NLRP3 | NOD-like receptor family | IUPHAR Guide to IMMUNOPHARMACOLOGY

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Target id: 1770

Nomenclature: NLRP3

Family: NOD-like receptor family

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: ON :     NLRP3 has curated data in GtoImmuPdb

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 1036 1q44 NLRP3 NLR family pyrin domain containing 3
Mouse - 1033 11 37.73 cM Nlrp3 NLR family, pyrin domain containing 3
Rat - - 10q22 Nlrp3 NLR family, pyrin domain containing 3
Previous and Unofficial Names
AVP | C1orf7 | CIAS1 | NALP3 | AGTAVPRL | CLR1.1 | FCAS | FCU | MWS | AII | cold autoinflammatory syndrome 1 | Cryopyrin | NACHT, LRR and PYD domains-containing protein 3 | NLR family, pyrin domain containing 3 | PYPAF1 | NLR family
Database Links
Ensembl Gene
Entrez Gene
Human Protein Atlas

Download all structure-activity data for this target as a CSV file

Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Reference
CY-09 Hs Inhibition 6.3 pKd 5
pKd 6.3 (Kd 5x10-7 M) [5]
Description: MST assay for the affinity between CY-09 and purified GFP-NLRP3 protein.
MCC950 Hs Inhibition >8.0 pIC50 2
pIC50 >8.0 (IC50 <1x10-8 M) [2]
dapansutrile Hs Inhibition - - 7
Description: As assessed by inhibition of recombinant NLRP3 ATPase activity by dapansutrile.
Physiological Functions Comments
Involved in inflammasome formation and caspase-1 activation.
Clinically-Relevant Mutations and Pathophysiology
Disease:  Alzheimer disease
Synonyms: Alzheimer's disease [Disease Ontology: DOID:10652]
Disease Ontology: DOID:10652
OMIM: 104300
Disease:  Atherosclerosis susceptibility
Disease Ontology: DOID:1936
OMIM: 108725
Disease:  CINCA syndrome
Synonyms: Chronic infantile neurological cutaneous articular syndrome
OMIM: 607115
Orphanet: ORPHA1451
Disease:  Diabetes mellitus, noninsulin-dependent; NIDDM
Synonyms: Diabetes mellitus, Type II; T2D [OMIM: 125853]
Maturity onset diabetes
Type 2 diabetes mellitus [Disease Ontology: DOID:9352]
Disease Ontology: DOID:9352
OMIM: 125853
Disease:  Familial cold autoinflammatory syndrome 1; FCAS1
Synonyms: Familial cold urticaria [Orphanet: ORPHA47045]
OMIM: 120100
Orphanet: ORPHA47045
Disease:  Gout
Disease Ontology: DOID:13189
Disease:  Inflammatory bowel disease 1; IBD1
Synonyms: Inflammatory bowel disease [Disease Ontology: DOID:0050589]
Disease Ontology: DOID:0050589
OMIM: 266600
Disease:  Muckle-Wells syndrome
Description: Muckle–Wells syndrome (MWS) is a rare autosomal dominant disease which causes sensorineural deafness, recurrent hives, and can lead to amyloidosis.
Synonyms: CAPS2
cryopyrin-associated periodic syndrome 2
urticaria-deafness-amyloidosis syndrome
OMIM: 191900
Orphanet: ORPHA575
Click column headers to sort
Type Species Amino acid change Nucleotide change Description Reference
Missense Human R260W 778C>T 3
Missense Human A352V 1055C>T 4
Missense Human G569R 1705G>C 3
Biologically Significant Variants
Type:  Naturally occurring mutation
Species:  Human
Nucleotide change:  C/T
Amino acids:  352
SNP accession: 
References:  4
Type:  Naturally occurring mutation
Species:  Human
Nucleotide change:  G/C
Amino acids:  569
SNP accession: 
References:  3
Type:  Naturally occurring mutation
Species:  Human
Nucleotide change:  C/T
Amino acids:  260
SNP accession: 
References:  3


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1. Baldwin AG, Brough D, Freeman S. (2016) Inhibiting the Inflammasome: A Chemical Perspective. J. Med. Chem., 59 (5): 1691-710. [PMID:26422006]

2. Coll RC, Robertson AA, Chae JJ, Higgins SC, Muñoz-Planillo R, Inserra MC, Vetter I, Dungan LS, Monks BG, Stutz A et al.. (2015) A small-molecule inhibitor of the NLRP3 inflammasome for the treatment of inflammatory diseases. Nat. Med., 21 (3): 248-55. [PMID:25686105]

3. Dodé C, Le Dû N, Cuisset L, Letourneur F, Berthelot JM, Vaudour G, Meyrier A, Watts RA, Scott DG, Nicholls A et al.. (2002) New mutations of CIAS1 that are responsible for Muckle-Wells syndrome and familial cold urticaria: a novel mutation underlies both syndromes. Am. J. Hum. Genet., 70 (6): 1498-506. [PMID:11992256]

4. Hoffman HM, Mueller JL, Broide DH, Wanderer AA, Kolodner RD. (2001) Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. Nat. Genet., 29 (3): 301-5. [PMID:11687797]

5. Jiang H, He H, Chen Y, Huang W, Cheng J, Ye J, Wang A, Tao J, Wang C, Liu Q et al.. (2017) Identification of a selective and direct NLRP3 inhibitor to treat inflammatory disorders. J. Exp. Med., 214 (11): 3219-3238. [PMID:29021150]

6. Mangan MSJ, Olhava EJ, Roush WR, Seidel HM, Glick GD, Latz E. (2018) Targeting the NLRP3 inflammasome in inflammatory diseases. Nat Rev Drug Discov, 17 (9): 688. [PMID:30116046]

7. Marchetti C, Swartzwelter B, Gamboni F, Neff CP, Richter K, Azam T, Carta S, Tengesdal I, Nemkov T, D'Alessandro A et al.. (2018) OLT1177, a β-sulfonyl nitrile compound, safe in humans, inhibits the NLRP3 inflammasome and reverses the metabolic cost of inflammation. Proc. Natl. Acad. Sci. U.S.A., 115 (7): E1530-E1539. [PMID:29378952]


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How to cite this page

Clare Bryant, Tom Monie.
NOD-like receptor family: NLRP3. Last modified on 21/08/2018. Accessed on 18/01/2019. IUPHAR/BPS Guide to PHARMACOLOGY,