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Anti-Complement C4/C4过敏毒素C4/补体C4抗体
点击次数:205发布时间:2012/12/26 7:49:47
更新日期:2024/9/5 14:43:40
所 在 地:其它
产品型号:BY-11274R
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货号:BY-11274R
英文名称:Anti-Complement C4/C4
中文名称:过敏毒素C4/补体C4抗体
其他名称:Acidic C4;Acidic complement C4;Basic C4;Basic complement C4;C4 Anaphylatoxin;C4 complement C4d region;C4 propeptide;complement C4 alpha chain; Complement C4 beta chain; Complement C4 gamma 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.
抗体来源:Rabbit
克隆类型:polyclonal
蛋白分子量:predicted molecular weight: 192kDa
纯化方法:affinity purified by Protein A
交叉反应:hu
产品介绍: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.