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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Beta-2 glycoprotein I and its role in antiphospholipid syndrome-lessons from knockout mice.
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Beta-2 glycoprotein I and its role in antiphospholipid syndrome-lessons from knockout mice.

机译：Beta 2糖蛋白I及其在敲除小鼠抗磷脂综合征中的作用。

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摘要

The antiphospholipid syndrome is characterized by the presence in serum of autoantibodies against beta(2)GPI. Although the role of beta(2)GPI in the pathogenesis of antiphospholipid antibody syndrome (APS) is well recognized, its exact physiological functions still remain undisclosed. Several interactions of beta(2)GPI with components of the coagulation cascade have been proposed, resulting in both procoagulant and anticoagulant effects. Additionally, beta(2)GPI has been implicated in the mechanism of recurrent fetal loss entailed in APS. Recently, using a homologous recombination approach, reproduction of mice homozygous for deletion of the beta(2)GPI gene has been feasible. beta(2)GPI knockout mice offer a valuable tool for revealing the physiological role of the protein. These mice show decreased in vitro ability for thrombin generation. Furthermore, although mice lacking beta(2)GPI are fertile, the success of early pregnancy is moderately compromised and functional beta(2)GPI is believed necessary for optimal implantation and placental morphogenesis.
机译：抗磷脂综合征的特征是血清中存在针对β（2）GPI的自身抗体。尽管人们公认β（2）GPI在抗磷脂抗体综合征（APS）发病机理中的作用，但其确切的生理功能仍未公开。已经提出了β（2）GPI与凝血级联组分的几种相互作用，从而导致促凝作用和抗凝作用。此外，beta（2）GPI已牵涉到APS所致的反复胎儿流失的机制。最近，使用同源重组方法，纯合小鼠的β（2）GPI基因缺失的复制是可行的。 beta（2）GPI基因敲除小鼠提供了揭示该蛋白质生理作用的有价值的工具。这些小鼠显示出降低的凝血酶生成能力。此外，尽管缺乏beta（2）GPI的小鼠可育，但早期妊娠的成功受到中等程度的损害，功能性beta（2）GPI被认为是最佳植入和胎盘形态发生所必需的。

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