凝血酶原希米；一种以凝血酶活性缺陷为特征的异常凝血酶原。

Prothrombin Himi; an abnormal prothrombin characterized by a defective thrombin activity.

作者信息

Morishita E, Saito M, Asakura H, Jokaji H, Uotani C, Kumabashiri I, Yamazaki M, Hachiya H, Okamura M, Matsuda T

机构信息

Department of Internal Medicine (III), Kanazawa University School of Medicine, Japan.

出版信息

Thromb Res. 1991 Jun 15;62(6):697-706. doi: 10.1016/0049-3848(91)90373-5.

DOI:10.1016/0049-3848(91)90373-5

PMID:1926060

Abstract

An abnormal prothrombin has been detected in a 26-year-old female, who had no history of excessive bleeding. Prothrombin activity was approximately 10% when measured using either the classical one-stage assay or the assay with Echis carinatus venom, whereas prothrombin antigen level was normal. In keeping with current nomenclature practices, the abnormal prothrombin was designated "Prothrombin Himi". The electrophoretic behavior and calcium binding properties of Prothrombin Himi did not differ significantly from normal. Prothrombin Himi was isolated by chromatography on Q-Sepharose. Electrophoretic migration of the purified abnormal prothrombin on SDS-PAGE was normal. Upon prothrombin activation by Echis carinatus venom, the clotting activity produced from Prothrombin Himi was only 37% of the normal level after 90 minutes of the activation time, where as the amidolytic activity was almost the same as normal. The cleavage patterns of Prothrombin Himi by factor Xa or Echis carinatus venom investigated by SDS-PAGE, were found to be normal. These results indicate that Prothrombin Himi was characterized by a defective thrombin enzymatic activity.

摘要

在一名26岁无过度出血史的女性中检测到一种异常凝血酶原。使用经典的一步法测定或用锯鳞蝰蛇毒进行测定时，凝血酶原活性约为10%，而凝血酶原抗原水平正常。按照当前的命名惯例，该异常凝血酶原被命名为“凝血酶原日米”。凝血酶原日米的电泳行为和钙结合特性与正常情况无显著差异。通过Q-琼脂糖柱层析分离出凝血酶原日米。纯化的异常凝血酶原在SDS-PAGE上的电泳迁移正常。在用锯鳞蝰蛇毒激活凝血酶原后，激活90分钟后，凝血酶原日米产生的凝血活性仅为正常水平的37%，而酰胺水解活性与正常情况几乎相同。通过SDS-PAGE研究因子Xa或锯鳞蝰蛇毒对凝血酶原日米的切割模式，发现其正常。这些结果表明，凝血酶原日米的特征是凝血酶酶活性存在缺陷。