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NLR Family, Pyrin Domain Containing Protein 3 (NLRP3)

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AVP; AGTAVPRL; AII; AII/AVP; C1orf7; CIAS1; CLR1.1; FCAS; FCU; MWS; NALP3; PYPAF1; Cryopyrin; Cold Induced Autoinflammatory Syndrome 1; Caterpiller-Like Receptor 1.1

NLR Family, Pyrin Domain Containing Protein 3 (NLRP3)
CCC K115
HGNC 16400
MGI 2653833
PubMed 23633957
RGD 1308314
UniProt Q96P20
Wiki NALP3
NLRP3 encodes a pyrin-like protein which contains a pyrin domain, a nucleotide-binding site (NBS) domain, and a leucine-rich repeat (LRR) motif. This protein interacts with apoptosis-associated speck-like protein containing a CARD. Proteins which contain the caspase recruitment domain, CARD, have been shown to be involved in inflammation and immune response. This protein may function as an activator of NF-κB signaling.
The protein may play a role in the regulation of inflammation and apoptosis. Mutations in this gene have been associated with a spectrum of dominantly inherited autoinflammatory diseases called cryopyrin-associated periodic syndrome (CAPS). Multiple alternatively spliced transcript variants encoding distinct isoforms have been identified for this gene.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPK115Hu01 Recombinant NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAK115Hu01 Polyclonal Antibody to NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) WB; IHC; ICC; IP.
Assay Kits SCK115Hu CLIA Kit for NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Chemiluminescent immunoassay for Antigen Detection.
SEK115Hu ELISA Kit for NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPK115Mu01 Recombinant NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Positive Control; Immunogen; SDS-PAGE; WB.
RPK115Mu02 Recombinant NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Positive Control; Immunogen; SDS-PAGE; WB.
APK115Mu01 Active NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Cell culture; Activity Assays.
Antibodies PAK115Mu01 Polyclonal Antibody to NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) WB
Assay Kits SEK115Mu ELISA Kit for NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPK115Ra01 Recombinant NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Polyclonal Antibody Customized Service Offer
Assay Kits SEK115Ra ELISA Kit for NLR Family, Pyrin Domain Containing Protein 3 (NLRP3) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome."Nat. Genet. 29:301-305(2001) [PubMed] [Europe PMC] [Abstract]
  2. "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. 46:2445-2452(2002) [PubMed] [Europe PMC] [Abstract]
  3. "PYPAF1: a PYRIN-containing APAF1-like protein that assembles with ASC and activates NF-kB."J. Biol. Chem. 277:11570-11575(2002) [PubMed] [Europe PMC] [Abstract]
  4. "CIAS1/cryopyrin/PYPAF1/NALP3/CATERPILLER 1.1 is an inducible inflammatory mediator with NF-kappa B suppressive properties."J. Immunol. 171:6329-6333(2003) [PubMed] [Europe PMC] [Abstract]
  5. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  6. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
  7. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  8. "Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells." Genome Res. 10:1546-1560(2000) [PubMed] [Europe PMC] [Abstract]
  9. "NALP3 forms an IL-1beta-processing inflammasome with increased activity in Muckle-Wells autoinflammatory disorder."Immunity 20:319-325(2004) [PubMed] [Europe PMC] [Abstract]
  10. "The SPRY domain of Pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing."Cell Death Differ. 14:1457-1466(2007) [PubMed] [Europe PMC] [Abstract]
  11. "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. 55:443-452(2007) [PubMed] [Europe PMC] [Abstract]
  12. "Osteoblasts express NLRP3, a nucleotide-binding domain and leucine-rich repeat region containing receptor implicated in bacterially induced cell death."J. Bone Miner. Res. 23:30-40(2008) [PubMed] [Europe PMC] [Abstract]
  13. "Novel role of PKR in inflammasome activation and HMGB1 release."Nature 488:670-674(2012) [PubMed] [Europe PMC] [Abstract]
  14. "Selective inhibition of the NLRP3 inflammasome by targeting to promyelocytic leukemia protein in mouse and human."Blood 121:3185-3194(2013) [PubMed] [Europe PMC] [Abstract]
  15. "Crystal structure of NALP3 protein pyrin domain (PYD) and its implications in inflammasome assembly."J. Biol. Chem. 286:39528-39536(2011) [PubMed] [Europe PMC] [Abstract]
  16. "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. 70:1498-1506(2002) [PubMed] [Europe PMC] [Abstract]
  17. "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. 71:198-203(2002) [PubMed] [Europe PMC] [Abstract]
  18. "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. 46:3340-3348(2002) [PubMed] [Europe PMC] [Abstract]
  19. "Fine structure mapping of CIAS1: identification of an ancestral haplotype and a common FCAS mutation, L353P."Hum. Genet. 112:209-216(2003) [PubMed] [Europe PMC] [Abstract]
  20. "Variant chronic infantile neurologic, cutaneous, articular syndrome due to a mutation within the leucine-rich repeat domain of CIAS1."Arthritis Rheum. 50:2719-2720(2004) [PubMed] [Europe PMC] [Abstract]
  21. "Clinical and genetic heterogeneity among Spanish patients with recurrent autoinflammatory syndromes associated with the CIAS1/PYPAF1/NALP3 gene."Arthritis Rheum. 50:4045-4050(2004) [PubMed] [Europe PMC] [Abstract]
  22. "Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU."Blood 103:2809-2815(2004) [PubMed] [Europe PMC] [Abstract]
  23. "A novel CIAS1 mutation and plasma/cerebrospinal fluid cytokine profile in a German patient with neonatal-onset multisystem inflammatory disease responsive to methotrexate therapy."Pediatrics 114:E124-E127(2004) [PubMed] [Europe PMC] [Abstract]
  24. "A novel missense mutation in CIAS1 encoding the pyrin-like protein, cryopyrin, causes familial cold autoinflammatory syndrome in a family of Ethiopian origin."Int. Arch. Allergy Immunol. 143:190-193(2007) [PubMed] [Europe PMC] [Abstract]