ITT1652
ITT1652
- Catalog: ITT1652
- Gene/Protein: CFI
- Product Description: Immunotag™ Factor I Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
|
| Target Protein | Factor I |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490 |
| Specificity | Factor I Polyclonal Antibody detects endogenous levels of Factor I protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | CFI |
| Accession No. | P05156 Q61129 |
| Alternate Names | CFI; IF; Complement factor I; C3B/C4B inactivator |
| Description | complement factor I(CFI) Homo sapiens 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 Ref |
| Cell Pathway/ Category | Complement and coagulation cascades, |
| Protein Expression | Heart,Liver,Plasma, |
| Subcellular Localization | extracellular region,extracellular space,membrane,extracellular exosome, |
| Protein Function | catalytic activity:Inactivates complement subcomponents C3b, iC3b and C4b by proteolytic cleavage.,disease: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.,disease:Defects in CFI are the cause of component I deficiency (CFI deficiency) [MIM:217030]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.,disease:Defects in CFI may be associated with or predispose to hemolytic uraemic syndrome (HUS) [MIM:235400]. HUS, the most frequent cause of acute renal failure in childhood, is characterized by the association of acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. The majority of HUS cases occur after an episode of infectious diarrhea, and are associated with E.coli O157:H7 infection. However, atypical cases of HUS occur in the absence of infectious diarrhea, although less commonly. Some are inherited in either an autosomal dominant or a recessive pattern and these patients often experience relapse and progress to hypertension and chronic renal disease. Sporadic forms can occur with many of the same signs and symptoms.,function:Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.,online information:CFI mutation db,similarity:Belongs to the peptidase S1 family.,similarity:Contains 1 peptidase S1 domain.,similarity:Contains 1 SRCR domain.,similarity:Contains 2 LDL-receptor class A domains.,subunit:Heterodimer of a light and heavy chains linked by disulfide bonds.,tissue specificity:Plasma., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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