凝血因子IX 德文特研究——血友病B异质性的证据
Factor IX Deventer-evidence for the heterogeneity of hemophilia BM.
作者信息
Bertina R M, van der Linden I K
出版信息
Thromb Haemost. 1982 Apr 30;47(2):136-40.
Factor IX Deventer was isolated from the plasma of a patient with severe hemophilia B. The patient was classified as BM because of an abnormal prolongation (2.1 times) of the ox-brain prothrombin time, that could be corrected by addition of antifactor IX serum. Experiments with the isolated factor IX Deventer showed that one of the two peptide bonds involved in the proteolytic activation of factor IX cannot be cleaved by physiological or non-physiological activators (XIa and RVV-X, respectively). Such a defect can explain why the molecule has no procoagulant activity. At present it is not clear why this defect makes factor IX Deventer such an effective inhibitor of the ox-brain prothrombin time. It is proposed that hemophilia BM is a heterogeneous disorder.
因子IX代芬特尔是从一名严重B型血友病患者的血浆中分离出来的。由于牛脑凝血酶原时间异常延长（2.1倍），该患者被归类为BM型，添加抗因子IX血清后可纠正这一异常。对分离出的因子IX代芬特尔进行的实验表明，参与因子IX蛋白水解激活的两个肽键之一不能被生理或非生理激活剂（分别为XIa和蛇毒凝血酶-X）裂解。这样的缺陷可以解释为什么该分子没有促凝活性。目前尚不清楚为什么这种缺陷会使因子IX代芬特尔成为牛脑凝血酶原时间的有效抑制剂。有人提出BM型血友病是一种异质性疾病。
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