NLRP3 (Homo sapiens)
Description [+]
- Synonyms: NLRP3, AGTAVPRL, AII, AVP, FCAS, FCU, NALP3, PYPAF1, MWS, CLR1.1
- Species: Metazoa;Bilateria;Deuterostoma;Chordata;Vertebrata;Mammalia;Primates;Hominidae; Homo sapiens
- Short gene description: NACHT, LRR and PYD domains-containing protein 3 (Cold autoinflammatory syndrome 1 protein)(Cryopyrin)(PYRIN-containing APAF1-like protein 1)(Angiotensin/vasopressin receptor AII/AVP-like) [Source:UniProtKB/Swiss-Prot;Acc:Q96P20]
- Family: other
- Process: immunity,
- Pathways: inflammasome,
- Criteria: manually curated
- Curator comment:
- Mouse ortholog(s): Nlrp3
- WIKI: NLRP3-H_sapiens
References [+]
- NALPs: a novel protein family involved in inflammation.
- Tschopp J, Martinon F, Burns K
- A newly discovered family of cytoplasmic proteins--the NALPs--has been implicated in the activation of caspase-1 by the Toll-like receptors (TLRs) during the cell's response to microbial infection. Like the structurally related apoptotic protease-activating factor-1 (APAF-1), which is responsible for the activation of caspase-9, the NALP1 protein forms a large, signal-induced multiprotein complex, the inflammasome, resulting in the activation of pro-inflammatory caspases. Nat Rev Mol Cell Biol. 2003 Feb;4(2):95-104.
- NOD-LRR proteins: role in host-microbial interactions and inflammatory disease.
- Inohara, Chamaillard, McDonald C, Nunez G
- Nods are cytosolic proteins that contain a nucleotide-binding oligomerization domain (NOD). These proteins include key regulators of apoptosis and pathogen resistance in mammals and plants. A large number of Nods contain leucine-rich repeats (LRRs), hence referred to as NOD-LRR proteins. Genetic variation in several NOD-LRR proteins, including human Nod2, Cryopyrin, and CIITA, as well as mouse Naip5, is associated with inflammatory disease or increased susceptibility to microbial infections. Nod1, Nod2, Cryopyrin, and Ipaf have been implicated in protective immune responses against pathogens. Together with Toll-like receptors, Nod1 and Nod2 appear to play important roles in innate and acquired immunity as sensors of bacterial components. Specifically, Nod1 and Nod2 participate in the signaling events triggered by host recognition of specific motifs in bacterial peptidoglycan and, upon activation, induce the production of proinflammatory mediators. Naip5 is involved in host resistance to Legionella pneumophila through cell autonomous mechanisms, whereas CIITA plays a critical role in antigen presentation and development of antigen-specific T lymphocytes. Thus, NOD-LRR proteins appear to be involved in a diverse array of processes required for host immune reactions against pathogens. Annu Rev Biochem. 2005;74:355-83.
- References from Mouse ortholog(s):
- NALPs: a novel protein family involved in inflammation.
- Tschopp J, Martinon F, Burns K
- A newly discovered family of cytoplasmic proteins--the NALPs--has been implicated in the activation of caspase-1 by the Toll-like receptors (TLRs) during the cell's response to microbial infection. Like the structurally related apoptotic protease-activating factor-1 (APAF-1), which is responsible for the activation of caspase-9, the NALP1 protein forms a large, signal-induced multiprotein complex, the inflammasome, resulting in the activation of pro-inflammatory caspases. Nat Rev Mol Cell Biol. 2003 Feb;4(2):95-104.
- NOD-LRR proteins: role in host-microbial interactions and inflammatory disease.
- Inohara, Chamaillard, McDonald C, Nunez G
- Nods are cytosolic proteins that contain a nucleotide-binding oligomerization domain (NOD). These proteins include key regulators of apoptosis and pathogen resistance in mammals and plants. A large number of Nods contain leucine-rich repeats (LRRs), hence referred to as NOD-LRR proteins. Genetic variation in several NOD-LRR proteins, including human Nod2, Cryopyrin, and CIITA, as well as mouse Naip5, is associated with inflammatory disease or increased susceptibility to microbial infections. Nod1, Nod2, Cryopyrin, and Ipaf have been implicated in protective immune responses against pathogens. Together with Toll-like receptors, Nod1 and Nod2 appear to play important roles in innate and acquired immunity as sensors of bacterial components. Specifically, Nod1 and Nod2 participate in the signaling events triggered by host recognition of specific motifs in bacterial peptidoglycan and, upon activation, induce the production of proinflammatory mediators. Naip5 is involved in host resistance to Legionella pneumophila through cell autonomous mechanisms, whereas CIITA plays a critical role in antigen presentation and development of antigen-specific T lymphocytes. Thus, NOD-LRR proteins appear to be involved in a diverse array of processes required for host immune reactions against pathogens. Annu Rev Biochem. 2005;74:355-83.
Structure & Sequence [+]
Pfam domains:
(Pfam is a large collection of protein families.)
| Source | Domain Name | Start | End |
|---|---|---|---|
| PFAM A | PAAD_DAPIN | 6 | 89 |
| PFAM A | NACHT | 220 | 389 |
Protein sequence [+]
NLRP3 | Homo sapiens | 9606 | length:1036
MKMASTRCKLARYLEDLEDVDLKKFKMHLEDYPPQKGCIPLPRGQTEKADHVDLATLMID
FNGEEKAWAMAVWIFAAINRRDLYEKAKRDEPKWGSDNARVSNPTVICQEDSIEEEWMGL
LEYLSRISICKMKKDYRKKYRKYVRSRFQCIEDRNARLGESVSLNKRYTRLRLIKEHRSQ
QEREQELLAIGKTKTCESPVSPIKMELLFDPDDEHSEPVHTVVFQGAAGIGKTILARKMM
LDWASGTLYQDRFDYLFYIHCREVSLVTQRSLGDLIMSCCPDPNPPIHKIVRKPSRILFL
MDGFDELQGAFDEHIGPLCTDWQKAERGDILLSSLIRKKLLPEASLLITTRPVALEKLQH
LLDHPRHVEILGFSEAKRKEYFFKYFSDEAQARAAFSLIQENEVLFTMCFIPLVCWIVCT
GLKQQMESGKSLAQTSKTTTAVYVFFLSSLLQPRGGSQEHGLCAHLWGLCSLAADGIWNQ
KILFEESDLRNHGLQKADVSAFLRMNLFQKEVDCEKFYSFIHMTFQEFFAAMYYLLEEEK
EGRTNVPGSRLKLPSRDVTVLLENYGKFEKGYLIFVVRFLFGLVNQERTSYLEKKLSCKI
SQQIRLELLKWIEVKAKAKKLQIQPSQLELFYCLYEMQEEDFVQRAMDYFPKIEINLSTR
MDHMVSSFCIENCHRVESLSLGFLHNMPKEEEEEEKEGRHLDMVQCVLPSSSHAACSHGL
VNSHLTSSFCRGLFSVLSTSQSLTELDLSDNSLGDPGMRVLCETLQHPGCNIRRLWLGRC
GLSHECCFDISLVLSSNQKLVELDLSDNALGDFGIRLLCVGLKHLLCNLKKLWLVSCCLT
SACCQDLASVLSTSHSLTRLYVGENALGDSGVAILCEKAKNPQCNLQKLGLVNSGLTSVC
CSALSSVLSTNQNLTHLYLRGNTLGDKGIKLLCEGLLHPDCKLQVLELDNCNLTSHCCWD
LSTLLTSSQSLRKLSLGNNDLGDLGVMMFCEVLKQQSCLLQNLGLSEMYFNYETKSALET
LQEEKPELTVVFEPSW
FNGEEKAWAMAVWIFAAINRRDLYEKAKRDEPKWGSDNARVSNPTVICQEDSIEEEWMGL
LEYLSRISICKMKKDYRKKYRKYVRSRFQCIEDRNARLGESVSLNKRYTRLRLIKEHRSQ
QEREQELLAIGKTKTCESPVSPIKMELLFDPDDEHSEPVHTVVFQGAAGIGKTILARKMM
LDWASGTLYQDRFDYLFYIHCREVSLVTQRSLGDLIMSCCPDPNPPIHKIVRKPSRILFL
MDGFDELQGAFDEHIGPLCTDWQKAERGDILLSSLIRKKLLPEASLLITTRPVALEKLQH
LLDHPRHVEILGFSEAKRKEYFFKYFSDEAQARAAFSLIQENEVLFTMCFIPLVCWIVCT
GLKQQMESGKSLAQTSKTTTAVYVFFLSSLLQPRGGSQEHGLCAHLWGLCSLAADGIWNQ
KILFEESDLRNHGLQKADVSAFLRMNLFQKEVDCEKFYSFIHMTFQEFFAAMYYLLEEEK
EGRTNVPGSRLKLPSRDVTVLLENYGKFEKGYLIFVVRFLFGLVNQERTSYLEKKLSCKI
SQQIRLELLKWIEVKAKAKKLQIQPSQLELFYCLYEMQEEDFVQRAMDYFPKIEINLSTR
MDHMVSSFCIENCHRVESLSLGFLHNMPKEEEEEEKEGRHLDMVQCVLPSSSHAACSHGL
VNSHLTSSFCRGLFSVLSTSQSLTELDLSDNSLGDPGMRVLCETLQHPGCNIRRLWLGRC
GLSHECCFDISLVLSSNQKLVELDLSDNALGDFGIRLLCVGLKHLLCNLKKLWLVSCCLT
SACCQDLASVLSTSHSLTRLYVGENALGDSGVAILCEKAKNPQCNLQKLGLVNSGLTSVC
CSALSSVLSTNQNLTHLYLRGNTLGDKGIKLLCEGLLHPDCKLQVLELDNCNLTSHCCWD
LSTLLTSSQSLRKLSLGNNDLGDLGVMMFCEVLKQQSCLLQNLGLSEMYFNYETKSALET
LQEEKPELTVVFEPSW
Structure links:
Evolution [+]
View protein alignment and tree with Jalview:
Explore tree at phylomeDB: Click here.
Homologs list [+]
| Name | Relationship | Species |
|---|---|---|
| NLRP3 | orthology | Chimpanzee |
| NP_001095689.1 | orthology | Cow |
| NLRP3 | orthology | Dog |
| Q68U61_9PRIM | orthology | Gorilla |
| NLRP3 | orthology | Horse |
| NLRP3 | orthology | Macaca |
| Nlrp3 | orthology | Mouse |
| NLRP3 | orthology | Orangutan |
| NLRP3 | orthology | Ornithorhynchus |
| NLRP3 | orthology | Rabbit |
| Nlrp3_predicted | orthology | Rat |
| Q5F3J4_CHICK | paralogy | Chicken |
| NLRP8 | paralogy | Chimpanzee |
| NALP5 | paralogy | Chimpanzee |
| NLRP4 | paralogy | Chimpanzee |
| NLRP2 | paralogy | Chimpanzee |
| NALP6 | paralogy | Chimpanzee |
| NLRP14 | paralogy | Chimpanzee |
| XR_024982.1 | paralogy | Chimpanzee |
| NLRP1 | paralogy | Chimpanzee |
| NALP12 | paralogy | Chimpanzee |
| NLRP7 | paralogy | Chimpanzee |
| NLRP9 | paralogy | Chimpanzee |
| NLRP11 | paralogy | Chimpanzee |
| NLRP13 | paralogy | Chimpanzee |
| IPI00690027.1 | paralogy | Cow |
| NALP9_BOVIN | paralogy | Cow |
| NALP5_BOVIN | paralogy | Cow |
| IPI00686327.3 | paralogy | Cow |
| NLRP13 | paralogy | Cow |
| NLRP8 | paralogy | Cow |
| NALP12 | paralogy | Cow |
| NALP6 | paralogy | Cow |
| NALP5 | paralogy | Dog |
| NLRP8 | paralogy | Dog |
| C_familiaris_ENSCAFP00000003655 | paralogy | Dog |
| NLRP13 | paralogy | Dog |
| C_familiaris_ENSCAFP00000003831 | paralogy | Dog |
| NALP12 | paralogy | Dog |
| NALP6 | paralogy | Dog |
| NLRP14 | paralogy | Dog |
| NLRP10 | paralogy | Dog |
| NLRP1 | paralogy | Dog |
| C_familiaris_ENSCAFP00000026585 | paralogy | Dog |
| T_rubripes_ENSTRUP00000000216 | paralogy | Fugu |
| T_rubripes_ENSTRUP00000033190 | paralogy | Fugu |
| NLRP2 | paralogy | Gorilla |
| NLRP13 | paralogy | Gorilla |
| NALP12 | paralogy | Gorilla |
| NLRP10 | paralogy | Gorilla |
| NLRP7 | paralogy | Gorilla |
| NLRP1 | paralogy | Gorilla |
| E_caballus_ENSECAP00000002676 | paralogy | Horse |
| NLRP1 | paralogy | Horse |
| NLRP14 | paralogy | Horse |
| E_caballus_ENSECAP00000014445 | paralogy | Horse |
| NALP12 | paralogy | Horse |
| NLRP10 | paralogy | Horse |
| E_caballus_ENSECAP00000017692 | paralogy | Horse |
| NLRP4 | paralogy | Horse |
| NLRP13 | paralogy | Horse |
| NALP5 | paralogy | Horse |
| NLRP2 | paralogy | Human |
| NLRP1 | paralogy | Human |
| NLRP8 | paralogy | Human |
| NLRP14 | paralogy | Human |
| NLRP4 | paralogy | Human |
| NALP6 | paralogy | Human |
| NLRP11 | paralogy | Human |
| NLRP10 | paralogy | Human |
| NLRP7 | paralogy | Human |
| NLRP9 | paralogy | Human |
| NLRP13 | paralogy | Human |
| NALP5 | paralogy | Human |
| NALP12 | paralogy | Human |
| A_carolinensis_ENSACAP00000017210 | paralogy | Lyzard |
| NLRP10 | paralogy | Macaca |
| NLRP13 | paralogy | Macaca |
| NALP12 | paralogy | Macaca |
| NLRP4 | paralogy | Macaca |
| NLRP2 | paralogy | Macaca |
| NLRP7 | paralogy | Macaca |
| NLRP9 | paralogy | Macaca |
| NLRP1 | paralogy | Macaca |
| NALP6 | paralogy | Macaca |
| NLRP11 | paralogy | Macaca |
| NLRP14 | paralogy | Macaca |
| NLRP8 | paralogy | Macaca |
| O_latipes_ENSORLP00000000732 | paralogy | Medaka |
| NLRP8 | paralogy | Monodelphis |
| NLRP1 | paralogy | Monodelphis |
| NALP12 | paralogy | Monodelphis |
| RNH1 | paralogy | Monodelphis |
| NLRP10 | paralogy | Monodelphis |
| Nlrp5 | paralogy | Mouse |
| Nlrp1a | paralogy | Mouse |
| Nlrp4f | paralogy | Mouse |
| Nlrp4b | paralogy | Mouse |
| Nlrp2 | paralogy | Mouse |
| Nlrp4c | paralogy | Mouse |
| Nlrp10 | paralogy | Mouse |
| Nlrp1c | paralogy | Mouse |
| Nlrp4g | paralogy | Mouse |
| Nlrp4a | paralogy | Mouse |
| Nlrp9b | paralogy | Mouse |
| Nlrp4e | paralogy | Mouse |
| AC171240.3-201 | paralogy | Mouse |
| Nlrp14 | paralogy | Mouse |
| Nlrp9c | paralogy | Mouse |
| Nlrp4d | paralogy | Mouse |
| Nlrp6 | paralogy | Mouse |
| Nlrp9a | paralogy | Mouse |
| Nlrp1b | paralogy | Mouse |
| Nlrp12 | paralogy | Mouse |
| NALP6 | paralogy | Orangutan |
| P_pygmaeus_ENSPPYP00000004039 | paralogy | Orangutan |
| P_pygmaeus_ENSPPYP00000004673 | paralogy | Orangutan |
| NLRP1 | paralogy | Orangutan |
| NALP12 | paralogy | Orangutan |
| NLRP7 | paralogy | Orangutan |
| NLRP2 | paralogy | Orangutan |
| NLRP9 | paralogy | Orangutan |
| NLRP11 | paralogy | Orangutan |
| NLRP4 | paralogy | Orangutan |
| NLRP13 | paralogy | Orangutan |
| NALP5 | paralogy | Orangutan |
| NALP12 | paralogy | Ornithorhynchus |
| O_anatinus_ENSOANP00000018607 | paralogy | Ornithorhynchus |
| O_anatinus_ENSOANP00000018610 | paralogy | Ornithorhynchus |
| NLRP14 | paralogy | Rabbit |
| NLRP11 | paralogy | Rabbit |
| NALP5 | paralogy | Rabbit |
| NALP12 | paralogy | Rabbit |
| NLRP10 | paralogy | Rabbit |
| NLRP9 | paralogy | Rabbit |
| NLRP8 | paralogy | Rabbit |
| Nlrp6 | paralogy | Rat |
| NP_001099761.1 | paralogy | Rat |
| Nalp9b_predicted | paralogy | Rat |
| R_norvegicus_ENSRNOP00000030734 | paralogy | Rat |
| RGD1560291_predicted | paralogy | Rat |
| LOC687766 | paralogy | Rat |
| RGD1559894_predicted | paralogy | Rat |
| RGD1563529_predicted | paralogy | Rat |
| Nalp12_predicted | paralogy | Rat |
| NP_001099690.1 | paralogy | Rat |
| T_nigroviridis_ENSTNIP00000008085 | paralogy | Tetraodon |
| NALP12 | paralogy | Zebra finch |
| zgc:136791 | paralogy | Zebrafish |
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Gene Ontology [+]
| GO id | Name | Ontology type | Evidence |
|---|---|---|---|
| GO:0009595 | detection of biotic stimulus | biological_proccess | TAS |
| GO:0043280 | positive regulation of caspase activity | biological_proccess | IDA |
| GO:0050718 | positive regulation of interleukin-1 beta secretion | biological_proccess | IDA |
| GO:0051259 | protein oligomerization | biological_proccess | TAS |
| GO:0042347 | negative regulation of NF-kappaB import into nucleus | biological_proccess | IDA |
| GO:0032088 | negative regulation of NF-kappaB transcription factor activity | biological_proccess | IDA |
| GO:0006915 | apoptosis | biological_proccess | IEA |
| GO:0006954 | inflammatory response | biological_proccess | IMP |
| GO:0007165 | signal transduction | biological_proccess | NAS |
| GO:0006917 | induction of apoptosis | biological_proccess | NAS |
| GO:0006952 | defense response | biological_proccess | IEA |
| GO:0006919 | activation of caspase activity | biological_proccess | IEA |
| GO:0032621 | interleukin-18 production | biological_proccess | IEA |
| GO:0050701 | interleukin-1 secretion | biological_proccess | IEA |
| GO:0051607 | defense response to virus | biological_proccess | IEA |
| GO:0042834 | peptidoglycan binding | mollecular_function | TAS |
| GO:0005515 | protein binding | mollecular_function | IPI |
| GO:0000166 | nucleotide binding | mollecular_function | IEA |
| GO:0005524 | ATP binding | mollecular_function | IEA |
| GO:0003677 | DNA binding | mollecular_function | IEA |
| GO:0005515 | protein binding | mollecular_function | IEA |
| GO:0005737 | cytoplasm | cell_component | TAS |
| GO:0005634 | nucleus | cell_component | IEA |
Check GO Evidence Codes here
Curated Isoforms [+]
| Transcript | Translation |
|---|---|
| OTTHUMT00000097740 | OTTHUMP00000038408 |
| OTTHUMT00000097741 | OTTHUMP00000038409 |
| OTTHUMT00000097742 * | OTTHUMP00000038410 * |
| OTTHUMT00000097743 | OTTHUMP00000038411 |
| OTTHUMT00000097744 |
Info from The Vertebrate Genome Annotation (VEGA) database.
(*) Canonical transcript and translation forms.
Information from other databases [+]
- Gene info from HGNC [?] :16400
- Gene related info from GeneCards [?] : NLRP3
- Ensembl genome browser [?] : ENSG00000162711
- Expression info from Arrayexpress [?] : ENSG00000162711
- Protein expression from Protein Atlas: [?] ENSG00000162711
- Community gene edition from Wikigenes: [?] 114548
- entrezgene: 114548
- refseq_dna: NM_001079821
- refseq_dna: NM_004895
- refseq_dna: NM_001127462
- refseq_dna: NM_183395
- refseq_dna: NM_001127461
- refseq_peptide: NP_001073289
- refseq_peptide: NP_004886
- refseq_peptide: NP_001120934
- refseq_peptide: NP_899632
- refseq_peptide: NP_001120933
Click on [?] for more information.
