Immunology in clinic review series; focus on autoinflammatory diseases: role of inflammasomes in autoinflammatory syndromes
Article first published online: 30 JAN 2012
© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology
Clinical & Experimental Immunology
Volume 167, Issue 3, pages 382–390, March 2012
How to Cite
Ozkurede, V. U. and Franchi, L. (2012), Immunology in clinic review series; focus on autoinflammatory diseases: role of inflammasomes in autoinflammatory syndromes. Clinical & Experimental Immunology, 167: 382–390. doi: 10.1111/j.1365-2249.2011.04535.x
- Issue published online: 30 JAN 2012
- Article first published online: 30 JAN 2012
- Accepted manuscript online: 25 NOV 2011 01:38PM EST
- Accepted for publication 18 November 2011
- innate immunity
OTHER THEMES PUBLISHED IN THIS IMMUNOLOGY IN THE CLINIC REVIEW SERIES
Allergy, Host Responses, Cancer, Type 1 diabetes and viruses, Metabolic diseases.
Autoinflammatory syndromes are disorders characterized by the hyperactivation of the innate immune system in the absence of microbial infection or autoantibody production. Some autoinflammatory syndromes are associated with recurrent episodes of fever and systemic inflammation that are caused by dysregulated activation of inflammasomes, molecular platforms responsible for the activation of caspase-1 and the production of interleukin (IL)-1β. In this review we will discuss the role of IL-1β and the inflammasomes in host defence and how mutations of two genes, NLRP3 and PYRIN, leads to the autoinflammatory syndromes, cryopyrin-associated periodic syndromes (CAPS) and familial Mediterranean fever (FMF). Both CAPS and FMF are characterized by increased inflammasome activity and overproduction of IL-1β which is ultimately responsible for disease manifestations. Importantly, understanding the molecular mechanisms of these syndromes has led to effective treatment for these rare diseases with biological drugs that target IL-1β-mediated signalling.