出血性血小板病(HT)是一组血小板聚集异常性出血性疾病。中药益气活血方治疗该病疗效显著，但机理尚未完全阐明。本课题组长期致力HT研究，前期发现该病的vWF和凝血酶共同受体，血小板膜糖蛋白(GP)Ⅰb-Ⅸ-Ⅴ及其亚基GPIbα表达下调，主要原因是ADAM17过度水解GPIbα和GPⅤ胞外段功能区，导致血小板聚集不良。新近发现，ADAM17的上游分子伴侣iRhom2是ADAM17转运与蛋白水解的关键基因。iRhom2在内质网与ADAM17前体蛋白结合，一起转运至高尔基体，转化成熟ADAM17，最后定位于细胞膜，完成蛋白水解。为此本项目拟采用免疫印迹、激光共聚焦、流式细胞术、Real time-PCR等法观察iRhom2基因在HT中的表达及其特异性作用于ADAM17转运及水解的功能活性，并研究益气活血方的干预作用，以期进一步阐明HT发病机理和益气活血方作用靶点。

Hemorrhagic thrombopathy (HT) is considered as a group of hemorrhagic diseases characterized by dysfunction of platelet aggregation. Yiqi Huoxue Fomula is effective for HT, but the mechanism remains unclear.Previously, we found that the expression of platelet membrane glycoprotein (GP) Ⅰb-Ⅸ-Ⅴ and its subunit GPⅠbα, which serve as a co-receptor of von willebr and factor and thrombin, is downregulated. The main reason is that abnormal regulation of ADAM17, leading to over hydrolysis of GPIbαand GPⅤ in the extracellular segment, thus resulting in platelet aggregation dysfunction in HT. Present research shows that iRhom2, which serve as the upstream molecular chaperone of the ADAM17, is the key gene for ADAM17 translocation and protein hydrolysis. The first iRhom2 directly binds with pro-ADAM17 in the endoplasmic reticulum, and then is modified into the mature ADAM17 in the Golgi, finally the mature ADAM17 is localized to cell membrane, and performs the function of protein hydrolysis. Therefore, we attempt to observe the expression of iRhom2 gene in HT patients, the its specific role in transport and hydrolysis activity of ADAM17, and the influence of Yiqi Huoxue Formula by using western blotting, confocal laser scanning microscopy, flow cytometry, Real time-PCR，ELISA, etc, in order to further elucidate the pathogenesis of HT and the mechanism of therapeutic effect of Yiqi Huoxue Fomula. We are sure that the result of this research subject will be of significant theoretical and practical importance.