Botto Marina, Walport Mark J
Division of Medicine, Faculty of Medicine, Imperial College, London, UK.
Immunobiology. 2002 Sep;205(4-5):395-406. doi: 10.1078/0171-2985-00141.
Deficiency of classical pathway complement components displays a hierarchical association with the development of systemic lupus erythematosus (SLE). Individuals with deficiency of C1q, the first component of the classical pathway of activation, have the highest prevalence of SLE and the most severe manifestations of the disease. However, complement is also implicated in the effector inflammatory phase of the autoimmune response that characterizes SLE. Complement proteins are deposited in inflamed tissues causing consumption of complement. In addition, autoantibodies to C1q develop as part of the autoantibody response. Understanding how C1q deficiency results in the autoimmune phenotype of SLE may provide valuable clues to the role of the complement system in the maintenance of immune tolerance. In this review firstly we discuss the relationship between C1q deficiency and/or consumption and lupus. Secondly, we consider the links between apoptosis and complement. Finally we review the lessons we have learned from a murine model of C1q deficiency discussing the experimental evidence in support of the hypothesis that C1q may critically influence the immune response to self-antigens contained within the surface blebs generated by apoptotic cells.
经典途径补体成分的缺乏与系统性红斑狼疮（SLE）的发生呈现出一种层级关联。具有经典激活途径第一成分C1q缺乏的个体，SLE患病率最高且疾病表现最为严重。然而，补体也参与了SLE所特有的自身免疫反应的效应炎症阶段。补体蛋白沉积于炎症组织导致补体消耗。此外，针对C1q的自身抗体作为自身抗体反应的一部分而产生。了解C1q缺乏如何导致SLE的自身免疫表型，可能为补体系统在维持免疫耐受中的作用提供有价值的线索。在这篇综述中，我们首先讨论C1q缺乏和/或消耗与狼疮之间的关系。其次，我们思考细胞凋亡与补体之间的联系。最后，我们回顾从C1q缺乏的小鼠模型中获得的经验教训，讨论支持C1q可能对凋亡细胞产生的表面小泡中所含自身抗原的免疫反应产生关键影响这一假说的实验证据。
