anti-Complement Factor I antibody
CAT.NO. : ARG42938
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概述
产品描述 | Rabbit Polyclonal antibody recognizes Complement Factor I |
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反应物种 | Hu, Ms |
应用 | WB |
宿主 | Rabbit |
克隆 | Polyclonal |
同位型 | IgG |
靶点名称 | Complement Factor I |
抗原物种 | Human |
抗原 | Recombinant fusion protein corresponding to aa. 19-300 of Human Complement Factor I (NP_000195.2). |
偶联标记 | Un-conjugated |
別名 | Complement factor I; KAF; C3B/C4B inactivator; C3b-INA; ARMD13; EC 3.4.21.45; FI; AHUS3; C3BINA; IF |
应用说明
应用建议 |
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应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||
阳性对照 | Human serum extracts | ||||
实际分子量 | ~ 65 kDa |
属性
形式 | Liquid |
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纯化 | Affinity purified. |
缓冲液 | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
抗菌剂 | 0.02% Sodium azide |
稳定剂 | 50% Glycerol |
存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
数据库连接 | |
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基因名称 | CFI |
全名 | complement factor I |
背景介绍 | This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015] |
生物功能 | Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. Inhibits these pathways by cleaving three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins (PubMed:7360115, PubMed:17320177). Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces (PubMed:2141838, PubMed:9605165, PubMed:12055245). The presence of these cofactors on healthy cells allows degradation of deposited C3b by CFI in order to prevent undesired complement activation, while in apoptotic cells or microbes, the absence of such cofactors leads to C3b-mediated complement activation and subsequent opsonization (PubMed:28671664). [UniProt] |
细胞定位 | Secreted, extracellular space. [UniProt] |
预测分子量 | 66 kDa |
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