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一名出血性疾病患者血小板中磷脂暴露减少,导致凝血因子X和凝血酶原激活受损。

Impaired factor X and prothrombin activation associated with decreased phospholipid exposure in platelets from a patient with a bleeding disorder.

作者信息

Rosing J, Bevers E M, Comfurius P, Hemker H C, van Dieijen G, Weiss H J, Zwaal R F

出版信息

Blood. 1985 Jun;65(6):1557-61.

PMID:3995186
Abstract

Platelets from a platelet factor 3-deficient patient, which was first described by Weiss et al (Am J Med 67:206, 1979), were found to be equally impaired in their ability to promote factor X and prothrombin activation. Compared to normal platelets, the patient's platelets showed upon stimulation with thrombin plus collagen a much slower generation and a considerably lower level of platelet prothrombin- and factor X-converting activities. Treatment of stimulated platelets with phospholipases revealed a decreased exposure of negatively charged phospholipid at the outer surface of the patient's platelets, relative to control's. We suggest that the combined impairment of prothrombin- and factor X-converting activities in this patient is due to a defect in the mechanism by which phosphatidylserine becomes exposed at the outer surface of stimulated platelets.

摘要

血小板第3因子缺乏症患者的血小板,最早由魏斯等人(《美国医学杂志》67:206,1979年)描述,其促进因子X和凝血酶原激活的能力同样受损。与正常血小板相比,该患者的血小板在用凝血酶加胶原蛋白刺激后,生成速度慢得多,血小板凝血酶原和因子X转化活性水平也低得多。用磷脂酶处理受刺激的血小板后发现,相对于对照组,该患者血小板外表面带负电荷磷脂的暴露减少。我们认为,该患者凝血酶原和因子X转化活性的联合受损是由于磷脂酰丝氨酸在受刺激血小板外表面暴露机制存在缺陷所致。

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