May function as an inducer of apoptosis. Interacts selectively with ASC and this complex may function as an upstream activator of NF-kappa-B signaling. Inhibits TNF-alpha induced activation and nuclear translocation of RELA/NF-KB p65. Also inhibits transcriptional activity of RELA. Activates caspase-1 in response to a number of triggers including bacterial or viral infection which leads to processing and release of IL1B and IL18. Defects in NLRP3 are the cause of familial cold autoinflammatory syndrome type 1 (FCAS1); also known as familial cold urticaria. FCAS are rare autosomal dominant systemic inflammatory diseases characterized by episodes of rash, arthralgia, fever and conjunctivitis after generalized exposure to cold. Defects in NLRP3 are a cause of Muckle-Wells syndrome (MWS); also known as urticaria-deafness-amyloidosis syndrome. MWS is a hereditary periodic fever syndrome characterized by fever, chronic recurrent urticaria, arthralgias, progressive sensorineural deafness, and reactive renal amyloidosis. The disease may be severe if generalized amyloidosis occurs. Defects in NLRP3 are the cause of chronic infantile neurologic cutaneous and articular syndrome (CINCA); also known as neonatal onset multisystem inflammatory disease (NOMID). CINCA is a rare congenital inflammatory disorder characterized by a triad of neonatal onset of cutaneous symptoms, chronic meningitis and joint manifestations with recurrent fever and inflammation. Belongs to the NLRP family. 5 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Molecular Function: identical protein binding; peptidoglycan binding; protein binding; sequence-specific DNA binding; transcription factor binding
Biological Process: activation of NF-kappaB transcription factor; defense response; detection of biotic stimulus; inflammatory response; inhibition of NF-kappaB transcription factor; negative regulation of acute inflammatory response; negative regulation of inflammatory response; negative regulation of interleukin-1 beta secretion; negative regulation of NF-kappaB import into nucleus; positive regulation of caspase activity; positive regulation of interleukin-1 beta secretion; positive regulation of interleukin-4 production; positive regulation of T-helper 2 cell differentiation; positive regulation of T-helper 2 type immune response; positive regulation of transcription from RNA polymerase II promoter; protein deubiquitination; protein oligomerization