NLRP3 a cytosolic pattern recognition receptor and scaffolding protein that plays a crucial role in innate immunity and inflammation. Crucial for the formation and activation of inflammasomes. In response to pathogens and other damage-associated signals, initiates the formation of the inflammasome polymeric complex, made of NLRP3, PYCARD and CASP1 (and possibly CASP4 and CASP5). Recruitment and activation of CASP1 to the inflammasome promotes the proteolytic processing, maturation and secretion of IL1B, IL18 and HMGB1, stimulating inflammatory responses. Interaction with MEFV leads to its degradation by autophagy, preventing excessive IL1B- and IL18-mediated inflammation. Inflammasomes can also induce pyroptosis, an inflammatory form of programmed cell death. Activating stimuli include extracellular ATP, reactive oxygen species, K(+) efflux, amyloid-beta fibers, cytosolic dsRNA, environmental or industrial particles and nanoparticles, etc. Activation in presence of cytosolic dsRNA is mediated by DHX33. Independently of inflammasome activation, regulates the differentiation of T helper 2 (Th2) cells. During Th2 differentiation, required for optimal IRF4 binding to IL4 promoter and for IRF4-dependent IL4 transcription. Involved in the regulation of IL4, IL5, and IL13 production. May function as an inducer of apoptosis. Interacts selectively with PYCARD and this complex may function as an upstream activator of NF-kappa-B signaling. Predominantly expressed in macrophages. Also expressed in dendritic cells, B- and T-cells, and LPS-treated granulocytes, but not in resting cells. Expressed weakly in monocytes and in oral, esophageal and ectocervical mucosa, Hassall's corpuscles in the thymus, the stratified epithelium covering the bladder and ureter, lung epithelial cells, chondrocytes, and in resting osteoblasts. 6 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Inhibitor
Chromosomal Location of human Ortholog: 1q44
Cellular Component:  cytoplasm; cytosol; endoplasmic reticulum; extracellular region; Golgi membrane; interphase microtubule organizing center; membrane; microtubule organizing center; mitochondrion; NLRP3 inflammasome complex; nucleus
Molecular Function:  ADP binding; ATP binding; ATP hydrolysis activity; cysteine-type endopeptidase activator activity; DNA-binding transcription factor binding; identical protein binding; molecular adaptor activity; molecular condensate scaffold activity; peptidoglycan binding; phosphatidylinositol phosphate binding; phosphatidylinositol-4-phosphate binding; protein binding; sequence-specific DNA binding; signaling adaptor activity; small molecule sensor activity
Biological Process:  apoptotic process; cellular response to lipopolysaccharide; cellular response to virus; defense response; detection of biotic stimulus; inflammatory response; innate immune response; negative regulation of acute inflammatory response; negative regulation of inflammatory response; negative regulation of interleukin-1 beta production; negative regulation of NF-kappaB transcription factor activity; negative regulation of NIK/NF-kappaB signaling; NLRP3 inflammasome complex assembly; osmosensory signaling pathway; pattern recognition receptor signaling pathway; positive regulation of cysteine-type endopeptidase activity involved in apoptotic process; positive regulation of inflammatory response; positive regulation of interleukin-1 beta production; positive regulation of interleukin-4 production; positive regulation of NF-kappaB transcription factor activity; positive regulation of NIK/NF-kappaB signaling; positive regulation of T-helper 2 cell cytokine production; positive regulation of T-helper 2 cell differentiation; positive regulation of transcription by RNA polymerase II; positive regulation of type 2 immune response; protein homooligomerization; protein maturation; pyroptosis; signal transduction
Disease: Cinca Syndrome; Deafness, Autosomal Dominant 34, With Or Without Inflammation; Familial Cold Autoinflammatory Syndrome 1; Keratoendotheliitis Fugax Hereditaria; Muckle-wells Syndrome
Reference #:  Q96P20 (UniProtKB)
Alt. Names/Synonyms: AGTAVPRL; AII; AII/AVP; AII/AVP receptor-like; Angiotensin/vasopressin receptor AII/AVP-like; AVP; C1orf7; Caterpiller protein 1.1; CIAS1; CLR1.1; Cold autoinflammatory syndrome 1 protein; Cold-induced autoinflammatory syndrome 1 protein; Cryopyrin; cryopyrin, NACHT, LRR and PYD domains - containing protein 3; deafness, autosomal dominant 34; DFNA34; FCAS; FCAS1; FCU; FLJ95925; KEFH; MWS; NACHT domain-, leucine-rich repeat-, and PYD-containing protein 3; NACHT, LRR and PYD containing protein 3; NACHT, LRR and PYD domains-containing protein 3; NALP3; NLR family pyrin domain containing 3; NLR family, pyrin domain containing 3; NLRP3; nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 3; PYPAF1; PYRIN-containing APAF1-like protein 1
Gene Symbols: NLRP3
Molecular weight: 118,173 Da
Basal Isoelectric point: 6.22  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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NLRP3

Protein Structure Not Found.


Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  CCLE  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein