摘要
目的 探讨血小板无力症 (Glanzmann’sThrombasthenia ,GT)患者血小板聚集功能异常的可能机制。方法 采用Western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。
Objective:To investigate the mechanism of the platelet aggregative dysfunction in patients with Glanzmann′s Thrombasthenia.Methods:Flow cytometry and Western blotting were used to detect the ability of platelet binding PAC-1 and the expressions of platelet membrane GPⅡbⅢa.Results:In the patients with GT, GPⅡb and GPⅢa expressions were remarkably decreased, and the ability of platelet binding to activated mono-antibody PAC-1 was lower than that of normal subjects. In his father, GPⅡbⅢa 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 receptor GPⅡbⅢa activated by ADP, which is probably one of the molecular basis of the platelet aggregative dysfunction in patients with GT.
出处
《中国现代医学杂志》
CAS
CSCD
2004年第3期34-37,共4页
China Journal of Modern Medicine
