血小板无力症患者血小板GPⅡbⅢa受体亲和力的改变及临床意义

摘要

Objective:To investigate the mechanism of the platelet aggregative dysfunction in patients with let binding PAC-1 and the expressions of platelet membrane GP Ⅱ b Ⅲ a.Results: In the patients with GT,GP Ⅱ b and GPⅢ a expressions were remarkably decreased,and the ability of platelet binding Objective:To activated mono-antibody PAC-1 was lower than that of normal subjects.In his father,GP Ⅱ b Ⅲ a was relatively lower,but PAC-1 binding was normal.In his mother,results were not different from those of normal subjects.Conclusions: The patients with GT have dysfunction of fibrinogen recepObjective:Tor GP Ⅱ bⅢ a activated by ADP,which is probably one of the molecular basis of the platelet aggregative dysfunction in patients with GT.%目的探讨血小板无力症(Glanzmann's Thrombasthenia,GT)患者血小板聚集功能异常的可能机制.方法采用Westem印迹分别检测正常人、GT患者及其父母血小板CPⅡbⅢa的含量,流式细胞术分析血小板膜CPⅢa的表达和血小板结合活化型单抗PAC-1的能力.结果与正常人相比,GT患者GPⅡbⅢa明显减少,其血小板结合PAC-1的能力亦明显低下.结论GPⅡbⅢa激活受阻可能为部分GT患者血小板聚集功能障碍的分子基础.