| Immunology in clinic review series; focus on autoinflammatory diseases: role of inflammasomes in autoinflammatory syndromes. | |
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MedLine Citation:
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PMID: 22288581 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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OTHER THEMES PUBLISHED IN THIS IMMUNOLOGY IN THE CLINIC REVIEW SERIES Allergy, Host Responses, Cancer, Type 1 diabetes and viruses, Metabolic diseases. SUMMARY: 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. |
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Authors:
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V U Ozkurede; L Franchi |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Clinical and experimental immunology Volume: 167 ISSN: 1365-2249 ISO Abbreviation: Clin. Exp. Immunol. Publication Date: 2012 Mar |
Date Detail:
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Created Date: 2012-01-31 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0057202 Medline TA: Clin Exp Immunol Country: England |
Other Details:
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Languages: eng Pagination: 382-90 Citation Subset: IM |
Copyright Information:
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© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology. |
Affiliation:
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Department of Pathology and Comprehensive Cancer Center, University of Michigan Medical School, Ann Arbor, MI, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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