Anti-NLRP3, NT (NACHT-, LRR- and PYD-Containing Protein 3, Cryopyrin, Cold Autoinflammatory Syndrome

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. Applications: Suitable for use in ELISA, Western Blot, Immunofluorescence and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1-2ug/ml, Immunohistochemistry (Formalin fixed paraffin embedded): 2.5ug/ml, Optimal dilutions to be determined by the researcher. Storage and 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 at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.