JAK-STAT信号过度活化连接免疫出生缺陷与自身免疫病
The Hyperactivation of JAK-STAT Signaling Connects Inborn Errors of Immunity and Autoimmune Diseases
蒋仪 1安云飞 1赵晓东1
作者信息
- 1. 重庆医科大学附属儿童医院风湿免疫科国家儿童健康与疾病临床医学研究中心儿童发育疾病研究教育部重点实验室儿童发育重大疾病国家国际科技合作基地儿童感染与免疫罕见病重庆市重点实验室,重庆 400014
- 折叠
摘要
JAK-STAT信号通路是重要的细胞因子信号转导通路,该通路的许多单基因功能缺失性或者功能获得性突变可导致免疫出生缺陷(IEI).自身免疫病由多基因多因素导致,病因复杂多样,JAK-STAT信号过度激活在该类疾病中发挥重要作用.本文总结阐述了 JAK-STAT通路的作用机制及其在IEI和自身免疫病的发生、发展中的作用,提示JAK-STAT通路的过度活化可将IEI和自身免疫病联系起来,使得IEI成为研究自身免疫病发病机制的良好模型,为自身免疫病的治疗提供新思路与方向.
Abstract
JAK-STAT signaling pathway is an important cytokine signaling transduction pathway.Many monogenic loss-of-function or gain-of-function mutations in this pathway can cause inborn errors of immunity(IEI).Autoimmune diseases are caused by polygenic and different factors.The etiology of the disease is com-plex and diverse.The over-activation of JAK-STAT signaling pathway plays a significant role in the pathogenesis of autoimmune diseases.In this article,we summarize the mechanism of the JAK-STAT pathway and its role in the occurrence and development of IEI and autoimmune diseases,suggesting that the hyperactivation of JAK-STAT pathway having connection with IEI and autoimmune diseases.Meanwhile,we hope to make IEI a good model to study the pathogenesis of autoimmune diseases and to provide new ideas and directions for the clinical treatment of autoimmune diseases.
关键词
JAK-STAT信号通路/免疫出生缺陷/自身免疫病Key words
JAK-STAT signaling pathway/inborn errors of immunity/autoimmune diseases引用本文复制引用
出版年
2024
NETL
NSTL
万方数据