title: Deregulated NLRP3 and NLRP1 inflammasomes and their correlations with disease activity in systemic lupus erythematosus.
abstract: OBJECTIVE
  NOD-like receptor family, pyrin domain containing 3 and 1 (NLRP3 and NLRP1) inflammasomes are molecular platforms that sense the damage or danger signals of cells. We investigated whether NLRP3/NLRP1 inflammasomes are involved in the pathogenesis and progression of systemic lupus erythematosus (SLE).
  
  
  METHODS
  Expressions of inflammasome components at the mRNA and protein levels in the peripheral blood mononuclear cells (PBMC) from patients with SLE and healthy controls were investigated by quantitative real-time transcription PCR and Western blot, respectively. Correlations between NLRP3/NLRP1 inflammasome components' expression and clinical disease progression were investigated. Expressions of NLRP3/NLRP1 inflammasomes before and after treatment in the patients with SLE were also analyzed and compared.
  
  
  RESULTS
  Our data showed that expressions of NLRP3/NLRP1 inflammasomes were significantly downregulated in PBMC from patients with SLE compared with PBMC from healthy controls. Further, expressions of NLRP3/NLRP1 inflammasomes were negatively correlated with the SLE Disease Activity Index, and regular glucocorticoid treatment significantly corrected this deregulation of these inflammasomes. Further analysis showed that type I interferon (IFN) level was significantly negatively correlated with expression of NLRP3/NLRP1 inflammasomes, which indicated that enhanced IFN-I level in patients with SLE was responsible, at least to a great degree, for the deregulation of inflammasomes.
  
  
  CONCLUSIONS
  These results indicated deregulation of NLRP3/NLRP1 inflammasomes in patients with SLE, and suggested an important role for inflammasomes in the pathogenesis and progression of SLE.
authors: From the Department of Immunology, Shandong University School of Medicine; the Provincial Hospital Affiliated to Shandong University; and the Department of Pathology, Shandong University School of Medicine, Jinan, China.
authors: Qingrui
authors: Q
authors: Yang
authors: Chengcheng
authors: C
authors: Yu
authors: Zhaowen
authors: Z
authors: Yang
authors: Qing
authors: Q
authors: Wei
authors: Kun
authors: K
authors: Mu
authors: Ying
authors: Y
authors: Zhang
authors: Wei
authors: W
authors: Zhao
authors: Xiaofeng
authors: X
authors: Wang
authors: Wanwan
authors: W
authors: Huai
authors: Lihui
authors: L
authors: Han
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000378:metabolism*; D000293:Adolescent; D000328:Adult; D051017:Apoptosis Regulatory Proteins / Q000378:metabolism*; D002352:Carrier Proteins / Q000378:metabolism*; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D058847:Inflammasomes / Q000378:metabolism*; D007963:Leukocytes, Mononuclear / Q000187:drug effects / Q000378:metabolism; D020945:Lupus Vasculitis, Central Nervous System / Q000188:drug therapy / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D000070576:NLR Proteins; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D051017:Apoptosis Regulatory Proteins; D002352:Carrier Proteins; D005938:Glucocorticoids; D058847:Inflammasomes; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D000070576:NLR Proteins; C427903:NLRP1 protein, human; C453881:NLRP3 protein, human
keywords: AUTOIMMUNE DISEASE; DISEASE ACTIVITY; INFLAMMATION; SYSTEMIC LUPUS ERYTHEMATOSUS