Gene name:
NLRP3 (C1orf7;CIAS1;NALP3;PYPAF1) ;
Protein name:
NACHT, LRR and PYD domains-containing protein 3 ;
Alternative:
Caterpiller protein 1.1 (CLR1.1) ;Angiotensin/vasopressin receptor AII/AVP-like ;Cryopyrin ;Cold autoinflammatory syndrome 1 protein ;PYRIN-containing APAF1-like protein 1 ;
Organism:
Human (Homo sapiens).
General Annotation
Sub Unit:
Interacts with PYCARD/ASC. Part of the NALP3 inflammasome complex which is involved in activation of caspase-1 and caspase-5, leading to processing of IL1B and IL18.
Function:
May function as an inducer of apoptosis. Interacts selectively with ASC and this complex may function as an upstream activator of NF-kappa-B signaling. Inhibits TNF-alpha induced activation and nuclear translocation of RELA/NF-KB p65. Also inhibits transcriptional activity of RELA. Activates caspase-1 in response to a number of triggers including bacterial or viral infection which leads to processing and release of IL1B and IL18.
Subcellular Location:
Cytoplasm
Protein Attributes:
50:
MKMASTRCKL | ARYLEDLEDV | DLKKFKMHLE | DYPPQKGCIP | LPRGQTEKAD |
100:
HVDLATLMID | FNGEEKAWAM | AVWIFAAINR | RDLYEKAKRD | EPKWGSDNAR |
150:
VSNPTVICQE | DSIEEEWMGL | LEYLSRISIC | KMKKDYRKKY | RKYVRSRFQC |
200:
IEDRNARLGE | SVSLNKRYTR | LRLIKEHRSQ | QEREQELLAI | GKTKTCESPV |
250:
SPIKMELLFD | PDDEHSEPVH | TVVFQGAAGI | GKTILARKMM | LDWASGTLYQ |
300:
DRFDYLFYIH | CREVSLVTQR | SLGDLIMSCC | PDPNPPIHKI | VRKPSRILFL |
350:
MDGFDELQGA | FDEHIGPLCT | DWQKAERGDI | LLSSLIRKKL | LPEASLLITT |
400:
RPVALEKLQH | LLDHPRHVEI | LGFSEAKRKE | YFFKYFSDEA | QARAAFSLIQ |
450:
ENEVLFTMCF | IPLVCWIVCT | GLKQQMESGK | SLAQTSKTTT | AVYVFFLSSL |
500:
LQPRGGSQEH | GLCAHLWGLC | SLAADGIWNQ | KILFEESDLR | NHGLQKADVS |
550:
AFLRMNLFQK | EVDCEKFYSF | IHMTFQEFFA | AMYYLLEEEK | EGRTNVPGSR |
600:
LKLPSRDVTV | LLENYGKFEK | GYLIFVVRFL | FGLVNQERTS | YLEKKLSCKI |
650:
SQQIRLELLK | WIEVKAKAKK | LQIQPSQLEL | FYCLYEMQEE | DFVQRAMDYF |
700:
PKIEINLSTR | MDHMVSSFCI | ENCHRVESLS | LGFLHNMPKE | EEEEEKEGRH |
750:
LDMVQCVLPS | SSHAACSHGL | VNSHLTSSFC | RGLFSVLSTS | QSLTELDLSD |
800:
NSLGDPGMRV | LCETLQHPGC | NIRRLWLGRC | GLSHECCFDI | SLVLSSNQKL |
850:
VELDLSDNAL | GDFGIRLLCV | GLKHLLCNLK | KLWLVSCCLT | SACCQDLASV |
900:
LSTSHSLTRL | YVGENALGDS | GVAILCEKAK | NPQCNLQKLG | LVNSGLTSVC |
950:
CSALSSVLST | NQNLTHLYLR | GNTLGDKGIK | LLCEGLLHPD | CKLQVLELDN |
1000:
CNLTSHCCWD | LSTLLTSSQS | LRKLSLGNND | LGDLGVMMFC | EVLKQQSCLL |
1036:
QNLGLSEMYF | NYETKSALET | LQEEKPELTV | VFEPSW
Vaild Sequence:
Related Databases
Uniprot:
ELISA Kit
CLIA Kit
Polyclonal Antibody
Monoclonal Antibody
Protein
FOR
Mouse
Human
Bovine
ELISA Kit for Human NACHT, LRR and PYD domains-containing protein 3
ELISA Kit for Human NACHT, LRR and PYD domains-containing protein 3
ELISA Kit for Human NACHT, LRR and PYD domains-containing protein 3
CLIA Kit for Human NACHT, LRR and PYD domains-containing protein 3
CLIA Kit for Human NACHT, LRR and PYD domains-containing protein 3
CLIA Kit for Human NACHT, LRR and PYD domains-containing protein 3
Polyclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Polyclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Polyclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Monoclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Monoclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Monoclonal Antibody for Human NACHT, LRR and PYD domains-containing protein 3
Protein for Human NACHT, LRR and PYD domains-containing protein 3
Protein for Human NACHT, LRR and PYD domains-containing protein 3
Protein for Human NACHT, LRR and PYD domains-containing protein 3
R&D Technical Data
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Precision
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Recovery
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Linearity
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Cited for : NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 1; 2 AND 3);VARIANT MWS MET-200;VARIANTS FCAS1/MWS TRP-262 AND PRO-307
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[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
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[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
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[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
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tissue :
Brain .
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[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
Cited for : NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
8.
Mural R.J.
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more...
Submitted (2005-07) to the EMBL/GenBank/DDBJ databases
[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
Cited for : NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
9.
[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
Cited for : NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 2; 4 AND 6)
tissue :
Colon .
10.
"Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells."
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Xu X.-R.
,
Han Z.-G.
,
Zhang J.-W.
,
Tao J.
,
Huang Q.-H.
,
Zhou J.
,
Hu G.-X.
,
Gu J.
,
Chen S.-J.
,
Chen Z.
more...
Genome Res.10:1546-1560(2000)
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PubMed ]
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Europe PMC ]
[
Abstract ]
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Cited for : NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 393-1036 (ISOFORM 1)
tissue :
Umbilical cord blood .
11.
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Cited for : IDENTIFICATION IN NALP3 INFLAMMASOME COMPLEX
12.
"The SPRY domain of Pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing."
Papin S.
,
Cuenin S.
,
Agostini L.
,
Martinon F.
,
Werner S.
,
Beer H.D.
,
Grutter C.
,
Grutter M.
,
Tschopp J.
Cell Death Differ.14:1457-1466(2007)
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PubMed ]
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Europe PMC ]
[
Abstract ]
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Cited for : INTERACTION WITH MEFV
13.
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Cited for : SUBCELLULAR LOCATION;TISSUE SPECIFICITY
14.
[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
Cited for : TISSUE SPECIFICITY
15.
"Novel role of PKR in inflammasome activation and HMGB1 release."
Lu B.
,
Nakamura T.
,
Inouye K.
,
Li J.
,
Tang Y.
,
Lundbaeck P.
,
Valdes-Ferrer S.I.
,
Olofsson P.S.
,
Kalb T.
,
Roth J.
,
Zou Y.
,
Erlandsson-Harris H.
,
Yang H.
,
Ting J.P.
,
Wang H.
,
Andersson U.
,
Antoine D.J.
,
Chavan S.S.
,
Hotamisligil G.S.
,
Tracey K.J.
more...
Nature488:670-674(2012)
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PubMed ]
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Europe PMC ]
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Abstract ]
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Cited for : INTERACTION WITH EIF2AK2
16.
"Selective inhibition of the NLRP3 inflammasome by targeting to promyelocytic leukemia protein in mouse and human."
Lo Y.H.
,
Huang Y.W.
,
Wu Y.H.
,
Tsai C.S.
,
Lin Y.C.
,
Mo S.T.
,
Kuo W.C.
,
Chuang Y.T.
,
Jiang S.T.
,
Shih H.M.
,
Lai M.Z.
Blood121:3185-3194(2013)
[
PubMed ]
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Europe PMC ]
[
Abstract ]
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Cited for : INTERACTION WITH PML
17.
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Cited for : X-RAY CRYSTALLOGRAPHY (1.7 ANGSTROMS) OF 3-112;SUBUNIT;DISULFIDE BOND
18.
"New mutations of CIAS1 that are responsible for Muckle-Wells syndrome and familial cold urticaria: a novel mutation underlies both syndromes."
Dode C.
,
Le Du N.
,
Cuisset L.
,
Letourneur F.
,
Berthelot J.-M.
,
Vaudour G.
,
Meyrier A.
,
Watts R.A.
,
Scott D.G.I.
,
Nicholls A.
,
Granel B.
,
Frances C.
,
Garcier F.
,
Edery P.
,
Boulinguez S.
,
Domergues J.-P.
,
Delpech M.
,
Grateau G.
Am. J. Hum. Genet.70:1498-1506(2002)
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PubMed ]
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Europe PMC ]
[
Abstract ]
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Cited for : VARIANT FCAS1 MET-200;VARIANTS MWS ASN-305; MET-350; THR-441 AND ARG-571;VARIANT FCAS/MWS TRP-262
19.
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Cited for : VARIANTS CINCA ASN-305; LEU-308; SER-311; ARG-360; ASN-438; SER-575 AND THR-664;TISSUE SPECIFICITY
20.
"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."
Aksentijevich I.
,
Nowak M.
,
Mallah M.
,
Chae J.J.
,
Watford W.T.
,
Hofmann S.R.
,
Stein L.
,
Russo R.
,
Goldsmith D.
,
Dent P.
,
Rosenberg H.F.
,
Austin F.
,
Remmers E.F.
,
Balow J.E. Jr.
,
Rosenzweig S.
,
Komarow H.
,
Shoham N.G.
,
Wood G.
,
Jones J.
,
Mangra N.
,
Carrero H.
,
Adams B.S.
,
Moore T.L.
,
Schikler K.
,
Hoffman H.
,
Lovell D.J.
,
Lipnick R.
,
Barron K.
,
O'Shea J.J.
,
Kastner D.L.
,
Goldbach-Mansky R.
more...
Arthritis Rheum.46:3340-3348(2002)
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PubMed ]
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Europe PMC ]
[
Abstract ]
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Cited for : VARIANTS CINCA HIS-266; ASN-305; LEU-525 AND CYS-572
21.
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Cited for : VARIANT FCAS1 PRO-355;VARIANT LYS-705
22.
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Cited for : VARIANT CINCA CYS-861
23.
"Clinical and genetic heterogeneity among Spanish patients with recurrent autoinflammatory syndromes associated with the CIAS1/PYPAF1/NALP3 gene."
Arostegui J.I.
,
Aldea A.
,
Modesto C.
,
Rua M.J.
,
Argueelles F.
,
Gonzalez-Ensenat M.A.
,
Ramos E.
,
Rius J.
,
Plaza S.
,
Vives J.
,
Yaguee J.
Arthritis Rheum.50:4045-4050(2004)
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PubMed ]
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Europe PMC ]
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Abstract ]
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Cited for : VARIANTS FCAS1 MET-200; PRO-307 AND LYS-490;VARIANT CINCA ASN-305;VARIANT MWS MET-350
24.
"Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU."
Neven B.
,
Callebaut I.
,
Prieur A.-M.
,
Feldmann J.
,
Bodemer C.
,
Lepore L.
,
Derfalvi B.
,
Benjaponpitak S.
,
Vesely R.
,
Sauvain M.J.
,
Oertle S.
,
Allen R.
,
Morgan G.
,
Borkhardt A.
,
Hill C.
,
Gardner-Medwin J.
,
Fischer A.
,
de Saint Basile G.
Blood103:2809-2815(2004)
[
PubMed ]
[
Europe PMC ]
[
Abstract ]
[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s)
Cited for : VARIANTS CINCA LEU-262; PRO-262; ASN-305; GLY-305; SER-311; MET-350; ASP-356; PRO-407; ILE-438; CYS-572 AND PHE-634
25.
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Cited for : VARIANT CINCA THR-174
26.
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Cited for : VARIANT FCAS1 CYS-525