自体免疫调节器 (AIRE) 基因,主激活器的自我抗原表达在腺体
Matthieu Giraud1, Pärt Peterson2
1INSERM, Nantes Université, Center for Research in Transplantation and Translational Immunology, UMR 1064, Nantes, France. matthieu.giraud@inserm.fr.
Advances in experimental medicine and biology
|March 11, 2025
概括
20多年前发现的AIRE基因对免疫耐受性至关重要. 突变会导致严重的自身免疫性疾病,因为它会破坏胸腺消除自我反应T细胞的能力.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
- 这是一种自身免疫力.
背景情况:
- 自免疫调节器 (AIRE) 基因在20多年前被发现.
- AIRE基因的突变导致一种罕见的,严重的自身免疫性疾病,影响多个器官.
- 了解AIRE的功能对于中央免疫耐受性至关重要.
研究的目的:
- 总结关于AIRE基因的作用和作用机制的关键见解.
- 突出AIRE在建立中央免疫耐受性的作用.
- 为了强调AIRE在预防自身免疫性方面的重要性.
主要方法:
- 审查自1997年AIRE基因鉴定以来出版的科学文献.
- 对研究AIRE分子功能的研究进行分析.
- 检查AIRE突变的免疫后果.
主要成果:
- AIRE在甲状腺中诱导成千上万种组织特异性自我抗原的表达.
- 这些自我抗原被呈现给正在发育的T细胞.
- 这一过程导致了自我反应性T细胞的消除和调节性T细胞的产生.
结论:
- 艾瑞 (AIRE) 作为建立和维持中央免疫耐受性的主调节器.
- AIRE的功能对于防止免疫系统攻击身体自身组织至关重要.
- AIRE的失调是严重的自身免疫性疾病的基础.
相关概念视频
Master Transcription Regulators
6.8K
Master transcription regulators are regulatory proteins that are predominantly responsible for regulating the expression of multiple genes. Often these genes work in concert to drive a complex process. Activation of a master transcription regulator can lead to a cascade of transcriptional activation necessary for that outcome. These regulators can directly bind to the regulatory sequences of the various genes involved, or they can indirectly regulate transcription by binding to regulatory...
6.8K
Diversity of Antigen Receptors
478
Antigen receptors are essential components of the immune system crucial in defending the body against foreign invaders. These receptors are present on the surface of B and T cells, enabling them to recognize antigens and mount an appropriate immune response.
Before encountering any antigen, lymphocytes express these receptors. On B cells, the antigen receptor is a membrane-bound antibody molecule called BCR; on T cells, it is a T cell receptor or TCR. B and T cell receptors are composed of two...
Before encountering any antigen, lymphocytes express these receptors. On B cells, the antigen receptor is a membrane-bound antibody molecule called BCR; on T cells, it is a T cell receptor or TCR. B and T cell receptors are composed of two...
478
Antigens Involved in Adaptive Immunity
434
An antigen is any substance the immune system identifies as foreign and potentially harmful to the body, prompting an immune response. Antigens have two functional properties: immunogenicity and reactivity. Immunogenicity is the ability of an antigen to stimulate a specific immune response. At the same time, reactivity describes the antigen's ability to react with the cells and antibodies produced in response to it.
Complete Antigens
Complete antigens possess both immunogenicity and...
Complete Antigens
Complete antigens possess both immunogenicity and...
434
T Cell Activation and Clonal Selection
622
T cells are integral to our adaptive immune system, recognizing and effectively responding to foreign antigens. T cell activation and clonal selection are pivotal in orchestrating this immune response. This article elucidates these mechanisms, detailing the roles of cluster of differentiation (CD) markers, major histocompatibility complex (MHC) molecules, costimulatory signals, and the process of clonal selection.
Naive T cells that have not yet encountered an antigen express two primary CD...
Naive T cells that have not yet encountered an antigen express two primary CD...
622
RNA Polymerase II Accessory Proteins
9.1K
Proteins that regulate transcription can do so either via direct contact with RNA Polymerase or through indirect interactions facilitated by adaptors, mediators, histone-modifying proteins, and nucleosome remodelers. Direct interactions to activate transcription is seen in bacteria as well as in some eukaryotic genes. In these cases, upstream activation sequences are adjacent to the promoters, and the activator proteins interact directly with the transcriptional machinery. For example, in...
9.1K
B Cell Activation and Differentiation
1.4K
The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
1.4K


