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系统性红斑狼疮中B淋巴细胞的选择与存活

B lymphocyte selection and survival in systemic lupus.

作者信息

Liossis Stamatis-Nick C, Zouali Moncef

机构信息

Institut National de Santé et de Recherche Médicale U 430, Immunopathologie Humaine, Paris, France.

出版信息

Int Arch Allergy Immunol. 2004 Jan;133(1):72-83. doi: 10.1159/000076130. Epub 2004 Jan 12.

Abstract

B lymphocytes are an essential element in the body's immune system. Engagement of the B cell receptor is responsible for initiating the signaling events that can activate, inactivate or physically eliminate B cells, depending on the magnitude and duration of the signal. Control of B cell signaling occurs through both positive and negative regulation, as well as through the actions of molecular scaffolds that contribute to the formation of signaling complexes. Inactivation of genes encoding signaling molecules was shown to result in clinical manifestations reminiscent of systemic autoimmunity in experimental animals. Aberrant expression of some signaling molecules was also observed in patients with systemic autoimmune diseases. Understanding the mechanisms that subvert B cell receptor transduction pathways is likely to aid in the development of therapeutic agents to treat autoimmune diseases.

摘要

B淋巴细胞是机体免疫系统的重要组成部分。B细胞受体的激活负责启动信号传导事件,根据信号的强度和持续时间,这些事件可激活、失活或物理性清除B细胞。B细胞信号传导的控制通过正调控和负调控以及有助于信号复合物形成的分子支架的作用来实现。实验动物中,编码信号分子的基因失活会导致类似于系统性自身免疫的临床表现。系统性自身免疫疾病患者也观察到一些信号分子的异常表达。了解破坏B细胞受体转导途径的机制可能有助于开发治疗自身免疫疾病的治疗药物。

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