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Journal of Endotoxin Research
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NALP3: a key player in caspase-1 activation

Fayyaz S. Sutterwala

Section of Immunobiology, Yale University School of Medicine, New Haven, Connecticut, USA, Section of Infectious Diseases, Yale University School of Medicine, New Haven, Connecticut, USA

Yasunori Ogura

Section of Immunobiology, Yale University School of Medicine, New Haven, Connecticut, USA

Dario S. Zamboni

Section of Microbial Pathogenesis, Yale University School of Medicine, New Haven, Conncticut, USA

Craig R. Roy

Section of Microbial Pathogenesis, Yale University School of Medicine, New Haven, Conncticut, USA

Richard A. Flavell

Section of Immunobiology, Yale University School of Medicine, New Haven, Connecticut, USA, Howard Hughes Medical Institute, Yale University School of Medicine, New Haven, Connecticut, USA, richard.flavell{at}yale.edu

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.

Key Words: Caspase-1 • innate immunity • interleukin-1ß • macrophages • NACHT-LRR (NLR)

Journal of Endotoxin Research, Vol. 12, No. 4, 251-256 (2006)
DOI: 10.1177/09680519060120040701
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