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蛇毒巴曲酶和鱼精蛋白诱导的血小板型血管性血友病中的血小板聚集

Botrocetin- and polybrene-induced platelet aggregation in platelet-type von Willebrand disease.

作者信息

Takahashi H, Nagayama R, Hattori A, Shibata A

出版信息

Am J Hematol. 1985 Feb;18(2):179-89. doi: 10.1002/ajh.2830180209.

Abstract

It has been reported that botrocetin, a Bothrops venom factor, induces platelet aggregation dependent on von Willebrand factor (vWF), and that platelet aggregation induced by Polybrene, a synthetic polycation, is enhanced by vWF. This report describes the platelet aggregability on stimulation with botrocetin and Polybrene in four patients with platelet-type von Willebrand disease (vWD) who showed increased platelet aggregation with low concentrations of ristocetin as the result of a platelet abnormality. Enhanced platelet aggregability with botrocetin was observed in platelet-rich plasma (PRP) from the patients. Platelet aggregation induced by botrocetin in a mixture of normal washed platelets and patient plasma was either decreased or normal, being dependent on the amount of plasma vWF. In contrast with ristocetin and botrocetin, Polybrene did not cause increased aggregation of patient PRP. Polybrene aggregated normal washed platelets less extensively in the presence of patient plasma than normal plasma. These studies demonstrated that botrocetin induced heightened interaction between platelets and vWF, but Polybrene did not, in platelet-type vWD, and that the enhanced responsiveness of patient platelets to botrocetin is related to an intrinsic platelet abnormality.

摘要

据报道,矛头蝮蛇毒因子(botrocetin),一种矛头蝮蛇毒液因子,可诱导依赖于血管性血友病因子(vWF)的血小板聚集,并且合成聚阳离子鱼精蛋白(Polybrene)诱导的血小板聚集会被vWF增强。本报告描述了4例血小板型血管性血友病(vWD)患者在用矛头蝮蛇毒因子和鱼精蛋白刺激时的血小板聚集能力,这些患者由于血小板异常,在低浓度瑞斯托霉素作用下血小板聚集增加。在患者的富血小板血浆(PRP)中观察到用矛头蝮蛇毒因子刺激时血小板聚集能力增强。在正常洗涤血小板与患者血浆的混合物中,矛头蝮蛇毒因子诱导的血小板聚集要么降低要么正常,这取决于血浆vWF的量。与瑞斯托霉素和矛头蝮蛇毒因子不同,鱼精蛋白不会导致患者PRP聚集增加。在患者血浆存在的情况下,鱼精蛋白使正常洗涤血小板的聚集程度低于正常血浆。这些研究表明,在血小板型vWD中,矛头蝮蛇毒因子诱导血小板与vWF之间的相互作用增强,但鱼精蛋白不会,并且患者血小板对矛头蝮蛇毒因子反应性增强与血小板内在异常有关。

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