β2-糖蛋白Ⅰ 及其抗体复合物在APS发生发展及临床诊断治疗中的作用

抗磷脂综合征（antiphospholipid syndrome, APS）的临床特征为动静脉血栓形成或习惯性流产。血清学特征为抗磷脂抗体（antiphospholipid antibody, aPL Ab）持续阳性等。目前，对APS治疗的主要方法为针对血小板的抗凝血治疗。然而，治疗过程中抗凝强度和持续时间难以把握，且对中风等重度患者疗效甚微。β2糖蛋白Ⅰ（β2 glycoprotein I, β2-GPI）是一种可与阴性磷脂结合的血浆糖蛋白，由5个结构域组成。第5结构域在与氧化低密度脂蛋白（oxidized low-density lipoprotein, oxLDL）等阴性磷脂结合后，改变自身构象并暴露位于第1结构域的抗原位点，随后被自身抗体特异性识别形成三元抗体复合物，激活血栓形成相关信号通路，促进APS的发生发展。因此，深入研究β2-GPI的分子结构及其在APS发生发展中的作用具有重要意义。本文对β2-GPI的结构，β2-GPI抗体复合物的形成过程，及其在APS发生发展和治疗诊断中的作用进行了阐述。最后，以β2-GPI为基础对APS的诊断和治疗方向进行了展望，旨在为β2-GPI作为APS精准治疗靶点药物的研发提供一定的参考。

The Role of β2-GPI and Its Antibody Complex in the Occurrence,Development, Clinical Diagnosis and Treatment of APS

The clinical features of antiphospholipid syndrome (APS) are venous or arterial thrombosis and a higher incidence of pregnancy, serologically characterized by a persistent positive antiphospholipid antibody (aPLAb). Currently, the main methods of treatment for APS are antiplatelets and anticoagulants. However, the anticoagulation intensity and duration during treatment are difficult to grasp and have little effect on severe diseases such as stroke. β2-glycoprotein I (β2-GPI) is a plasma glycoprotein that binds to negative phospholipids and it consists of five domains. When the fifth domain binds to a negative phospholipid such as oxLDL, its conformation is altered and the antigenic site located in the first domain is exposed. Subsequently, it is specifically recognized by autoantibodies to form a ternary antibody complex and activation of thrombosis-related signaling pathways, promoting the development of APS. This paper describes the structure of β2-GPI and its antibody complex formation process, and the role of antibody complexes in the development and therapeutic diagnosis of APS. Finally, based on β2-GPI, the diagnosis and treatment direction of APS are reviewed. The purpose of this study is to provide a reference for the research and development of β2-GPI as a precise therapeutic drug for APS.