| 產(chǎn)品編號 | bs-10339R |
| 英文名稱 | Factor I heavy chain Rabbit pAb |
| 中文名稱 | 補(bǔ)體因子I重鏈抗體 |
| 別 名 | Complement factor I heavy chain; Factor I heavy chain; AHUS3; C3b INA; C3b inactivator; C3B/C4B inactivator; C3BINA; CFAI_HUMAN; CFI; Complement component I; Complement control protein factor I; Complement factor I; F1; factor I; FactorI; FI; I factor; IF; KAF; Konglutinogen activating factor; Heavy chain of factor I; OTTHUMP00000219728. |
| 研究領(lǐng)域 | 心血管 免疫學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 35/63 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞外基質(zhì) 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Complement factor I heavy chain: 261-360/582 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The complement pathway is an important host defense system that contributes to both innate and acquired immunity. There are three pathways of complement activation: the classical pathway, lectin pathway and alternative pathway. Complement protein Factor I is a key serine protease that modulates the complement cascade by regulating the levels of C3 convertases. It circulates in plasma as a heavily N-glycosylated heterodimer made up of two disulfide linked chains, each carrying three N-linked oligosaccharide chains that may have both structural and functional roles in the interactions with the natural substrate and the cofactor during catalysis. Factor I is a serine protease with a high degree of specificity for C3b and C4b. It requires protein cofactors for cleavage of these complement proteins; Factor H, CR1 or MCP are required for C3b cleavage, and C4bp or CR1 are required for C4b cleavage. Function: Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Subcellular Location: Secreted; extracellular space. Tissue Specificity: Plasma. DISEASE: Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3) [MIM:612923]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency) [MIM:610984]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections. Similarity: Belongs to the peptidase S1 family. Contains 1 Kazal-like domain. Contains 2 LDL-receptor class A domains. Contains 1 peptidase S1 domain. SWISS: P05156 Gene ID: 3426 Database links: Entrez Gene: 3426 Human Omim: 217030 Human SwissProt: P05156 Human Unigene: 312485 Human |
| 產(chǎn)品圖片 |
Protein: Hela(human)cell lyates at 30ug; HepG2(human)cell lyates at 30ug; Primary: Rabbit Anti-Factor I heavy chain(bs-10339R) at 1:300; Secondary: 800CW Conjugated Goat(polyclonal) Anti-Rabbit IgG(H+L) at 1: 10000; Predicted band size:39/63 kD Observed band size:63 kD
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