货号：GZ8592
NLRP3;C1orf7;CIAS1;NALP3;PYPAF1;NACHT, LRR and PYD domains-containing protein 3;Angiotensin/vasopressin receptor AII/AVP-like;Caterpiller protein 1.1CLR1.1;Cold autoinflammatory syndrome 1 protein;Cryopyrin;PYRIN-containing APAF1-like protein 1
| 别名 | NLRP3;C1orf7;CIAS1;NALP3;PYPAF1;NACHT, LRR and PYD domains-containing protein 3;Angiotensin/vasopressin receptor AII/AVP-like;Caterpiller protein 1.1CLR1.1;Cold autoinflammatory syndrome 1 protein;Cryopyrin;PYRIN-containing APAF1-like protein 1 |
| 基因名称 | NLRP3 |
| 蛋白名称 | NACHT LRR and PYD domains-containing protein 3 |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Swissprot No. | Q96P20 |
| Mouse Swissprot No. | Q8R4B8 |
| 特异性 | Endogenous |
| 稀释度 | IHC-P 1:200-1000, WB 1:500-5000, IF-P/IF-F/IF-ICC 1:200-1000, ELISA 1:5000-20000, IP 1:50-200 |
| 参考分子量 | 115kDa |
| 运输及保存条件 | Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | 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 s |
| 细胞定位 | Cytoplasm, Nuclear |
| 功能 | Disease:Defects in NLRP3 are a cause of Muckle-Wells syndrome (MWS) [MIM:191900]; 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.,Disease:Defects in NLRP3 are the cause of chronic infantile neurologic cutaneous and articular syndrome (CINCA) [MIM:607115]; also known as 'neonatal onset multisystem inflammatory disease,' or NOMID, 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.,Disease:Defects in NLRP3 are the cause of familial cold autoinflammatory syndrome type 1 (FCAS1) [MIM:120100]; commonly 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.,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.,induction:By TNF-alpha.,online information:Repertory of FMF and hereditary autoinflammatory disorders mutations,similarity:Belongs to the NLRP family.,similarity:Contains 1 DAPIN domain.,similarity:Contains 1 NACHT domain.,similarity:Contains 7 LRR (leucine-rich) repeats.,subunit: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.,tissue specificity:Expressed in blood leukocytes. Strongly expressed in polymorphonuclear cells and osteoblasts. Undetectable or expressed at a lower magnitude in B- and T-lymphoblasts, respectively. High level of expression detected in chondrocytes. Detected in non-keratinizing epithelia of oropharynx, esophagus and ectocervix and in the urothelial layer of the bladder., |
| 纯化 | Protein A |
| Clonality | Monoclonal |
| 细胞定位 | Cytoplasm, Nuclear |
周一至周日 8:00-18:00
