catalog number :
MBS604215
products full name :
NALP3 (NACHT-, LRR- and PYD-Containing Protein 3, Cryopyrin, Cold Autoinflammatory Syndrome 1 Protein, PYPAF1, PYRIN-Containing APAF1-like Protein 1, CIAS1)
products short name :
[NALP3]
products name syn :
[Anti -NALP3 (NACHT-, LRR- and PYD-Containing Protein 3, Cryopyrin, Cold Autoinflammatory Syndrome 1 Protein, PYPAF1, PYRIN-Containing APAF1-like Protein 1, CIAS1)]
other names :
[NACHT, LRR and PYD domains-containing protein 3 isoform d; AII/AVP receptor-like; NACHT, LRR and PYD domains-containing protein 3; cryopyrin; OTTHUMP00000038408; OTTHUMP00000038409; OTTHUMP00000038410; OTTHUMP00000038411; OTTHUMP00000230825; caterpiller protein 1.1; PYRIN-containing APAF1-like protein 1; NACHT, LRR and PYD containing protein 3; cold autoinflammatory syndrome 1 protein; NACHT domain-, leucine-rich repeat-, and PYD-containing protein 3; nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 3; NLR family, pyrin domain containing 3]
other gene names :
[NLRP3; CIAS1; AII; AVP; FCU; MWS; FCAS; CIAS1; NALP3; C1orf7; CLR1.1; PYPAF1; AGTAVPRL; FLJ95925]
uniprot entry name :
Q65Z67_HUMAN
specificity :
Recognizes endogenous human NALP3 by Immunoprecipitation and Western Blot.
purity :
Highly Purified. Purified from concentrated hybridoma tissue culture supernatant. 95% (SDS-PAGE).
form :
Supplied as a liquid in PBS containing 0.02% sodium azide.
storage stability :
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
Suitable for use in Western Blot, Immunoprecipitation and immunohistochemistry. Other applications have not been tested.
app notes :
Immunohistochemistry: frozen sections. Immunoprecipitation: 1:50. Western Blot: 1:1000. Optimal dilutions to be determined by researcher.
image1 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: Recombinant human NALP3 (NACHT-, LRR- and PYD-containing protein 3) (pyrin domain).
products categories :
Antibodies; Abs to Disease Markers
ncbi acc num :
NP_001120934.1
ncbi gb acc num :
NM_001127462.2
ncbi pathways :
Immune System Pathway (106386); Inflammasomes Pathway (366166); Influenza A Pathway (217173); Influenza A Pathway (217150); Innate Immunity Signaling Pathway (106387); NOD-like Receptor Signaling Pathway (122191); NOD-like Receptor Signaling Pathway (122131); Nucleotide-binding Domain, Leucine Rich Repeat Containing Receptor (NLR) Signaling Pathways (366164); Pertussis Pathway (218111); Pertussis Pathway (218099)
ncbi summary :
This gene encodes a pyrin-like protein containing a pyrin domain, a nucleotide-binding site (NBS) domain, and a leucine-rich repeat (LRR) motif. This protein interacts with the apoptosis-associated speck-like protein PYCARD/ASC, which contains a caspase recruitment domain, and is a member of the NALP3 inflammasome complex. This complex functions as an upstream activator of NF-kappaB signaling, and it plays a role in the regulation of inflammation, the immune response, and apoptosis. Mutations in this gene are associated with familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), chronic infantile neurological cutaneous and articular (CINCA) syndrome, and neonatal-onset multisystem inflammatory disease (NOMID). Multiple alternatively spliced transcript variants encoding distinct isoforms have been identified for this gene. Alternative 5' UTR structures are suggested by available data; however, insufficient evidence is available to determine if all of the represented 5' UTR splice patterns are biologically valid. [provided by RefSeq]
uniprot summary :
NLRP3: 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. Defects in NLRP3 are the cause of familial cold autoinflammatory syndrome type 1 (FCAS1); also known as familial cold urticaria. FCAS are rare autosomal dominant systemic inflammatory diseases characterized by episodes of rash, arthralgia, fever and conjunctivitis after generalized exposure to cold. Defects in NLRP3 are a cause of Muckle-Wells syndrome (MWS); also known as urticaria-deafness-amyloidosis syndrome. MWS is a hereditary periodic fever syndrome characterized by fever, chronic recurrent urticaria, arthralgias, progressive sensorineural deafness, and reactive renal amyloidosis. The disease may be severe if generalized amyloidosis occurs. Defects in NLRP3 are the cause of chronic infantile neurologic cutaneous and articular syndrome (CINCA); also known as neonatal onset multisystem inflammatory disease (NOMID). CINCA is a rare congenital inflammatory disorder characterized by a triad of neonatal onset of cutaneous symptoms, chronic meningitis and joint manifestations with recurrent fever and inflammation. Belongs to the NLRP family. 5 isoforms of the human protein are produced by alternative splicing. Protein type: Inhibitor. Chromosomal Location of Human Ortholog: 1q44. Cellular Component: cytoplasm; cytosol. Molecular Function: protein binding; peptidoglycan binding; ATP binding. Biological Process: caspase activation; apoptosis; negative regulation of acute inflammatory response; positive regulation of caspase activity; interleukin-1 beta production; defense response; negative regulation of NF-kappaB import into nucleus; positive regulation of interleukin-1 beta secretion; signal transduction; protein oligomerization; activation of NF-kappaB transcription factor; negative regulation of interleukin-1 beta secretion; inhibition of NF-kappaB transcription factor; negative regulation of inflammatory response; innate immune response; interleukin-18 production; interleukin-1 secretion; inflammatory response; detection of biotic stimulus; defense response to virus. Disease: Cinca Syndrome; Familial Cold Autoinflammatory Syndrome 1; Muckle-wells Syndrome