Published 7 September 2004. doi:10.1084/jem.20032234
Rockefeller University Press, 0022-1007 $8.00
JEM, Volume 200, Number 5, 551-558
The PYRIN Connection
:
Novel Players in Innate Immunity and Inflammation
Christian Stehlik1 and
John C. Reed2
1 Mary Babb Randolph Cancer Center and Department of Microbiology, Immunology and Cell Biology, West Virginia University School of Medicine, 1814 MBRCC, Morgantown, WV 26506
2 The Burnham Institute, La Jolla, CA 92037
Address correspondence to Christian Stehlik, Mary Babb Randolph Cancer Center and Department of Microbiology, Immunology and Cell Biology, West Virginia University School of Medicine, 1814 MBRCC, Morgantown, WV 26506-9300. Phone: (304) 293-8785; Fax: (304) 293-4667; email: cstehlik{at}hsc.wvu.edu; or John C. Reed, The Burnham Institute, 10901 North Torrey Pines Rd., La Jolla, CA 92037. Phone: (858) 646-3140; Fax: (858) 646-3194; email: reedoffice{at}burnham.org
Abstract
Periodic fever syndromes (PFSs) comprise a subset of the hereditary autoinflammatory disorders that are defined by recurrent self-resolving attacks of systemic inflammatory reactions in the absence of infection or autoimmunity. Recent advances have led to the discovery that members of a new family of genes, the PYRIN family, account for several hereditary PFSs. Here we discuss new insights into the function of PYRIN proteins and the molecular basis of PFSs.

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