NALP3, Polyclonal Antibody

Goat Anti-NALP3, ID (NACHT-, LRR- and PYD-Containing Protein 3, Cryopyrin, Cold Autoinflammatory Syndrome 1 Protein, PYPAF1, PYRIN-Containing APAF1-like Protein 1, CIAS1, Caterpiller Protein 1.1, CLR1.1, C1orf7, CIAS1, NLRP3, PYPAF1, Angiotensin/Vasopressin Receptor AII/AVP-like

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 1; NC_000001.10 (247579247..247612410). Location: 1q44

Polyclonal

IgG

Goat

Recognizes human NLRP3. Expected to recognize all five reported isoforms (NP_004886.3; NP_899632.1; NP_001230062.1; NP_001120933.1; NP_001120934.1).

Purified by immunoaffinity chromatography.

Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

0.5 mg/ml (lot specific)

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-NLRP3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

NALP3 is a cytoplasmic, pyrin-like protein belonging to the NLRP family with a DPIN domain, seven leucine rich repeats and a NACHT domain. It is known to interact with apoptosis-associated speck-like protein PYCARD/ASC and is a part of the NALP3 inflammasome complex. NALP3 is involved in the activation of caspase-1 and caspase-5 in response to a number of triggers including bacterial or viral infection, thus leading to the processing of IL-1B and IL-18. It may also inhibit TNF-alpha-induced activation and nuclear translocation of RELA/NF-KB p65 and function in the activation of NF-kappa-B signaling, thus regulating inflammation and apoptosis. Expression is predominantly in peripheral blood leukocytes, polymorphonuclear cells, osteoblasts and chondrocytes. NALP3 defects leads to several diseases including familial cold auto inflammatory syndrome type 1 (FCAS1), Muckle-Wells syndrome (MWS), chronic infantile neurological cutaneous and articular (CINCA) syndrome, and neonatal-onset multisystem inflammatory disease (NOMID).

Antibodies; Abs to Inflammasomes

ELISA, Western Blot, Immunohistochemistry

Peptide ELISA: 1:64,000 (detection limit) Western Blot: 0.3ug/ml detects a band at ~70kD in humn lymph node and tonsil lysates. Immunohistochemistry: 2-4ug/ml Requires HIER using sodium citrate buffer pH6.0. Shows focal and interstitial staining of the inflammatory elements close to the epithelia of the tonsil crypts in paraffin embedded human tonsil. Optimal dilutions to be determined by the researcher.

118,173 Da[Similar Products]

NLR family, pyrin domain containing 3

NACHT, LRR and PYD domains-containing protein 3; cryopyrin; 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

NACHT, LRR and PYD domains-containing protein 3

C1orf7; CIAS1; NALP3; PYPAF1; CLR1.1  [Similar Products]

NALP3_HUMAN

This gene encodes a pyrin-like protein containing a pyrin domain, a nucleotide- (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, Oct 2008]

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

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