炎症小体相关自身炎症性疾病研究进展

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 multiprotein complex in cells,and its activation can regulate the activation of cysteine aspartic protease-1(CASP1),promote the activation and secretion of IL-1βand 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 inflammasome 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 inflammasomerelated autoinflammatory diseases.