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Rabbit Anti-NALP3/CIAS1/FITC Conjugated antibody (bs-10021R-FITC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-10021R-FITC
英文名称 Anti-NALP3/CIAS1/FITC
中文名称 FITC标记的细胞凋亡诱导蛋白NALP3抗体
别    名 LRR and PYD domains-containing protein 3; AGTAVPRL; AII/AVP antibody Angiotensin/vasopressin receptor AII/AVP like; Angiotensin/vasopressin receptor AII/AVP-like; C1orf7; Caterpiller protein 1.1; CIAS 1; CIAS1; CLR1.1; Cold autoinflammatory syndrome 1; Cold autoinflammatory syndrome 1 protein; Cryopyrin; Familial cold autoinflammatory syndrome; FCAS; FCU; Muckle-Wells syndrome; MWS; NACHT; NACHT LRR and PYD containing protein 3; NALP 3; NALP3; NALP3_HUMAN; NLRP3; PYPAF 1; PYPAF1 antibody PYRIN containing APAF1 like protein 1; PYRIN-containing APAF1-like protein 1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  心血管  细胞生物  免疫学  信号转导  细胞凋亡  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, 
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 114kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Cryopyrin
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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.

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.

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.

DISEASE:
Defects in NLRP3 are the cause of familial cold autoinflammatory syndrome type 1 (FCAS1) [MIM:120100]; 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) [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.
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 (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.

Similarity:
Belongs to the NLRP family.
Contains 1 DAPIN domain.
Contains 9 LRR (leucine-rich) repeats.
Contains 1 NACHT domain.

Database links:

Entrez Gene: 114548 Human

Entrez Gene: 216799 Mouse

Entrez Gene: 287362 Rat

Omim: 606416 Human

SwissProt: Q96P20 Human

SwissProt: Q8R4B8 Mouse

Unigene: 159483 Human

Unigene: 54174 Mouse




Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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