NLRP3

NLRP3
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3QF2, 2NAQ
Identifiers
Aliases	NLRP3, AGTAVPRL, AII, AVP, C1orf7, CIAS1, CLR1.1, FCAS, FCAS1, FCU, MWS, NALP3, PYPAF1, NLR family, pyrin domain containing 3, NLR family pyrin domain containing 3, DFNA34, KEFH
External IDs	OMIM: 606416; MGI: 2653833; HomoloGene: 3600; GeneCards: NLRP3; OMA:NLRP3 - orthologs
Gene location (Human)
Chr.	Chromosome 1 (human)
End	247,449,108 bp
Gene location (Mouse)
Chr.	Chromosome 11 (mouse)
End	59,457,782 bp
RNA expression pattern
	Top expressed in
	monocyte; ; granulocyte; ; blood; ; bone marrow cell; ; gallbladder; ; testicle; ; right lung; ; appendix; ; upper lobe of left lung; ; spleen;
	Top expressed in
	granulocyte; ; blood; ; stroma of bone marrow; ; morula; ; calvaria; ; spleen; ; submandibular gland; ; gastrula; ; olfactory epithelium; ; meninges;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	sequence-specific DNA binding; nucleotide binding; transcription factor binding; protein binding; peptidoglycan binding; ATP binding; identical protein binding;
Cellular component	cytoplasm; cytosol; extracellular region; endoplasmic reticulum; inflammasome complex; nucleus; NLRP3 inflammasome complex; Golgi membrane; Golgi apparatus; membrane;
Biological process	defense response; detection of biotic stimulus; NLRP3 inflammasome complex assembly; regulation of transcription, DNA-templated; negative regulation of acute inflammatory response; positive regulation of interleukin-5 production; interleukin-18 production; immune system process; positive regulation of interleukin-4 production; transcription, DNA-templated; positive regulation of cysteine-type endopeptidase activity involved in apoptotic process; positive regulation of interleukin-13 production; positive regulation of T-helper 17 cell differentiation; defense response to virus; protein complex oligomerization; positive regulation of T-helper 2 cell differentiation; regulation of inflammatory response; interleukin-1 beta production; positive regulation of type 2 immune response; inflammatory response; negative regulation of NF-kappaB transcription factor activity; activation of cysteine-type endopeptidase activity involved in apoptotic process; negative regulation of inflammatory response; cellular response to lipopolysaccharide; positive regulation of T-helper 2 cell cytokine production; signal transduction; positive regulation of transcription by RNA polymerase II; apoptotic process; innate immune response; protein deubiquitination; defense response to Gram-positive bacterium; positive regulation of NF-kappaB transcription factor activity; cellular response to peptidoglycan; negative regulation of NIK/NF-kappaB signaling;
	Sources:Amigo / QuickGO
Orthologs
	114548
	216799
	ENSG00000162711
	ENSMUSG00000032691
	Q96P20
	Q8R4B8
NM_001079821; NM_001127461; NM_001127462; NM_001243133; NM_004895;
	NM_183395
	NM_145827; NM_001359638
NP_001073289; NP_001120933; NP_001120934; NP_001230062; NP_004886;
	NP_899632
	NP_665826; NP_001346567
	Wikidata
View/Edit Human	View/Edit Mouse

NLR family pyrin domain containing 3 (NLRP3) is a protein that in humans is encoded by the NLRP3 gene located on the long arm of chromosome 1. NLRP3 has previously been known as NACHT, LRR, and PYD domains-containing protein 3 [NALP3]; cryopyrin; cold induced autoinflammatory syndrome 1 (CIAS1), caterpillar-like receptor 1.1 (CLR1.1), and PYRIN-containing APAF1-like protein 1 (PYPAF1).

NLRP3 is a component of the innate immune system that functions as a pattern recognition receptor (PRR) – a cytosolic sensor that responds to pathogen-associated molecular patterns (PAMPs) and damage-associated molecular patterns (DAMPs). NLRP3 belongs to the NOD-like receptor (NLR) family of PRRs.

NLRP3 is expressed predominantly in macrophages, where it serves as a component of the inflammasome. The NLPR3 inflammasome triggers inflammation and an immune response, and causes cell death through pyroptosis or PANoptosis. Since its discovery in 2004, the NLRP3 inflammasome has emerged as a critical component of innate immunity, and mutations in the NLRP3 gene have been associated with a number of organ-specific autoimmune diseases, autoinflammatory diseases, and cancers. Hence, the NLRP3 inflammasome has become the best-understood inflammasome and a central focus of inflammation research.