A critical role for complement in maintenance of self-tolerance
A critical role for complement in maintenance of self-tolerance
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DOI：
10.1016/s1074-7613(00)80669-x
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发表时间：
1998-11-01
期刊：
影响因子：
32.4
通讯作者：
Carroll, MC
中科院分区：
文献类型：
--
作者：
Prodeus, AP;Goerg, S;Carroll, MC
补体在维持自身耐受中的作用已在两个模型中得到验证：免疫球蛋白转基因外周耐受模型和狼疮样小鼠CD95(Fas)缺乏模型。我们发现，自身反应性B淋巴细胞缺乏补体受体CD21/CD35或转移到缺乏补体蛋白C4的小鼠体内，不能被可溶性自身抗原激活。在第二种模式中，CD21/CD35或C4缺陷合并CD95缺陷会导致高滴度的抗核抗体，导致严重的狼疮样疾病。这些发现提示补体系统在B细胞耐受中的新作用，并为补体缺乏与系统性红斑狼疮易感性的遗传关联提供了洞察力。
The role of complement in the maintenance of self-tolerance has been examined in two models: an immunoglobulin transgenic model of peripheral tolerance and a lupus-like murine model of CD95 (Fas) deficiency. We find that self-reactive B lymphocytes deficient in complement receptors CD21/CD35 or transferred into mice deficient in the complement protein C4 are not anergized by soluble self-antigen. In the second model, deficiency in CD21/CD35 or C4 combined with CD95 deficiency results in high titers of anti-nuclear antibodies leading to severe lupus-like disease. These findings suggest a novel role for the complement system in B cell tolerance and provide insight into the genetic association of complement deficiency with susceptibility to systemic lupus erythematosus.