Department of Cardiology, St George Hospital, Sydney, Australia; Department of Medicine, University of NSW, Sydney, Australia.
Department of Medicine, University of NSW, Sydney, Australia; Department of Infectious Diseases, Immunology and Sexual Health, St George Hospital, Sydney, Australia.
Free Radic Biol Med. 2018 Sep;125:98-103. doi: 10.1016/j.freeradbiomed.2018.03.048. Epub 2018 Mar 29.
The anti-phospholipid syndrome (APS) is a prothrombotic autoimmune disorder characterized by either thrombosis or pregnancy complications in the setting of persistent anti-phospholipid antibodies (aPL). βeta 2-glycoprotein I (β2-GPI) is the major autoantigen in APS that binds anionic phospholipids as well as specific receptors on platelets and endothelial cells resulting in activation of prothrombotic pathways. β2-GPI consists of 5 Domains that exist in a circular or linear form, with the latter occurring after binding to anionic phospholipids. β2-GPI also undergoes dynamic posttranslational modification between oxidized and free thiol forms. The relationship between posttranslational modification and structural conformation is yet to be definitively clarified. Compared with controls, patients with the APS have higher levels of total β2-GPI and lower levels of free thiol β2-GPI. This raises the possibility of using quantification of β2-GPI posttranslational modification as a redox biomarker in the management and diagnosis of the APS.
抗磷脂综合征（APS）是一种以持续存在抗磷脂抗体（aPL）为特征的促血栓形成自身免疫性疾病，表现为血栓形成或妊娠并发症。β2-糖蛋白 I（β2-GPI）是 APS 中的主要自身抗原，它与阴离子磷脂以及血小板和内皮细胞上的特定受体结合，导致促血栓形成途径的激活。β2-GPI 由 5 个结构域组成，以环形或线性形式存在，后者发生在与阴离子磷脂结合之后。β2-GPI 还在氧化和游离巯基形式之间进行动态的翻译后修饰。翻译后修饰和结构构象之间的关系尚未得到明确阐明。与对照组相比，APS 患者的总β2-GPI 水平较高，游离巯基β2-GPI 水平较低。这提出了使用β2-GPI 翻译后修饰的定量作为 APS 管理和诊断的氧化还原生物标志物的可能性。
