NLRP3

Summary

Gene Symbol: NLRP3
Description: NLR family pyrin domain containing 3
Alias: AGTAVPRL, AII, AVP, C1orf7, CIAS1, CLR1.1, DFNA34, FCAS, FCAS1, FCU, KEFH, MWS, NALP3, PYPAF1, NACHT, LRR and PYD domains-containing protein 3, NACHT domain-, leucine-rich repeat-, and PYD-containing protein 3, NACHT, LRR and PYD containing protein 3, PYRIN-containing APAF1-like protein 1, caterpiller protein 1.1, cold autoinflammatory syndrome 1 protein, cold-induced autoinflammatory syndrome 1 protein, cryopyrin, nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 3
Species: human
Products:     NLRP3

Top Publications

  1. Allam R, Lawlor K, Yu E, Mildenhall A, Moujalled D, Lewis R, et al. Mitochondrial apoptosis is dispensable for NLRP3 inflammasome activation but non-apoptotic caspase-8 is required for inflammasome priming. EMBO Rep. 2014;15:982-90 pubmed publisher
    A current paradigm proposes that mitochondrial damage is a critical determinant of NLRP3 inflammasome activation...
  2. Pontillo A, Brandão L, Guimarães R, Segat L, Athanasakis E, Crovella S. A 3'UTR SNP in NLRP3 gene is associated with susceptibility to HIV-1 infection. J Acquir Immune Defic Syndr. 2010;54:236-40 pubmed publisher
    ..We performed an association study analyzing 2 NLRP1 and NLRP3 SNPs in HIV-1 vertically infected Brazilian children (n = 135), HIV-1-infected Brazilain adults (n = 192) and HIV-..
  3. Lee H, Yuk J, Kim K, Jang J, Kang G, Park J, et al. Mycobacterium abscessus activates the NLRP3 inflammasome via Dectin-1-Syk and p62/SQSTM1. Immunol Cell Biol. 2012;90:601-10 pubmed publisher
    ..activates the nucleotide binding and oligomerization domain-like receptor family pyrin domain containing 3 (NLRP3) inflammasome via dectin-1/Syk-dependent signaling and the cytoplasmic scaffold protein p62/SQSTM1 (p62) in human ..
  4. Feldmann J, Prieur A, Quartier P, Berquin P, Certain S, Cortis E, et al. Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes. Am J Hum Genet. 2002;71:198-203 pubmed
    ..with CINCA syndrome, distinct missense mutations within the nucleotide-binding site of CIAS1, a gene encoding cryopyrin and previously shown to cause Muckle-Wells syndrome and familial cold urticaria...
  5. Barlan A, Griffin T, McGuire K, Wiethoff C. Adenovirus membrane penetration activates the NLRP3 inflammasome. J Virol. 2011;85:146-55 pubmed publisher
    ..macrophages results in rapid secretion of interleukin-1? (IL-1?) and is dependent on the inflammasome components NLRP3 and ASC and the catalytic activity of caspase-1...
  6. Vilaysane A, Chun J, Seamone M, Wang W, Chin R, Hirota S, et al. The NLRP3 inflammasome promotes renal inflammation and contributes to CKD. J Am Soc Nephrol. 2010;21:1732-44 pubmed publisher
    ..caspase-1, IL-1?, and IL-18 after unilateral ureteral obstruction (UUO) in mice, which suggested activation of the Nlrp3 inflammasome during renal injury...
  7. Villani A, Lemire M, Fortin G, Louis E, Silverberg M, Collette C, et al. Common variants in the NLRP3 region contribute to Crohn's disease susceptibility. Nat Genet. 2009;41:71-6 pubmed publisher
    ..located in a predicted regulatory region on chromosome 1q44 downstream of NLRP3 (previously known as CIAS1 and NALP3) that are associated with Crohn's disease...
  8. McElvania TeKippe E, Allen I, Hulseberg P, Sullivan J, McCann J, Sandor M, et al. Granuloma formation and host defense in chronic Mycobacterium tuberculosis infection requires PYCARD/ASC but not NLRP3 or caspase-1. PLoS ONE. 2010;5:e12320 pubmed publisher
    ..This paper addressed the role of NLRP3 (NALP3), its adaptor protein PYCARD (ASC), and caspase-1 during infection with Mycobacterium tuberculosis (Mtb)...
  9. Neven B, Callebaut I, Prieur A, Feldmann J, Bodemer C, Lepore L, et al. Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU. Blood. 2004;103:2809-15 pubmed
    ..in the CIAS1 gene, which encodes a member of the NALP (NACHT-, LRR-, and PYD-containing proteins) family, the cryopyrin/NALP3/PYPAF1 protein, expressed primarily in phagocytic cells, were recently found to be associated with a ..

More Information

Publications94

  1. Mishra B, Moura Alves P, Sonawane A, Hacohen N, Griffiths G, Moita L, et al. Mycobacterium tuberculosis protein ESAT-6 is a potent activator of the NLRP3/ASC inflammasome. Cell Microbiol. 2010;12:1046-63 pubmed publisher
    ..Most importantly, NLRP3, ASC and caspase-1 form an infection-inducible inflammasome complex that is essential for IL-1beta secretion...
  2. Lamkanfi M, Mueller J, Vitari A, Misaghi S, Fedorova A, Deshayes K, et al. Glyburide inhibits the Cryopyrin/Nalp3 inflammasome. J Cell Biol. 2009;187:61-70 pubmed publisher
    ..b>Cryopyrin/NALP3/NLRP3 is an essential component of inflammasomes triggered by microbial ligands, danger-associated ..
  3. Kummer J, Broekhuizen R, Everett H, Agostini L, Kuijk L, Martinon F, et al. Inflammasome components NALP 1 and 3 show distinct but separate expression profiles in human tissues suggesting a site-specific role in the inflammatory response. J Histochem Cytochem. 2007;55:443-52 pubmed
    Several autoinflammatory disorders such as Muckle-Wells syndrome are characterized by mutations in the NALP3/cryopyrin gene...
  4. Kinoshita T, Wang Y, Hasegawa M, Imamura R, Suda T. PYPAF3, a PYRIN-containing APAF-1-like protein, is a feedback regulator of caspase-1-dependent interleukin-1beta secretion. J Biol Chem. 2005;280:21720-5 pubmed
    ..Among the members of this family, PYPAF1, PYPAF5, PYPAF7, and NALP1 have been shown to induce caspase-1-dependent interleukin-1beta secretion and NF-kappaB ..
  5. Yu J, Wu J, Zhang Z, Datta P, Ibrahimi I, Taniguchi S, et al. Cryopyrin and pyrin activate caspase-1, but not NF-kappaB, via ASC oligomerization. Cell Death Differ. 2006;13:236-49 pubmed
    Mutations in cryopyrin and pyrin proteins are responsible for several autoinflammatory disorders in humans, suggesting that these proteins play important roles in regulating inflammation...
  6. Aksentijevich I, Putnam C, Remmers E, Mueller J, Le J, Kolodner R, et al. The clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North American patients and a new cryopyrin model. Arthritis Rheum. 2007;56:1273-1285 pubmed publisher
    ..are a group of rare autoinflammatory disorders that are caused by mutations in CIAS1, encoding the cryopyrin protein...
  7. Cummings J, Cooney R, Clarke G, Beckly J, Geremia A, Pathan S, et al. The genetics of NOD-like receptors in Crohn's disease. Tissue Antigens. 2010;76:48-56 pubmed publisher
    ..Four single nucleotide polymorphisms (SNPs) in NLRP3 had P values < 0.05 and are in high linkage disequilibrium (LD) with each other (r(2) > 0...
  8. Martinon F, Tschopp J. NLRs join TLRs as innate sensors of pathogens. Trends Immunol. 2005;26:447-54 pubmed
  9. Wang W, Wang X, Chun J, Vilaysane A, Clark S, French G, et al. Inflammasome-independent NLRP3 augments TGF-? signaling in kidney epithelium. J Immunol. 2013;190:1239-49 pubmed publisher
    ..We recently demonstrated that the NOD-like receptor, pyrin domain containing-3 (NLRP3) contributes to renal inflammation, injury, and fibrosis following unilateral ureteric obstruction in mice...
  10. Heneka M, Kummer M, Stutz A, Delekate A, Schwartz S, Vieira Saecker A, et al. NLRP3 is activated in Alzheimer's disease and contributes to pathology in APP/PS1 mice. Nature. 2013;493:674-8 pubmed publisher
    ..Indeed, amyloid-? activation of the NLRP3 inflammasome in microglia is fundamental for interleukin-1? maturation and subsequent inflammatory events...
  11. Lu A, Magupalli V, Ruan J, Yin Q, Atianand M, Vos M, et al. Unified polymerization mechanism for the assembly of ASC-dependent inflammasomes. Cell. 2014;156:1193-1206 pubmed publisher
    ..AIM2 and NLRP3 are representative sensor proteins in two major families of inflammasomes...
  12. Lamkanfi M, Dixit V. Inflammasomes: guardians of cytosolic sanctity. Immunol Rev. 2009;227:95-105 pubmed publisher
    ..factor (IPAF), NALP1b (NACHT domain-, leucine-rich repeat-, and PYD-containing protein 1b), and cryopyrin/NALP3 assemble caspase-1-activating inflammasomes in a stimulus-dependent manner...
  13. Belibasakis G, Johansson A. Aggregatibacter actinomycetemcomitans targets NLRP3 and NLRP6 inflammasome expression in human mononuclear leukocytes. Cytokine. 2012;59:124-30 pubmed publisher
    ..However, NLRP3 was up-regulated, while NLRP6 was down-regulated...
  14. van Bruggen R, Koker M, Jansen M, van Houdt M, Roos D, Kuijpers T, et al. Human NLRP3 inflammasome activation is Nox1-4 independent. Blood. 2010;115:5398-400 pubmed publisher
    The NLRP3 inflammasome can be activated by pathogen-associated molecular patterns or endogenous danger-associated molecular patterns...
  15. Wang W, Stassen F, Surcel H, Ohman H, Tiitinen A, Paavonen J, et al. Analyses of polymorphisms in the inflammasome-associated NLRP3 and miRNA-146A genes in the susceptibility to and tubal pathology of Chlamydia trachomatis infection. Drugs Today (Barc). 2009;45 Suppl B:95-103 pubmed
    ..Single nucleotide polymorphisms (SNPs) in NLRP3 are associated with decreased NLRP3 expression and hypoproduction of interleukin (IL)-1beta...
  16. Dode C, Le Dû N, Cuisset L, Letourneur F, Berthelot J, Vaudour G, et al. 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. 2002;70:1498-506 pubmed
    Mutations of CIAS1 have recently been shown to underlie familial cold urticaria (FCU) and Muckle-Wells syndrome (MWS), in three families and one family, respectively...
  17. Hitomi Y, Ebisawa M, Tomikawa M, Imai T, Komata T, Hirota T, et al. Associations of functional NLRP3 polymorphisms with susceptibility to food-induced anaphylaxis and aspirin-induced asthma. J Allergy Clin Immunol. 2009;124:779-85.e6 pubmed publisher
    NLR family, pyrin domain containing 3 (NLRP3), controls the activity of inflammatory caspase-1 by forming inflammasomes, which leads to cleavage of the procytokines IL-1beta and IL-18...
  18. Kastbom A, Verma D, Eriksson P, Skogh T, Wingren G, Soderkvist P. Genetic variation in proteins of the cryopyrin inflammasome influences susceptibility and severity of rheumatoid arthritis (the Swedish TIRA project). Rheumatology (Oxford). 2008;47:415-7 pubmed publisher
    ..We investigated whether two polymorphisms in the genes encoding cryopyrin (CIAS1) and TUCAN (CARD8) influence susceptibility and disease course in RA...
  19. Hoffman H, Mueller J, Broide D, Wanderer A, Kolodner R. Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. Nat Genet. 2001;29:301-5 pubmed
    ..This gene, called CIAS1, is expressed in peripheral blood leukocytes and encodes a protein with a pyrin domain, a nucleotide-binding site (..
  20. Nakamura Y, Kambe N, Saito M, Nishikomori R, Nishikomiri R, Kim Y, et al. Mast cells mediate neutrophil recruitment and vascular leakage through the NLRP3 inflammasome in histamine-independent urticaria. J Exp Med. 2009;206:1037-46 pubmed publisher
    Urticarial rash observed in cryopyrin-associated periodic syndrome (CAPS) caused by nucleotide-binding oligomerization domain-leucine-rich repeats containing pyrin domain 3 (NLRP3) mutations is effectively suppressed by anti-interleukin (..
  21. Agostini L, Martinon F, Burns K, McDermott M, Hawkins P, Tschopp J. NALP3 forms an IL-1beta-processing inflammasome with increased activity in Muckle-Wells autoinflammatory disorder. Immunity. 2004;20:319-25 pubmed
    Mutations within the NALP3/cryopyrin/CIAS1 gene are responsible for three autoinflammatory disorders: Muckle-Wells syndrome, familial cold autoinflammatory syndrome, and CINCA...
  22. Schoultz I, Verma D, Halfvarsson J, Torkvist L, Fredrikson M, Sjoqvist U, et al. Combined polymorphisms in genes encoding the inflammasome components NALP3 and CARD8 confer susceptibility to Crohn's disease in Swedish men. Am J Gastroenterol. 2009;104:1180-8 pubmed publisher
    ..Production of mature IL-1beta is dependent on a caspase-1-activating protein complex called the NALP3 inflammasome, composed of NALP3, ASC, and CARD8...
  23. Petrilli V, Papin S, Dostert C, Mayor A, Martinon F, Tschopp J. Activation of the NALP3 inflammasome is triggered by low intracellular potassium concentration. Cell Death Differ. 2007;14:1583-9 pubmed
    ..The inflammasomes harboring the NLR members NALP1, NALP3 and IPAF have been best characterized...
  24. Dowds T, Masumoto J, Chen F, Ogura Y, Inohara N, Nunez G. Regulation of cryopyrin/Pypaf1 signaling by pyrin, the familial Mediterranean fever gene product. Biochem Biophys Res Commun. 2003;302:575-80 pubmed
    b>Cryopyrin, a member of the Nod protein family mutated in familial cold urticaria and Muckle-Wells syndrome, has been recently implicated in inflammation...
  25. Omi T, Kumada M, Kamesaki T, Okuda H, Munkhtulga L, Yanagisawa Y, et al. An intronic variable number of tandem repeat polymorphisms of the cold-induced autoinflammatory syndrome 1 (CIAS1) gene modifies gene expression and is associated with essential hypertension. Eur J Hum Genet. 2006;14:1295-305 pubmed
    Cold-induced autoinflammatory syndrome 1 (CIAS1) gene is a member of the NALP subfamily of the CATERPILLER protein family that is expressed predominantly in peripheral blood leukocytes, which is to regulate apoptosis or inflammation ..
  26. Segovia J, Sabbah A, Mgbemena V, Tsai S, Chang T, Berton M, et al. TLR2/MyD88/NF-?B pathway, reactive oxygen species, potassium efflux activates NLRP3/ASC inflammasome during respiratory syncytial virus infection. PLoS ONE. 2012;7:e29695 pubmed publisher
    ..Here in, we have characterized the mechanism of IL-1? secretion following RSV infection. Our study revealed that NLRP3/ASC inflammasome activation is crucial for IL-1? production during RSV infection...
  27. Tarallo V, Hirano Y, Gelfand B, Dridi S, Kerur N, Kim Y, et al. DICER1 loss and Alu RNA induce age-related macular degeneration via the NLRP3 inflammasome and MyD88. Cell. 2012;149:847-59 pubmed publisher
    ..Here we show that DICER1 deficit or Alu RNA exposure activates the NLRP3 inflammasome and triggers TLR-independent MyD88 signaling via IL18 in the RPE...
  28. Zhang A, Zeng L, Gu W, Zhang L, Zhou J, Jiang D, et al. Clinical relevance of single nucleotide polymorphisms within the entire NLRP3 gene in patients with major blunt trauma. Crit Care. 2011;15:R280 pubmed publisher
    ..receptor (NLR) family has been recognized as comprising intracellular pattern recognition receptors in which NLRP3 (NLR family, pyrin domain containing 3) plays an important role in the initiation of host immune inflammatory ..
  29. Fujisawa A, Kambe N, Saito M, Nishikomori R, Tanizaki H, Kanazawa N, et al. Disease-associated mutations in CIAS1 induce cathepsin B-dependent rapid cell death of human THP-1 monocytic cells. Blood. 2007;109:2903-11 pubmed
    ..CIAS1 encodes cryopyrin, a protein that localizes to the cytosol and functions as pattern recognition receptor...
  30. Yang C, Huang S, Chiang B. Association of NLRP3 and CARD8 genetic polymorphisms with juvenile idiopathic arthritis in a Taiwanese population. Scand J Rheumatol. 2014;43:146-52 pubmed publisher
    ..We aimed to determine whether genetic polymorphisms of the NLRP3 inflammasome components confer risk for oligoarticular and polyarticular JIA in a Taiwanese population...
  31. Saïd Sadier N, Padilla E, Langsley G, Ojcius D. Aspergillus fumigatus stimulates the NLRP3 inflammasome through a pathway requiring ROS production and the Syk tyrosine kinase. PLoS ONE. 2010;5:e10008 pubmed publisher
    ..Our results show that in contrast to conidia, hyphal fragments induce NLRP3 inflammasome assembly, caspase-1 activation and IL-1beta release from a human monocyte cell line...
  32. Anderson J, Mueller J, Misaghi A, Anderson S, Sivagnanam M, Kolodner R, et al. Initial description of the human NLRP3 promoter. Genes Immun. 2008;9:721-6 pubmed publisher
    ..in a continuum of related inflammatory disorders, known as cryopyrinopathies since NLRP3 codes for the protein cryopyrin. Approximately 40% of patients with classic presentation lack mutations in the coding region of NLRP3 suggesting ..
  33. Rajamäki K, Lappalainen J, Öörni K, Välimäki E, Matikainen S, Kovanen P, et al. Cholesterol crystals activate the NLRP3 inflammasome in human macrophages: a novel link between cholesterol metabolism and inflammation. PLoS ONE. 2010;5:e11765 pubmed publisher
    ..The most intensively studied inflammasome, NLRP3 inflammasome, is activated by diverse substances, including crystalline and particulate materials...
  34. Verma D, Lerm M, Blomgran Julinder R, Eriksson P, Söderkvist P, Särndahl E. Gene polymorphisms in the NALP3 inflammasome are associated with interleukin-1 production and severe inflammation: relation to common inflammatory diseases?. Arthritis Rheum. 2008;58:888-94 pubmed publisher
    b>NALP3, ASC, and TUCAN are components of the NALP3 inflammasome, which triggers caspase 1-mediated interleukin-1beta (IL-1beta) release...
  35. Hornung V, Ablasser A, Charrel Dennis M, Bauernfeind F, Horvath G, Caffrey D, et al. AIM2 recognizes cytosolic dsDNA and forms a caspase-1-activating inflammasome with ASC. Nature. 2009;458:514-8 pubmed publisher
    ..Collectively, these observations identify AIM2 as a new receptor for cytoplasmic DNA, which forms an inflammasome with the ligand and ASC to activate caspase-1. ..
  36. Craven R, Gao X, Allen I, Gris D, Bubeck Wardenburg J, McElvania TeKippe E, et al. Staphylococcus aureus alpha-hemolysin activates the NLRP3-inflammasome in human and mouse monocytic cells. PLoS ONE. 2009;4:e7446 pubmed publisher
    ..the Nucleotide-binding domain and leucine-rich repeat containing gene family, pyrin domain containing 3 protein (NLRP3)-inflammasome, a host inflammatory signaling complex involved in responses to pathogens and endogenous danger ..
  37. Li Y, Xu S, Jiang B, Cohen R, Zang M. Activation of sterol regulatory element binding protein and NLRP3 inflammasome in atherosclerotic lesion development in diabetic pigs. PLoS ONE. 2013;8:e67532 pubmed publisher
    ..Chronic inflammation and activation of NLRP3 inflammasome have been implicated in atherosclerosis and fatty liver disease...
  38. Aksentijevich I, Nowak M, Mallah M, Chae J, Watford W, Hofmann S, et al. De novo CIAS1 mutations, cytokine activation, and evidence for genetic heterogeneity in patients with neonatal-onset multisystem inflammatory disease (NOMID): a new member of the expanding family of pyrin-associated autoinflammatory diseases. Arthritis Rheum. 2002;46:3340-8 pubmed
    ..We investigated whether patients with this disorder have mutations in CIAS1, the gene which causes Muckle-Wells syndrome and familial cold autoinflammatory syndrome, two dominantly inherited ..
  39. O Connor W, Harton J, Zhu X, Linhoff M, Ting J. Cutting edge: CIAS1/cryopyrin/PYPAF1/NALP3/CATERPILLER 1.1 is an inducible inflammatory mediator with NF-kappa B suppressive properties. J Immunol. 2003;171:6329-33 pubmed
    ..These data suggest CIAS1/cryopyrin may act as a key regulator of inflammation, induced to dampen NF-kappaB-dependent proinflammatory signals.
  40. Damiano J, Oliveira V, Welsh K, Reed J. Heterotypic interactions among NACHT domains: implications for regulation of innate immune responses. Biochem J. 2004;381:213-9 pubmed
    ..to bind all NACHT domains that were tested, including the NACHT domains from CLAN itself, Nod1, Nod2, cryopyrin, NAC, PAN2 [PAAD [pyrin, AIM (absent-in-melanoma), ASC (apoptosis-associated speck-like protein containing a ..
  41. Saito M, Nishikomori R, Kambe N, Fujisawa A, Tanizaki H, Takeichi K, et al. Disease-associated CIAS1 mutations induce monocyte death, revealing low-level mosaicism in mutation-negative cryopyrin-associated periodic syndrome patients. Blood. 2008;111:2132-41 pubmed
    b>Cryopyrin-associated periodic syndrome (CAPS) is a spectrum of systemic autoinflammatory disorders in which the majority of patients have mutations in the cold-induced autoinflammatory syndrome (CIAS)1 gene...
  42. Subramanian N, Natarajan K, Clatworthy M, Wang Z, Germain R. The adaptor MAVS promotes NLRP3 mitochondrial localization and inflammasome activation. Cell. 2013;153:348-61 pubmed publisher
    b>NLRP3 is a key component of the macromolecular signaling complex called the inflammasome that promotes caspase 1-dependent production of IL-1β...
  43. Ben Hamad M, Cornelis F, Marzouk S, Chabchoub G, Bahloul Z, Rebai A, et al. Association study of CARD8 (p.C10X) and NLRP3 (p.Q705K) variants with rheumatoid arthritis in French and Tunisian populations. Int J Immunogenet. 2012;39:131-6 pubmed publisher
    ..domain 8 (CARD8) and nucleotide-binding oligomerization domain, leucine-rich repeat and pyrin domain containing 3 (NLRP3) polymorphisms with rheumatoid arthritis (RA) in Tunisian and French populations. CARD8 (c...
  44. Gattorno M, Tassi S, Carta S, Delfino L, Ferlito F, Pelagatti M, et al. Pattern of interleukin-1beta secretion in response to lipopolysaccharide and ATP before and after interleukin-1 blockade in patients with CIAS1 mutations. Arthritis Rheum. 2007;56:3138-48 pubmed
    ..This indicates that cryopyrin is the direct target of ATP and that the mutations release the protein from the requirement of ATP for ..
  45. Duncan J, Gao X, Huang M, O Connor B, Thomas C, Willingham S, et al. Neisseria gonorrhoeae activates the proteinase cathepsin B to mediate the signaling activities of the NLRP3 and ASC-containing inflammasome. J Immunol. 2009;182:6460-9 pubmed publisher
    ..We demonstrate that NLRP3 (cryopyrin, NALP3) is the primary NLR required for IL-1beta/IL-18 secretion in response to N. gonorrhoeae in monocytes...
  46. Pedra J, CASSEL S, Sutterwala F. Sensing pathogens and danger signals by the inflammasome. Curr Opin Immunol. 2009;21:10-6 pubmed publisher
    ..Dysregulation of the inflammasome has also been linked to a number of autoinflammatory and autoimmune disorders. Recent advances in the inflammasome field will be discussed in this review. ..
  47. Lewis G, Massey D, Zhang H, Bredin F, Tremelling M, Lee J, et al. Genetic association between NLRP3 variants and Crohn's disease does not replicate in a large UK panel. Inflamm Bowel Dis. 2011;17:1387-91 pubmed publisher
    NLRP3 (formerly known as CIAS1 or NALP3) encodes a key component of the inflammasome and is a strong candidate gene for Crohn's disease (CD) susceptibility...
  48. Zhou R, Yazdi A, Menu P, Tschopp J. A role for mitochondria in NLRP3 inflammasome activation. Nature. 2011;469:221-5 pubmed publisher
    An inflammatory response initiated by the NLRP3 inflammasome is triggered by a variety of situations of host 'danger', including infection and metabolic dysregulation...
  49. Blomgran R, Patcha Brodin V, Verma D, Bergström I, Söderkvist P, Sjöwall C, et al. Common genetic variations in the NALP3 inflammasome are associated with delayed apoptosis of human neutrophils. PLoS ONE. 2012;7:e31326 pubmed publisher
    ..NLR mutations are associated with auto-inflammatory syndromes, and our previous data propose NLRP3 (Q705K)/CARD-8 (C10X) polymorphisms to contribute to increased risk and severity of inflammatory disease by acting ..
  50. Pontillo A, Brandao L, Guimaraes R, Segat L, Araujo J, Crovella S. Two SNPs in NLRP3 gene are involved in the predisposition to type-1 diabetes and celiac disease in a pediatric population from northeast Brazil. Autoimmunity. 2010;43:583-9 pubmed publisher
    Recent findings provide evidence of the critical role of innate immunity NALP1/NLRP1 and NALP3/NLRP3/CIAS1 genes in inflammatory diseases, and also in the predisposition to autoimmune disorders...
  51. Srinivasula S, Poyet J, Razmara M, Datta P, Zhang Z, Alnemri E. The PYRIN-CARD protein ASC is an activating adaptor for caspase-1. J Biol Chem. 2002;277:21119-22 pubmed
    ..These results indicate that ASC is involved in the caspase-1 signaling pathway by mediating the assembly of a caspase-1-inflammasome signaling complex in response to pro-inflammatory cytokine stimulation. ..
  52. Verma D, Särndahl E, Andersson H, Eriksson P, Fredrikson M, Jönsson J, et al. The Q705K polymorphism in NLRP3 is a gain-of-function alteration leading to excessive interleukin-1? and IL-18 production. PLoS ONE. 2012;7:e34977 pubmed publisher
    The Q705K polymorphism in NLRP3 has been implicated in several chronic inflammatory diseases. In this study we determine the functional role of this commonly occurring polymorphism using an in-vitro system...
  53. Roberts R, Topless R, Phipps Green A, Gearry R, Barclay M, Merriman T. Evidence of interaction of CARD8 rs2043211 with NALP3 rs35829419 in Crohn's disease. Genes Immun. 2010;11:351-6 pubmed publisher
    The location of CARD8 within an inflammatory bowel disease (IBD) locus and its role in the NALP3 inflammasome and as a nuclear factor (NF)kappaB inhibitor make it an attractive candidate risk gene for IBD...
  54. Jiang Y, Wang M, Huang K, Zhang Z, Shao N, Zhang Y, et al. Oxidized low-density lipoprotein induces secretion of interleukin-1? by macrophages via reactive oxygen species-dependent NLRP3 inflammasome activation. Biochem Biophys Res Commun. 2012;425:121-6 pubmed publisher
    ..Here we show that the phagocytosis of ox-LDL can induce human macrophages to secrete IL-1? by activating the NLRP3 inflammasome, and we further show that the activation of the NLRP3 inflammasome is dependent on the generation of ..
  55. Pontillo A, Vendramin A, Catamo E, Fabris A, Crovella S. The missense variation Q705K in CIAS1/NALP3/NLRP3 gene and an NLRP1 haplotype are associated with celiac disease. Am J Gastroenterol. 2011;106:539-44 pubmed publisher
    ..Variations in the NALP1/NLRP1 and NALP3/NLRP3 genes have been reported to confer risk for several autoimmune conditions...
  56. Pontillo A, Oshiro T, Girardelli M, Kamada A, Crovella S, Duarte A. Polymorphisms in inflammasome' genes and susceptibility to HIV-1 infection. J Acquir Immune Defic Syndr. 2012;59:121-5 pubmed publisher
    ..These findings supported the previously hypothesized involvement of NALP3-inflammasome in HIV-1 pathogenesis, underlining once more the key role of inflammation and innate immunity in the ..
  57. Day T, Ramanan A, Hinks A, Lamb R, Packham J, Wise C, et al. Autoinflammatory genes and susceptibility to psoriatic juvenile idiopathic arthritis. Arthritis Rheum. 2008;58:2142-6 pubmed publisher
    To investigate the association of NLRP3, NOD2, MEFV, and PSTPIP1, genes that cause 4 of the autoinflammatory hereditary periodic fever syndromes (HPFS), with juvenile idiopathic arthritis (JIA)...
  58. Meixenberger K, Pache F, Eitel J, Schmeck B, Hippenstiel S, Slevogt H, et al. Listeria monocytogenes-infected human peripheral blood mononuclear cells produce IL-1beta, depending on listeriolysin O and NLRP3. J Immunol. 2010;184:922-30 pubmed publisher
    Different NOD-like receptors, including NLRP1, NLRP3, and NLRC4, as well as the recently identified HIN-200 protein, AIM2, form multiprotein complexes called inflammasomes, which mediate caspase-1-dependent processing of pro-IL-1beta...
  59. Willingham S, Bergstralh D, O Connor W, Morrison A, Taxman D, Duncan J, et al. Microbial pathogen-induced necrotic cell death mediated by the inflammasome components CIAS1/cryopyrin/NLRP3 and ASC. Cell Host Microbe. 2007;2:147-59 pubmed
    b>Cryopyrin (CIAS1, NLRP3) and ASC are components of the inflammasome, a multiprotein complex required for caspase-1 activation and cytokine IL-1beta production...
  60. Manji G, Wang L, Geddes B, Brown M, Merriam S, Al Garawi A, et al. PYPAF1, a PYRIN-containing Apaf1-like protein that assembles with ASC and regulates activation of NF-kappa B. J Biol Chem. 2002;277:11570-5 pubmed
    ..We report here that PYPAF1 (PYRIN-containing Apaf1-like protein 1) is a novel PYRIN-containing signaling protein that belongs to the ..
  61. Roberts R, Van Rij A, Phillips L, Young S, McCormick S, Merriman T, et al. Interaction of the inflammasome genes CARD8 and NLRP3 in abdominal aortic aneurysms. Atherosclerosis. 2011;218:123-6 pubmed publisher
    ..have been shown to cause inflammation, and ultimately atherosclerotic lesions through the activation of the NLRP3 inflammasome...
  62. Ungerbäck J, Belenki D, Jawad ul Hassan A, Fredrikson M, Fransen K, Elander N, et al. Genetic variation and alterations of genes involved in NF?B/TNFAIP3- and NLRP3-inflammasome signaling affect susceptibility and outcome of colorectal cancer. Carcinogenesis. 2012;33:2126-34 pubmed publisher
    ..Heterozygous and polymorphic TNFAIP3 (rs6920220), heterozygous NLRP3 (Q705K) and polymorphic NF?B -94 ATTG ins/del genotypes were found to be associated with poorer survival in ..
  63. Hirota J, Hirota S, Warner S, Stefanowicz D, Shaheen F, Beck P, et al. The airway epithelium nucleotide-binding domain and leucine-rich repeat protein 3 inflammasome is activated by urban particulate matter. J Allergy Clin Immunol. 2012;129:1116-25.e6 pubmed publisher
    ..The airway epithelium is the first line of defense against inhaled insults and therefore must be capable of coordinating appropriate inflammatory and immune responses...
  64. Aganna E, Martinon F, Hawkins P, Ross J, Swan D, Booth D, et al. Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis. Arthritis Rheum. 2002;46:2445-52 pubmed
    ..Mutations in the candidate gene NALP3, which has also been named CIAS1 and PYPAF1, were sought in the study families, in a British/Spanish patient with apparent sporadic MWS, and in matched ..
  65. Simard J, Cesaro A, Chapeton Montes J, Tardif M, Antoine F, Girard D, et al. S100A8 and S100A9 induce cytokine expression and regulate the NLRP3 inflammasome via ROS-dependent activation of NF-?B(1.). PLoS ONE. 2013;8:e72138 pubmed publisher
    ..S100A8- and S100A9-mediated activation of NF-?B, the NLR family, pyrin domain-containing 3 (NLRP3) protein, and pro-IL-1? expression was dependent on the generation of reactive oxygen species...
  66. Rossol M, Pierer M, Raulien N, Quandt D, Meusch U, Rothe K, et al. Extracellular Ca2+ is a danger signal activating the NLRP3 inflammasome through G protein-coupled calcium sensing receptors. Nat Commun. 2012;3:1329 pubmed publisher
    Activation of the NLRP3 inflammasome enables monocytes and macrophages to release high levels of interleukin-1? during inflammatory responses...
  67. Abdul Sater A, Koo E, Hacker G, Ojcius D. Inflammasome-dependent caspase-1 activation in cervical epithelial cells stimulates growth of the intracellular pathogen Chlamydia trachomatis. J Biol Chem. 2009;284:26789-96 pubmed publisher
    ..trachomatis leads to activation of caspase-1, through a process requiring the NOD-like receptor family member NLRP3 and the inflammasome adaptor protein ASC...
  68. Niemi K, Teirilä L, Lappalainen J, Rajamäki K, Baumann M, Öörni K, et al. Serum amyloid A activates the NLRP3 inflammasome via P2X7 receptor and a cathepsin B-sensitive pathway. J Immunol. 2011;186:6119-28 pubmed publisher
    ..b>NLRP3 inflammasome consists of the NLRP3 receptor and the adaptor protein apoptosis-associated speck-like protein ..
  69. Xiao H, Lu M, Lin T, Chen Z, Chen G, Wang W, et al. Sterol regulatory element binding protein 2 activation of NLRP3 inflammasome in endothelium mediates hemodynamic-induced atherosclerosis susceptibility. Circulation. 2013;128:632-42 pubmed publisher
    ..Here, we explored whether disturbed flow patterns activate an innate immune response to form the NLRP3 inflammasome scaffold in vascular endothelial cells via sterol regulatory element binding protein 2 (SREBP2)...
  70. Vajjhala P, Mirams R, Hill J. Multiple binding sites on the pyrin domain of ASC protein allow self-association and interaction with NLRP3 protein. J Biol Chem. 2012;287:41732-43 pubmed publisher
    ..immune response to pathogens or cellular stress is activation of members of the NOD-like receptor family, such as NLRP3, to assemble caspase-1-activating inflammasome complexes...
  71. Dehghan A, Yang Q, Peters A, Basu S, Bis J, Rudnicka A, et al. Association of novel genetic Loci with circulating fibrinogen levels: a genome-wide association study in 6 population-based cohorts. Circ Cardiovasc Genet. 2009;2:125-33 pubmed publisher
    ..9 x 10(-10)), and rs1539019 on the NLR family pyrin domain containing 3 isoforms (NLRP3) gene (P=1.04 x 10(-8))...
  72. Cuisset L, Jeru I, Dumont B, Fabre A, Cochet E, Le Bozec J, et al. Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France. Ann Rheum Dis. 2011;70:495-9 pubmed publisher
    b>Cryopyrin-associated periodic syndromes (CAPS) consist of a continuum of autoinflammatory diseases caused by a defect in interleukin 1? regulation...
  73. Hise A, Tomalka J, Ganesan S, Patel K, Hall B, Brown G, et al. An essential role for the NLRP3 inflammasome in host defense against the human fungal pathogen Candida albicans. Cell Host Microbe. 2009;5:487-97 pubmed publisher
    ..The known fungal pattern recognition receptors TLR2 and Dectin-1 regulate IL-1beta gene transcription, whereas the NLRP3-containing proinflammatory multiprotein complex, the NLRP3 inflammasome, controls caspase-1-mediated cleavage of ..
  74. Lee H, Kim J, Kim H, Shong M, Ku B, Jo E. Upregulated NLRP3 inflammasome activation in patients with type 2 diabetes. Diabetes. 2013;62:194-204 pubmed publisher
    ..on the roles of the nucleotide binding and oligomerization domain-like receptor family pyrin domain-containing 3 (NLRP3) inflammasome in the pathogenesis of type 2 diabetes, little is known about the ex vivo profile of inflammasome ..
  75. Kinoshita T, Kondoh C, Hasegawa M, Imamura R, Suda T. Fas-associated factor 1 is a negative regulator of PYRIN-containing Apaf-1-like protein 1. Int Immunol. 2006;18:1701-6 pubmed
    ..In these assays, FAF1 interacted strongly with PYPAF1, PYPAF3 and PYPAF7, moderately with PYPAF2 and PYNOD but not at all with the pyrin domains of pyrin or the adaptor ..
  76. Vandanmagsar B, Youm Y, Ravussin A, Galgani J, Stadler K, Mynatt R, et al. The NLRP3 inflammasome instigates obesity-induced inflammation and insulin resistance. Nat Med. 2011;17:179-88 pubmed publisher
    ..domain, leucine-rich-containing family, pyrin domain-containing-3 (Nlrp3, but also known as Nalp3 or cryopyrin) inflammasome are implicated in recognizing certain nonmicrobial originated 'danger signals' leading to caspase-..
  77. Bauernfeind F, Horvath G, Stutz A, Alnemri E, MacDonald K, Speert D, et al. Cutting edge: NF-kappaB activating pattern recognition and cytokine receptors license NLRP3 inflammasome activation by regulating NLRP3 expression. J Immunol. 2009;183:787-91 pubmed publisher
    ..The NLRP3 inflammasome, however, assembles in response to extracellular ATP, pore-forming toxins, or crystals only in the ..
  78. Lev Sagie A, Prus D, Linhares I, Lavy Y, Ledger W, Witkin S. Polymorphism in a gene coding for the inflammasome component NALP3 and recurrent vulvovaginal candidiasis in women with vulvar vestibulitis syndrome. Am J Obstet Gynecol. 2009;200:303.e1-6 pubmed publisher
    Patients with vulvar vestibulitis syndrome (VVS) and control subjects were tested for a polymorphism in the gene coding for the NALP3 component of inflammasomes, cytoplasmic structures regulating interleukin (IL)-1beta production.
  79. Kankkunen P, Teirilä L, Rintahaka J, Alenius H, Wolff H, Matikainen S. (1,3)-beta-glucans activate both dectin-1 and NLRP3 inflammasome in human macrophages. J Immunol. 2010;184:6335-42 pubmed publisher
    ..RNA interference-mediated gene knockdown experiments demonstrated that cytoplasmic NLRP3 inflammasome is essential for beta-glucan-induced IL-1beta secretion...
  80. Verma D, Eriksson P, Sahdo B, Persson A, Ejdeback M, Särndahl E, et al. Two adult siblings with atypical cryopyrin-associated periodic syndrome due to a novel M299V mutation in NLRP3. Arthritis Rheum. 2010;62:2138-43 pubmed publisher
    ..Mutations in the gene encoding NALP3 (NLRP3) underlie the cryopyrin-associated periodic syndrome (CAPS)...
  81. Shigeoka A, Mueller J, Kambo A, Mathison J, King A, Hall W, et al. An inflammasome-independent role for epithelial-expressed Nlrp3 in renal ischemia-reperfusion injury. J Immunol. 2010;185:6277-85 pubmed publisher
    ..One cytoplasmic receptor complex, the Nlrp3 inflammasome, responds to an extensive array of molecules associated with cellular stress...
  82. Sahdo B, Fransen K, Asfaw Idosa B, Eriksson P, Söderquist B, Kelly A, et al. Cytokine profile in a cohort of healthy blood donors carrying polymorphisms in genes encoding the NLRP3 inflammasome. PLoS ONE. 2013;8:e75457 pubmed publisher
    The NLRP3 inflammasome has been recognized as one of the key components of the innate immunity by sensing a diversity of insults...
  83. Atianand M, Duffy E, Shah A, Kar S, Malik M, Harton J. Francisella tularensis reveals a disparity between human and mouse NLRP3 inflammasome activation. J Biol Chem. 2011;286:39033-42 pubmed publisher
    ..Francisella infection leads to seemingly exclusive activation of the Aim2 inflammasome with no apparent role for Nlrp3. Here we examine the IL-1? response of human cells to Francisella infection...
  84. Park S, Juliana C, Hong S, Datta P, Hwang I, Fernandes Alnemri T, et al. The mitochondrial antiviral protein MAVS associates with NLRP3 and regulates its inflammasome activity. J Immunol. 2013;191:4358-66 pubmed publisher
    b>NLRP3 assembles an inflammasome complex that activates caspase-1 upon sensing various danger signals derived from pathogenic infection, tissue damage, and environmental toxins...
  85. Dowds T, Masumoto J, Zhu L, Inohara N, Nunez G. Cryopyrin-induced interleukin 1beta secretion in monocytic cells: enhanced activity of disease-associated mutants and requirement for ASC. J Biol Chem. 2004;279:21924-8 pubmed
    ..disorders are associated with missense mutations within the nucleotide-binding oligomerization domain of cryopyrin. The mechanism by which cryopyrin mutations cause inflammatory disease remains elusive...