title: The PYRIN connection: novel players in innate immunity and inflammation.
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.
authors.affiliation: 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, USA. cstehlik@hsc.wvu.edu
authors.forename: Christian
authors.initials: C
authors.lastname: Stehlik
authors.forename: John C
authors.initials: JC
authors.lastname: Reed
mesh_terms: D002352:Carrier Proteins / Q000235:genetics / Q000502:physiology*; D010505:Familial Mediterranean Fever / Q000209:etiology* / Q000235:genetics; D006801:Humans; D007107:Immune System / Q000502:physiology*; D007249:Inflammation / Q000473:pathology*; D008957:Models, Genetic; D005810:Multigene Family; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D017434:Protein Structure, Tertiary
chemical_list: D002352:Carrier Proteins; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human