免疫相关放线菌病处于免疫缺陷、自身免疫和自身炎症的交叉路口

IF 60.9 1区 医学 Q1 IMMUNOLOGY
Loïc Dupré, Irinka Castanon, Kaan Boztug
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引用次数: 0

摘要

肌动蛋白细胞骨架重塑驱动免疫细胞的迁移及其参与动态细胞-细胞接触。肌动蛋白细胞骨架动力学在免疫细胞功能中的重要性通过发现由单个肌动蛋白调节蛋白缺陷引起的先天性免疫错误(IEIs)而得到强调,从而导致免疫相关的放线蛋白病。除了对感染的易感性外,这些通常表现为大量的自身免疫和自身炎症表现。在这里,我们回顾了肌动蛋白子网络在淋巴细胞和髓细胞的激活和功能中的作用。我们关注肌动蛋白缺陷导致淋巴细胞功能异常的机制,包括T细胞和B细胞介导的耐受性失调和细胞因子产生偏倚,这可能导致自身免疫。我们还强调了肌动蛋白缺陷和炎性小体活化之间的关系,以及骨髓细胞中作为自身炎症的潜在原因的其他病理机制。最后,我们讨论了基于对免疫相关放线菌病的分子理解的未来研究和治疗干预途径。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Immune-related actinopathies at the cross-road of immunodeficiency, autoimmunity and autoinflammation

Immune-related actinopathies at the cross-road of immunodeficiency, autoimmunity and autoinflammation

Actin cytoskeleton remodelling drives the migration of immune cells and their engagement in dynamic cell–cell contacts. The importance of actin cytoskeleton dynamics in immune cell function is highlighted by the discovery of inborn errors of immunity (IEIs) that are caused by defects in individual actin-regulatory proteins, resulting in immune-related actinopathies. In addition to susceptibility to infection, these often present with a vast array of autoimmune and autoinflammatory manifestations. Here, we review the role of actin subnetworks in the activation and function of lymphoid and myeloid cells. We focus on the mechanisms by which actin defects result in aberrant lymphocyte function, including dysregulation of T cell- and B cell-mediated tolerance and biased cytokine production, which can result in autoimmunity. We also highlight the relationship between actin defects and inflammasome activation and other pathomechanisms in myeloid cells as the underlying cause of autoinflammation. Finally, we discuss future avenues for research and therapeutic intervention based on a molecular understanding of immune-related actinopathies.

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来源期刊
Nature Reviews Immunology
Nature Reviews Immunology 医学-免疫学
CiteScore
93.40
自引率
0.40%
发文量
131
审稿时长
6-12 weeks
期刊介绍: Nature Reviews Immunology is a journal that provides comprehensive coverage of all areas of immunology, including fundamental mechanisms and applied aspects. It has two international standard serial numbers (ISSN): 1474-1733 for print and 1474-1741 for online. In addition to review articles, the journal also features recent developments and new primary papers in the field, as well as reflections on influential people, papers, and events in the development of immunology. The subjects covered by Nature Reviews Immunology include allergy and asthma, autoimmunity, antigen processing and presentation, apoptosis and cell death, chemokines and chemokine receptors, cytokines and cytokine receptors, development and function of cells of the immune system, haematopoiesis, infection and immunity, immunotherapy, innate immunity, mucosal immunology and the microbiota, regulation of the immune response, signalling in the immune system, transplantation, tumour immunology and immunotherapy, and vaccine development.
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