血小板表面CD47介导的ITP自身免疫机制研究

免疫性血小板减少症(ITP)是一种由于自身免疫异常所致的血小板生成、破坏加速的出血性疾病。但是具体的发病机制尚未完全阐明。我们前期研究结果表明血小板表面CD47存在明显异常表达，并且与疾病进程相关，提示CD47可能在ITP的自身免疫机制中发挥作用，但是具体的机制及作用尚不明确。因此本项目拟通过针对CD47基因的慢病毒载体和SIRPα-Ig 融合蛋白作为诱饵受体阻断 CD47/SIRP-α系统后观察对ITP自身免疫T、B细胞增殖和凋亡的影响，抗CD47单克隆抗体对ITP患者Th分化/激活;细胞毒性作用;巨噬细胞吞噬功能的影响及其机制，以进一步明确CD47在ITP自身免疫调控中的具体作用和地位，为ITP的免疫异常机制提供一个新的思路和发展新的治疗措施提供理论基础。

Immune thrombocytopenia (ITP) is a bleeding disorder that accelerates platelet production and destruction due to autoimmune abnormalities. But the specific pathogenesis has not been fully elucidated. Our previous research results showed that there was obvious abnormal expression of CD47 on the platelet surface, and it was related to the disease process, suggesting that CD47 may play a role in the autoimmune mechanism of ITP, but the specific mechanism and role are not yet clear. Therefore, this project intends to observe the effect on the proliferation and apoptosis of ITP autoimmune T and B cells after blocking the CD47/SIRP-α system by using the lentiviral vector targeting CD47 gene and SIRPα-Ig fusion protein as a bait receptor. Cloning antibodies on Th differentiation / activation in ITP patients; cytotoxicity; the effect and mechanism of macrophage phagocytosis to further clarify the specific role and position of CD47 in ITP autoimmune regulation and provide a new mechanism for ITP immune abnormalities The idea and development of new treatment measures provide a theoretical basis.