FITC标记的补体C4a+C4b蛋白抗体
产品名称： FITC标记的补体C4a+C4b蛋白抗体
英文名称： Anti-C4a+C4b/FITC
产品编号： HZ-15186R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-C4a+C4b/FITC Conjugated antibody
FITC标记的补体C4a+C4b蛋白抗体
| 英文名称 | Anti-C4a+C4b/FITC |
| 中文名称 | FITC标记的补体C4a+C4b蛋白抗体 |
| 别 名 | Complement C4 beta chain; complement C4-A proprotein; Acidic complement C4; Basic complement C4; CH; Chido blood group; Complement component 4A; Complement component 4B; RG; Rodgers blood group; CO4A_HUMAN; CO4B_HUMAN; C4A; C4; C4A2; C4A3; C4A4; C4A6; C4AD; C4S; CO4; CPAMD2; RG. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 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 | 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 | 72/190kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human C4a and C4b Complement C4 beta 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. |
| 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: Non-enzymatic component of C3 and C5 convertases and thus essential for the propagation of the classical complement pathway. Covalently binds to immunoglobulins and immune complexes and enhances the solubilization of immune aggregates and the clearance of IC through CR1 on erythrocytes. C4A isotype is responsible for effective binding to form amide bonds with immune aggregates or protein antigens, while C4B isotype catalyzes the transacylation of the thioester carbonyl group to form ester bonds with carbohydrate antigens. Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes. Subunit: Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain. Subcellular Location: Secreted. Tissue Specificity: Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland,and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extra-hepatic sites of expression may be important for the local protection and inflammatory response. Post-translational modifications: Prior to secretion, the single-chain precursor is enzymatically cleaved to yield non-identical chains alpha, beta and gamma. During activation, the alpha chain is cleaved by C1 into C4a and C4b, and C4b stays linked to the beta and gamma chains. Further degradation of C4b by C1 into the inactive fragments C4c and C4d blocks the generation of C3 convertase. The proteolytic cleavages often are incomplete so that many structural forms can be found in plasma. N- and O-glycosylated. O-glycosylated with a core 1 or possibly core 8 glycan. DISEASE: Defects in C4A are the cause of complement component 4A deficiency (C4AD) [MIM:614380]. A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis. Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE) [MIM:152700]. A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Note=Interindividual copy-number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE. Similarity: Contains 1 anaphylatoxin-like domain. Contains 1 NTR domain. Database links: UniProtKB/Swiss-Prot: P0C0L4.2 UniProtKB/Swiss-Prot: P0C0L5.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码补体因子4的酸性形式，这是经典激活途径的一部分。该蛋白被表达为单链前体，在分泌前被蛋白水解成α、β和γ链的三聚体。三聚体为抗原-抗体复合物与其他补体成分之间的相互作用提供了一个表面。α链可能被裂解，释放C4过敏原，一种局部炎症介质。这种蛋白质的缺乏与系统性红斑狼疮和I型糖尿病有关。该基因定位于6号染色体上的主要组织相容性复合体（MHC）Ⅲ类区域。存在该基因簇的不同单倍型，使得个体可以有1, 2个或3个拷贝该基因。发现了两个编码不同亚型的转录变体。[ RefSeq，NOV 2011 ]提供。