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Journal of ZheJiang University(Medical Science)  2017, Vol. 46 Issue (4): 449-453    DOI: 10.3785/j.issn.1008-9292.2017.08.17
    
Progress on the study of NLRP3 inflammasome in autoinflammatory diseases of children
LI Yandie, LU Meiping
Department of Rheumatology Immunology & Allergy, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China
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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.



Key wordsMultiprotein complexes      Autoimmune diseases      Interleukin-1 beta      Chromosome disorders      Review     
Received: 28 April 2017      Published: 25 August 2017
CLC:  R725.9  
Cite this article:

LI Yandie, LU Meiping. Progress on the study of NLRP3 inflammasome in autoinflammatory diseases of children. Journal of ZheJiang University(Medical Science), 2017, 46(4): 449-453.

URL:

http://www.zjujournals.com/xueshu/med/10.3785/j.issn.1008-9292.2017.08.17     OR     http://www.zjujournals.com/xueshu/med/Y2017/V46/I4/449


NLRP3炎症小体与儿童自身炎症性疾病研究进展

儿童自身炎症性疾病(AID)是难治性疾病之一,发病机制尚未完全明确。近年来大量研究表明,NLRP3炎症小体失调在儿童AID的发生、发展中具有重要作用。NLRP3炎症小体是细胞内的一种多蛋白复合物,它能激活半胱氨酸天冬氨酸特异蛋白酶1(caspase-1),进一步促进炎症因子IL-1β和IL-18的成熟和分泌,从而促进细胞凋亡,调节固有免疫应答。IL-1受体拮抗剂(Anakinra)和IL-1β单克隆抗体(Canakinumab)治疗儿童AID取得了较好的疗效。本文就NLRP3炎症小体在该类疾病发病机制中的研究进展作一综述。


关键词: 多蛋白复合物,  自身免疫疾病,  白细胞介素1β,  染色体障碍,  综述 

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