Document Detail

Cryopyrin-associated periodic syndromes: background and therapeutics.
MedLine Citation:
PMID:  20140476     Owner:  NLM     Status:  MEDLINE    
Cryopyrin-associated periodic syndromes (CAPS) are caused by mutations of the gene encoding the NLR family protein NLRP3, which together with caspase-1 and adaptor proteins constitutes a protein complex termed the inflammasome. In innate immune reactions, a variety of stimuli activate the NLRP3 inflammasome, triggering caspase-1 to process proIL-1 and thus to produce mature IL-1. Excessive production of IL-1 by monocytes/macrophages is the central pathophysiology of CAPS, and daily injection of the IL-1 receptor antagonist anakinra rapidly ameliorates the inflammatory symptoms in most cases. Furthermore, double-blind, placebo-controlled clinical trials have recently confirmed the efficacy and safety of rilonacept, a fusion protein of the IL-1 receptor and IgG Fc, and canakinumab, a human anti-IL-1 monoclonal antibody, as novel long-lasting agents for treating CAPS.
Tetsuo Kubota; Ryuji Koike
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Publication Detail:
Type:  Journal Article; Review     Date:  2010-02-06
Journal Detail:
Title:  Modern rheumatology / the Japan Rheumatism Association     Volume:  20     ISSN:  1439-7609     ISO Abbreviation:  Mod Rheumatol     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-06-07     Completed Date:  2010-09-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100959226     Medline TA:  Mod Rheumatol     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  213-21     Citation Subset:  IM    
Graduate School of Health Care Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan.
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MeSH Terms
Antibodies, Monoclonal / therapeutic use
Carrier Proteins / genetics*,  immunology
Cryopyrin-associated Periodic Syndromes / genetics*,  immunology,  therapy*
Interleukin-1 / antagonists & inhibitors,  genetics,  immunology
Monocytes / immunology
Recombinant Fusion Proteins / therapeutic use
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Carrier Proteins; 0/Interleukin-1; 0/NLRP3 protein, human; 0/Recombinant Fusion Proteins; 0/canakinumab; 0/rilonacept

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