β2-糖蛋白I，抗磷脂综合征的关键因素。

beta2-glycoprotein I, the playmaker of the antiphospholipid syndrome.

作者信息

Bas de Laat H, Derksen Ronald H W M, de Groot Philip G

机构信息

Department of Haematology, Thrombosis and Haemostasis Laboratory, University Medical Center Utrecht, The Netherlands.

出版信息

Clin Immunol. 2004 Aug;112(2):161-8. doi: 10.1016/j.clim.2004.02.012.

DOI:10.1016/j.clim.2004.02.012

PMID:15240159

Abstract

From its discovery in the early 60s till the beginning of the 90s, there was not much interest in plasma protein beta2-glycoprotein I (beta2-GPI). The finding that beta2-GPI acts as an essential cofactor for the detection of antiphospholipid antibodies (aPL) tremendously increased the interest in beta2-GPI [Lancet 335 (1990) 1544; Lancet 336 (1990) 177; Proc. Natl. Acad. Sci. U. S. A. 87 (1990) 4120]. It is now generally accepted that autoantibodies directed towards beta2-GPI are not only a serological marker but that they are involved in the pathology of the antiphospholipid syndrome (APS). In this review, we will first discuss the biochemistry of the protein beta2-GPI and the influence that the antibodies have on the function of beta2-GPI. Next, we will discuss the problems that are faced when assays to detect the presence of the autoantibodies are performed, emphasizing the urgent need for standardization of the anti-beta2-GPI-ELISA. Finally, we will discuss our latest insights into beta2-GPI and its role in the pathology of APS. Thereby, we will focus on the role of dimerized beta2-GPI on platelet and endothelial cell function.

摘要

从20世纪60年代初发现到90年代初，人们对血浆蛋白β2-糖蛋白I（β2-GPI）的兴趣不大。β2-GPI作为检测抗磷脂抗体（aPL）的必需辅助因子这一发现极大地增加了人们对β2-GPI的兴趣[《柳叶刀》335卷（1990年）第1544页；《柳叶刀》336卷（1990年）第177页；《美国国家科学院院刊》87卷（1990年）第4120页]。现在人们普遍认为，针对β2-GPI的自身抗体不仅是一种血清学标志物，而且它们参与了抗磷脂综合征（APS）的病理过程。在这篇综述中，我们将首先讨论蛋白质β2-GPI的生物化学以及抗体对β2-GPI功能的影响。接下来，我们将讨论在进行检测自身抗体存在的检测时所面临的问题，强调对抗β2-GPI-ELISA进行标准化的迫切需求。最后，我们将讨论我们对β2-GPI及其在APS病理过程中作用的最新见解。因此，我们将重点关注二聚化β2-GPI对血小板和内皮细胞功能的作用。