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RabbitAnti-Complement C2 Rabbit pAb  antibody (bs-8613R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-8613R
英文名称 Complement C2 Rabbit pAb
中文名称 补体C2抗体
别    名 CO2_MOUSE; C2; C3/C5 convertase; ARMD14; CO2;   
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Human,  (predicted: Mouse, Rat, Dog, Pig, Horse, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 57/81 kDa
检测分子量 87
细胞定位 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Complement C2/Complement C2a fragment: 401-500/752 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 The complement component proteins: C2, C3, C4, and C5 are potent anaphylatoxins that are released during complement activation. Binding of these proteins to their respective G protein-coupled receptors induces proinflammatory events such as cellular degranulation, smooth muscle contraction, arachidonic acid metabolism, cytokine release, leukocyte activation, and cellular chemotaxis. C2 deficiency (C2D) is the most common deficiency of the classical complement pathway and is mostly found in patients with autoimmune disease or susceptibility to bacterial infections. The N-terminal extracellular domain 1 of complement C2 receptor inhibitory trispanning, or CRIT, binds to C2 and specifically interacts with the C2a fragment. In doing so, CRIT blocks C2 cleavage and also prevents the classical pathway of C3 convertase formation.

Function:
Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor 4b to generate the C3 or C5 convertase.

Subunit:
C2a interacts with Schistosoma haematobium TOR (via N-terminal extracellular domain). This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection.

Subcellular Location:
Secreted.

DISEASE:
Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000]. A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections.

Similarity:
Belongs to the peptidase S1 family.
Contains 1 peptidase S1 domain.
Contains 3 Sushi (CCP/SCR) domains.
Contains 1 VWFA domain.

Gene ID:
717

Database links:

Entrez Gene: 717 Human

SwissProt: P06681 Human

Unigene: 408903 Human



产品图片
Sample: Lane 1: Human HeLa cell lysates Lane 2: Human HepG2 cell lysates Lane 3: Human A431 cell lysates Primary: Anti-Complement C2 (bs-8613R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 57/81 kDa Observed band size: 87 kDa
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