Progress on the study of NLRP3 inflammasome in autoinflammatory diseases of children.

Author: Yandie LI ¹ ; Meiping LU ²
Author Information

1. Department of Rheumatology Immunology & Allergy, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
2. Department of Rheumatology Immunology & Allergy, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China. meipinglu@zju.edu.cn.
Publication Type:Journal Article
From: Journal of Zhejiang University. Medical sciences 2017;46(4):449-453
CountryChina
Language:Chinese
Abstract: Autoinflammatory diseases (AID) in childhood is one of refractory diseases, whose pathogenesis is not completely clear. In recent years, a large number of studies have shown that NLRP3 inflammasome plays an important role in the development of AIDs in children. Inflammasome is a cytosolic multiprotein complex that can activate cysteinyl aspartate-specific protease-1 (caspase-1), to further promote the maturation and secretion of proinflammatory cytokines IL-1β and IL-18 as well as pyroptosis and regulate innate immune response. IL-1 receptor antagonist (Anakinra) and IL-1β monoclonal antibody (Canakinumab) have good therapeutic effects in children with AIDs. This article reviews the research progress of NLRP3 inflammasome in the pathogenesis of autoinflammatory diseases.