Key features and details
- Sheep polyclonal to Factor I/CFI
- Suitable for: Other, Double Immunodiffusion, Immunoelectrophoresis, RID
- Reacts with: Human
- Isotype: IgG
製品名Anti-Factor I/CFI antibody
Factor I/CFI 一次抗体 製品一覧
製品の詳細Sheep polyclonal to Factor I/CFI
アプリケーション適用あり: Other, Double Immunodiffusion, Immunoelectrophoresis, RIDmore details
Full length native protein (purified) corresponding to Factor I/CFI. Purified from plasma.
Database link: P05156
This antibody is also known as KAF/C3b Inactivator. The IgG concentration will range between 10-20 mg/ml.
Factor I/CFI is a plasma protein of molecular weight 88 kDa, consisting of two disulphide linked chains of 50 and 38 kDa. Factor I/CFI is a serine protease with a high degree of specificity for C3b and C4b. It has a requirement for protein cofactors for cleavage of these complement proteins, Factor H, CR1 or MCP are required for C3b cleavage and C4bp or CR1 for C4b cleavage.
This product was previously labelled as Factor I
保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Preservative: 0.1% Sodium azide
Constituents: 0.1% EACA, 0.01% Benzamidine, 0.0292% EDTA
Concentration information loading...
特記事項（精製）Antiserum is prepared by immunisation of sheep with the human Factor I/CFI and, if necessary, adsorption to monospecificity by use of solid-phase adsorbents. An immunoglobulin fraction is then produced. The titre is adjusted so that inter-batch variation is within 10%. The product is finally 0.2µm filtered.
一次抗体 備考Factor I is a plasma protein of molecular weight 88 kDa, consisting of two disulphide linked chains of 50 and 38 kDa. Factor I is a serine protease with a high degree of specificity for C3b and C4b. It has a requirement for protein cofactors for cleavage of these complement proteins, Factor H, CR1 or MCP are required for C3b cleavage and C4bp or CR1 for C4b cleavage.
Our Abpromise guarantee covers the use of ab8843 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Other||Use at an assay dependent concentration.|
|Double Immunodiffusion||Use at an assay dependent concentration. 10µL antiserum vs 10µL fresh plasma.|
|Immunoelectrophoresis||Use at an assay dependent concentration. 100µL antiserum vs 10µL fresh plasma.|
|RID||Use at an assay dependent concentration. 1.5µL antiserum/cm² gel vs 10µL fresh plasma.|
機能Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.
関連疾患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.
配列類似性Belongs to the peptidase S1 family.
Contains 1 Kazal-like domain.
Contains 2 LDL-receptor class A domains.
Contains 1 peptidase S1 domain.
Contains 1 SRCR domain.
細胞内局在Secreted > extracellular space.
- Information by UniProt
- AHUS3 antibody
- ARMD13 antibody
- C3b INA antibody
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab8843 は 3 報の論文で使用されています。
- Hallam TM et al. Rare Genetic Variants in Complement Factor I Lead to Low FI Plasma Levels Resulting in Increased Risk of Age-Related Macular Degeneration. Invest Ophthalmol Vis Sci 61:18 (2020). PubMed: 32516404
- Lemaire M et al. Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome. Nat Genet 45:531-6 (2013). PubMed: 23542698
- Bienaime F et al. Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome. Kidney Int 77:339-49 (2010). ELISA . PubMed: 20016463