The PYRIN connection: novel players in innate immunity and inflammation.
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.
Stehlik
Christian
C
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
Reed
John C
JC
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
D002352:Carrier Proteins; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human