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[人血小板的膜糖蛋白:结构、功能以及血小板无力症和巨大血小板综合征中的异常情况]

[Membrane glycoproteins of human platelets: structures, functions, and abnormalities in Glanzmann's thrombasthenia and Bernard-Soulier syndrome].

作者信息

Ando Y, Fusegawa H, Kawada T, Tanaka Y, Watanabe K, Kobayashi N

出版信息

Rinsho Byori. 1989 Dec;37(12):1344-52.

PMID:2614964
Abstract

Platelet membrane glycoproteins, especially those of GP II b-III a complex and GP Ib-IX complex, serve as receptors for adhesive proteins including fibrinogen, von Willebrand factor and fibronectin, thus supporting cell-cell and cell-matrix interactions. Thrombasthenia (TA) and Bernard-Soulier syndrome (BSS) are inherited bleeding disorders in which these membrane glycoprotein complexes are defective. This paper deals with the recent advances in platelet membrane glycoprotein research, and the membrane glycoprotein abnormalities in TA and BSS platelets, which have been disclosed by the development of improved methods of analysis. Furthermore, a case of TA is reported in which flow cytometry using murine monoclonal antibody revealed 18% of the normal level of GP II b-III a complexes in her platelets. The platelets of this patient aggregated well by the addition of high concentrations of collagen (40 micrograms/ml). The results of this patient show the necessity of new criteria for the diagnosis of TA based on the quantification of the residual contents of GP II b-III a complexes in platelets in each patient.

摘要

血小板膜糖蛋白,尤其是糖蛋白IIb-IIIa复合物和糖蛋白Ib-IX复合物,作为包括纤维蛋白原、血管性血友病因子和纤连蛋白在内的黏附蛋白的受体,从而支持细胞间和细胞与基质间的相互作用。血小板无力症(TA)和伯-苏综合征(BSS)是遗传性出血性疾病,其中这些膜糖蛋白复合物存在缺陷。本文论述了血小板膜糖蛋白研究的最新进展,以及通过改进分析方法所揭示的TA和BSS血小板中的膜糖蛋白异常。此外,报告了1例TA病例,使用鼠单克隆抗体的流式细胞术显示其血小板中糖蛋白IIb-IIIa复合物水平为正常水平的18%。该患者的血小板在添加高浓度胶原蛋白(40微克/毫升)后聚集良好。该患者的结果表明,有必要根据每位患者血小板中糖蛋白IIb-IIIa复合物残留量的定量分析制定TA诊断的新标准。

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