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人类和动物的补体缺陷:与自身免疫的联系。

Complement deficiencies in humans and animals: links to autoimmunity.

作者信息

Lewis M J, Botto M

机构信息

Division of Medicine, Faculty of Medicine, Rheumatology Section, Imperial College, Hammersmith Campus, Du Cane Road, London, W12 0NN, UK.

出版信息

Autoimmunity. 2006 Aug;39(5):367-78. doi: 10.1080/08916930600739233.

DOI:10.1080/08916930600739233
PMID:16923536
Abstract

Complement is involved in the pathogenesis of systemic lupus erythematosus (SLE) in multiple ways and may act as both friend and foe. Inherited homozygous deficiency of one of the earliest components of the classical pathway is strongly associated with susceptibility to the development of SLE. However, complement is also implicated in the effector inflammatory phase of the autoimmune response that characterizes the disease. A further paradox in the links between complement and SLE is the observation that autoantibodies to some complement proteins, especially to C1q, develop as part of the autoantibody response. In this chapter, the role of the complement system in SLE is reviewed and hypotheses advanced to explain the complex relationships between complement and lupus.

摘要

补体通过多种方式参与系统性红斑狼疮(SLE)的发病机制,其作用可谓亦敌亦友。经典途径中最早成分之一的遗传性纯合缺陷与SLE发病易感性密切相关。然而,补体也参与了该疾病特征性自身免疫反应的效应炎症阶段。补体与SLE之间联系的另一个矛盾之处在于,观察到针对某些补体蛋白,尤其是C1q的自身抗体,会作为自身抗体反应的一部分而产生。在本章中,我们将综述补体系统在SLE中的作用,并提出一些假说以解释补体与狼疮之间的复杂关系。

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1
Complement deficiencies in humans and animals: links to autoimmunity.人类和动物的补体缺陷:与自身免疫的联系。
Autoimmunity. 2006 Aug;39(5):367-78. doi: 10.1080/08916930600739233.
2
C1q, autoimmunity and apoptosis.C1q、自身免疫与细胞凋亡。
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Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.疾病机制:补体系统与系统性红斑狼疮的发病机制
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Complement deficiencies and systemic lupus erythematosus.补体缺陷与系统性红斑狼疮
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Complement and systemic lupus erythematosus.补体与系统性红斑狼疮
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The association between systemic lupus erythematosus and deficiencies of the complement system.系统性红斑狼疮与补体系统缺陷之间的关联。
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C1q and systemic lupus erythematosus.补体C1q与系统性红斑狼疮
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Complement deficiency and systemic lupus erythematosus: consensus and dilemma.补体缺陷与系统性红斑狼疮:共识与困境。
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