Hiromi Kubagawa, Pedram Mahmoudi Aliabadi, Khlowd Al-Qaisi, Peter K Jani, Kazuhito Honjo, Shozo Izui, Andreas Radbruch, Fritz Melchers
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引用次数: 0
摘要
与切换免疫球蛋白(Ig)异型的 Fc 受体不同,IgM 的 Fc 受体(FcµR)由淋巴细胞选择性表达。小鼠 FcµR 基因的消减会损害 B 细胞耐受性,同时产生 IgM 和 IgG 同型的自身抗体就是证明。在这篇文章中,我们重申了在突变小鼠中观察到的自身免疫表型,即 IgM 稳态、包括自身抗体在内的体液免疫反应失调和莫特细胞形成。我们还提出了 FCMR 缺乏症患者的潜在表型以及 FcµR 介导的自我反应 B 细胞调控模型。
Functions of IgM fc receptor (FcµR) related to autoimmunity.
Unlike Fc receptors for switched immunoglobulin (Ig) isotypes, Fc receptor for IgM (FcµR) is selectively expressed by lymphocytes. The ablation of the FcµR gene in mice impairs B cell tolerance as evidenced by concomitant production of autoantibodies of IgM and IgG isotypes. In this essay, we reiterate the autoimmune phenotypes observed in mutant mice, ie IgM homeostasis, dysregulated humoral immune responses including autoantibodies, and Mott cell formation. We also propose the potential phenotypes in individuals with FCMR deficiency and the model for FcµR-mediated regulation of self-reactive B cells.
期刊介绍:
Autoimmunity is an international, peer reviewed journal that publishes articles on cell and molecular immunology, immunogenetics, molecular biology and autoimmunity. Current understanding of immunity and autoimmunity is being furthered by the progress in new molecular sciences that has recently been little short of spectacular. In addition to the basic elements and mechanisms of the immune system, Autoimmunity is interested in the cellular and molecular processes associated with systemic lupus erythematosus, rheumatoid arthritis, Sjogren syndrome, type I diabetes, multiple sclerosis and other systemic and organ-specific autoimmune disorders. The journal reflects the immunology areas where scientific progress is most rapid. It is a valuable tool to basic and translational researchers in cell biology, genetics and molecular biology of immunity and autoimmunity.