Published in:
Open Access
01-11-2013 | Meeting abstract
OR11-006 - A mutation in NLRP1A causes autoinflammation
Authors:
SL Masters, M Gerlic, BT Kile, BA Croker
Published in:
Pediatric Rheumatology
|
Special Issue 1/2013
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Excerpt
The NLRs (Nucleotide-binding domain and Leucine-rich repeat containing Receptors) are a family of intracellular innate immune receptors involved in host defense. Upon activation, NLRs form large protein complexes called “inflammasomes” that bind and activate Caspase-1, resulting in proteolytic activation of the pro-inflammatory cytokines pro-IL-1β and pro-IL-18 and also induce a Caspase-1-dependent form of cell death known as pyroptosis. …