炎症小体相关自身炎症性疾病研究进展OA北大核心CSTPCD
Research progress of inflammasome-associated autoinflammatory diseases
炎症小体是细胞内一种大分子多蛋白复合物,通过激活调节半胱氨酸天冬氨酸特异蛋白酶-1(caspase-1,CASP1)的活化,促进白介素(IL)-1β、IL-18分泌,引起机体炎症反应.近年来,对不同炎症小体的分子组成和激活机制进行了广泛研究,已对多种炎症小体进行了分子表征,其中4种炎症小体的自激活突变可导致人类单基因自身炎症性疾病.文章主要对NLRP1、CARD8、NLRP3、NLRC4和Pyrin炎症小体相关自身炎症性疾病的致病机制及临床表现、治疗研究进展进行综述.
The inflammasome is a macromolecular multi-protein complex in cells,and its activation can regulate the activation of cysteine aspartic protease-1(CASP1),promote the activation and secretion of IL-1β and IL-18,and cause inflammation in the body.In recent years,researchers have conducted extensive studies on the molecular composition and activation mechanisms of different inflammasomes,and a variety of inflammasome molecules have been characterized,among which the self-activating mutations of four inflamm-asome can lead to single-gene autoinflammatory diseases in humans.This review focuses on the research progress of the pathogenesis,clinical manifestations and treatment of NLRP1,CARD8,NLRP3,NLRC4 and Pyrin inflammasome-related autoinflammatory diseases.
吴俊峰;唐雪梅
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 儿童感染与免疫罕见病重庆市重点实验室,重庆 400014
临床医学
自身炎症性疾病炎症小体炎症小体病
autoinflammatory diseasesinflammasomeinfla-mmasomapathies
《中国实用儿科杂志》 2024 (006)
432-438 / 7
国家重点研发计划(2021YFC2702003);重庆市教委科学技术研究项目(KJQN202200418);国家儿童健康与疾病临床医学研究中心临床医学研究一般项目(NCRCCHD-2022-YP-07)
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