NALP3: a key player in caspase-1 activation

J Endotoxin Res. 2006;12(4):251-6. doi: 10.1177/09680519060120040701.


The NLR (NACHT-LRR) family of proteins have been implicated in the regulation of immune responses and cell death pathways. Some NLR family members can form multiprotein complexes, called inflammasomes, involved in the activation of pro-inflammatory caspases. Mutations in the NALP3/CIAS1/cryopyrin gene, a member of the NLR family, are linked to three auto-inflammatory disorders: Muckle-Wells syndrome, familial cold auto-inflammatory syndrome and neonatal-onset multisystem inflammatory disease. NALP3 along with the adaptor molecule ASC activates caspase-1 in response to a wide variety of stimuli. Here we review recent findings on the biology of NALP3 suggesting that it has functions beyond that of pathogen recognition.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis / immunology*
  • Carrier Proteins / physiology*
  • Caspase 1 / metabolism*
  • Enzyme Activation
  • Genetic Predisposition to Disease
  • Humans
  • Immunity, Innate / immunology
  • Inflammation / immunology
  • Inflammation / metabolism*
  • Mice
  • Mutation
  • NLR Family, Pyrin Domain-Containing 3 Protein


  • Carrier Proteins
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • NLRP3 protein, human
  • Nlrp3 protein, mouse
  • Caspase 1