The PYRIN Connection

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  • Christian Stehlik
    1Mary Babb Randolph Cancer Center and Department of Microbiology, Immunology and Cell Biology, West Virginia University School of Medicine, 1814 MBRCC, Morgantown, WV 26506
  • John C. Reed
    2The Burnham Institute, La Jolla, CA 92037

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<jats:p>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.</jats:p>

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