Department of Clinical Chemistry and Haematology, University Medical Center, Utrecht, The Netherlands.
Int J Lab Hematol. 2012 Jun;34(3):223-31. doi: 10.1111/j.1751-553X.2012.01414.x. Epub 2012 Mar 6.
The antiphospholipid syndrome is an autoimmune disease that manifests clinically as recurrent thrombotic complications or foetal losses and serologically with elevated levels of antiphospholipid antibodies in the plasmas of these patients. The term 'antiphospholipid syndrome' is confusing, because the auto-antibodies are not directed against phospholipids but towards a plasma protein, β(2) -glycoprotein I. For many years, the reason why auto-antibodies against β(2) -glycoprotein I were pro-thrombotic was unclear, because β(2) -glycoprotein I seems to be an obsolete protein in our circulation. Human and mice deficient in this protein do not express a clear phenotype. Recent studies on the structure and function of β(2) -glycoprotein I have provided novel insights into the importance of this protein in physiology and its role in the pathology of the antiphospholipid syndrome.
抗磷脂综合征是一种自身免疫性疾病，临床上表现为复发性血栓并发症或胎儿丢失，血清学上表现为这些患者血浆中抗磷脂抗体水平升高。“抗磷脂综合征”一词令人困惑，因为自身抗体不是针对磷脂，而是针对一种血浆蛋白，即β(2)-糖蛋白 I。多年来，针对β(2)-糖蛋白 I 的自身抗体为何具有促血栓形成作用尚不清楚，因为β(2)-糖蛋白 I 似乎是我们循环中的一种废弃蛋白。缺乏这种蛋白质的人和老鼠并没有表现出明显的表型。最近对β(2)-糖蛋白 I 的结构和功能的研究为该蛋白在生理学中的重要性及其在抗磷脂综合征病理中的作用提供了新的见解。
