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遗传性抗凝血酶III功能障碍新变异型(抗凝血酶III“维琴察”)中凝血酶诱导异常的免疫电泳证据。

Immunoelectrophoretic evidence of a thrombin-induced abnormality in a new variant of hereditary dysfunctional antithrombin III (AT III 'Vicenza').

作者信息

Barbui T, Finazzi G, Rodeghiero F, Dini E

出版信息

Br J Haematol. 1983 Aug;54(4):561-5. doi: 10.1111/j.1365-2141.1983.tb02134.x.

DOI:10.1111/j.1365-2141.1983.tb02134.x
PMID:6871107
Abstract

An Italian family with thrombosis and hereditary dysfunctional AT III, i.e. reduced biological activity and normal levels of the immunoreactive protein is presented. The abnormal molecule, called AT III 'Vicenza', was characterized by two-dimensional crossed immunoelectrophoresis either in the absence or presence of heparin. In the presence of heparin, a normal pattern was found in plasma, but abnormality was evident in serum. A similar abnormality was found in plasma artificially clotted with thrombin. The role of thrombin in inducing the immunoelectrophoretic abnormality of AT III 'Vicenza' is discussed.

摘要

本文介绍了一个患有血栓形成和遗传性抗凝血酶III功能障碍的意大利家庭,即免疫反应性蛋白水平正常但生物活性降低。这种异常分子被称为抗凝血酶III“维琴察”,通过二维交叉免疫电泳在有无肝素的情况下进行了表征。在有肝素存在的情况下,血浆中发现了正常模式,但血清中则明显异常。在用凝血酶人工凝结的血浆中也发现了类似的异常。讨论了凝血酶在诱导抗凝血酶III“维琴察”免疫电泳异常中的作用。

相似文献

1
Immunoelectrophoretic evidence of a thrombin-induced abnormality in a new variant of hereditary dysfunctional antithrombin III (AT III 'Vicenza').遗传性抗凝血酶III功能障碍新变异型(抗凝血酶III“维琴察”)中凝血酶诱导异常的免疫电泳证据。
Br J Haematol. 1983 Aug;54(4):561-5. doi: 10.1111/j.1365-2141.1983.tb02134.x.
2
Purification of antithrombin 'Vicenza': a molecule with normal heparin affinity and impaired reactivity to thrombin.抗凝血酶“维琴察”的纯化:一种具有正常肝素亲和力但对凝血酶反应性受损的分子。
Br J Haematol. 1985 Feb;59(2):259-63. doi: 10.1111/j.1365-2141.1985.tb02992.x.
3
Abnormal reactivity with thrombin of a new variant of dysfunctional antithrombin (AT 'Vicenza').功能异常抗凝血酶新变体(抗凝血酶“维琴察”)与凝血酶的异常反应性
Ric Clin Lab. 1984 Jul-Sep;14(3):515-9. doi: 10.1007/BF02904879.
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Antithrombin III Toyama: a hereditary abnormal antithrombin III of a patient with recurrent thrombophlebitis.富山抗凝血酶III:一名复发性血栓性静脉炎患者的遗传性异常抗凝血酶III 。
Thromb Res. 1983 Jul 15;31(2):305-17. doi: 10.1016/0049-3848(83)90333-x.
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A functional abnormal antithrombin III (AT III) deficiency: AT III Charleville.一种功能性异常抗凝血酶III(AT III)缺乏症:夏勒维尔AT III。
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Antithrombin III Glasgow: a variant with increased heparin affinity and reduced ability to inactivate thrombin, associated with familial thrombosis.抗凝血酶III格拉斯哥型:一种肝素亲和力增加但灭活凝血酶能力降低的变体,与家族性血栓形成有关。
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Antithrombin III Pescara: a defective AT III variant with no alterations of plasma crossed immunoelectrophoresis, but with an abnormal crossed immunoelectrofocusing pattern.
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A Swedish family with abnormal antithrombin III.
Scand J Haematol. 1985 May;34(5):412-6. doi: 10.1111/j.1600-0609.1985.tb00770.x.