremmer T, Samu A

出版信息

Thromb Res. 1982 May 1;26(3):211-9. doi: 10.1016/0049-3848(82)90142-6.

Abstract

Plasma from two different thrombophilic families with functional inherited antithrombin III deficiency, i.e., with low antithrombin III activity but normal immunoreactive antithrombin III concentration, were investigated simultaneously in the same laboratory. The experiments (thrombin and Factor Xa inactivation, heparin affinity chromatography, modified two dimensional immunoelectrophoresis and gel filtration) showed a distinct difference between the two antithrombin III anomalies. The antithrombin III "Aalborg' had decreased thrombininactivating activity but normal Factor Xa-inactivating activity. The heparin affinity and the molecule weight are normal. The antithrombin III "Budapest' displays a more profound abnormality with pathologic thrombin and Factor Xa inactivation, decreased heparin affinity and abnormal molecular weight.

摘要

在同一实验室同时研究了来自两个不同血栓形成倾向家族的血浆，这两个家族存在功能性遗传性抗凝血酶III缺乏，即抗凝血酶III活性低但免疫反应性抗凝血酶III浓度正常。实验（凝血酶和因子Xa灭活、肝素亲和层析、改良二维免疫电泳和凝胶过滤）显示两种抗凝血酶III异常之间存在明显差异。抗凝血酶III“Aalborg”的凝血酶灭活活性降低，但因子Xa灭活活性正常。肝素亲和力和分子量正常。抗凝血酶III“Budapest”表现出更严重的异常，凝血酶和因子Xa灭活异常、肝素亲和力降低和分子量异常。