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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2013

Open Access 01-11-2013 | Meeting abstract

OR11-004 - IL-1, IL-18 and cell death in NLRP3 driven disease

Authors: L Broderick, SD Brydges, MD McGeough, CA Pena, JL Mueller, HM Hoffman

Published in: Pediatric Rheumatology | Special Issue 1/2013

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Excerpt

Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome that drives overproduction of the pro-inflammatory cytokines interleukin-1b (IL-1b) and IL-18. Mice expressing mutations associated with familial cold autoinflammatory syndrome (FCAS) or Muckle-Wells syndrome (MWS) exhibit severe, spontaneous inflammation, early death, and hyperresponsiveness to stimuli in vitro. Abrogating IL-1 signaling either genetically or pharmacologically results in modest improvement of life expectancy in murine CAPS, but clearly indicates a role for players in addition to IL-1b. …
Metadata
Title
OR11-004 - IL-1, IL-18 and cell death in NLRP3 driven disease
Authors
L Broderick
SD Brydges
MD McGeough
CA Pena
JL Mueller
HM Hoffman
Publication date
01-11-2013
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue Special Issue 1/2013
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/1546-0096-11-S1-A193

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