Ant-Complement C4 / C4a抗体This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is

产品编号 yb-11274R
英文名称 Ant-Complement C4 / C4a抗体
中文名称 过敏毒素C4/补体C4抗体
别    名 C4a anaphylatoxin; Complement C4-A alpha chain; complement C4-A proprotein; Acidic C4; Acidic complement C4; Basic C4; Basic complement C4; C4 Anaphylatoxin; C4 complement C4d region; complement C4 alpha chain; C4A; C4A13; C4A2; C4A3; C4A4; C4A6; C4A91; C4B; C4B1; C4B12; C4B2; C4B3; C4B5; C4F; C4S; CH; Chido form of C4; CO4; Complement C4 A; Complement C4 B; Complement C4B; Complement component 4A (Rodgers blood group); Complement component 4A; Complement component 4B (Childo blood group); Complement component 4B; Complement component C4B; CPAMD2; CPAMD3. RG; Rodgers Form Of C4.
Ant-Complement C4 / C4a抗体  
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, 
产品应用 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
分 子 量 8/84/190kDa
细胞定位 分泌型蛋白 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Complement C4 C4a anaphylatoxin or complement C4 alpha chain
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.

PubMed PubMed
产品介绍 background:
This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011].

Function:
C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin.

Subunit:
This protein is synthesized as a single-chain precursor and, prior to secretion, is enzymatically cleaved to form a trimer of non-identical chains (alpha, beta and gamma).

Subcellular Location:
Secreted.

DISEASE:
Defects in C4A are the cause of complement component 4A deficiency (C4AD). A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.

Similarity:
Contains 1 anaphylatoxin-like domain.
Contains 1 NTR domain.