The PYRIN connection: novel players in innate immunity and inflammation

J Exp Med. 2004 Sep 6;200(5):551-8. doi: 10.1084/jem.20032234.


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.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Carrier Proteins / genetics
  • Carrier Proteins / physiology*
  • Familial Mediterranean Fever / etiology*
  • Familial Mediterranean Fever / genetics
  • Humans
  • Immune System / physiology*
  • Inflammation / pathology*
  • Models, Genetic
  • Multigene Family
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • Protein Structure, Tertiary


  • Carrier Proteins
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • NLRP3 protein, human