Abstract：
Beta 2 glycoprotein I (β2GPI) is the major autoantigen in the antiphospholipid syndrome, an autoimmune disorder characterized by thrombotic and obstetric complications. The autoantibodies that target beta 2 glycoprotein I are pathogenic and contribute to disease pathogenesis. The β2GPI molecule is composed of 5 domains that are numbered 1 through to 5. Autoantibodies bind mainly to domain 1 whereas the majority of the biological functions of the β2GPI molecule in diverse processes such as apoptotic cell clearance, complement regulation, lipopolysaccharide clearance and anticoagulation have been localised to domain 5 and its unique biochemistry, reviewed in this article. The role of purified domain 5 peptide as a potential therapeutic agent in APS and ischemia reperfusion injury is discussed.
摘要：
β2糖蛋白I（B2GPI）是抗磷脂综合征中主要的自身抗原，以血栓性和产科并发症为特征的自身免疫性疾病。靶向β2糖蛋白I的自身抗体是致病性的，并有助于疾病的发病机理。B2GPI分子由编号为1至5的5个结构域组成。自身抗体主要与结构域1结合，而B2GPI分子的大多数生物学功能在不同的过程中，如凋亡细胞清除，补体调节，脂多糖清除，抗凝作用已被定位在第5域及其独特的生物化学领域，本文对此进行了综述。讨论了纯化的结构域5肽作为APS和缺血再灌注损伤的潜在治疗剂的作用。
