| Product Name | Factor I |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | CFI antibody; IF antibody; Complement factor I antibody; C3B/C4B inactivator antibody; |
| Product description | 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 |
| Immunogen | The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490 |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WB |
| IHC:1:500-1:2000 |
|
| Species Reactivity | Human |
| Concentration | 1mg/ml |
| Alternative Names | CFI antibody IF antibody Complement factor I antibody C3B/C4B inactivator antibody |
|---|---|
| Molecular Weight(MW) | 66 kDa |
| SwissProt ID | P05156 |
|---|
| Positive Control | Heart,Liver,Plasma, |
|---|---|
| Application Notes | IHC:1:500-1:2000 |
| Form | Liquid |
|---|---|
| Storage Instructions | Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles. |
| Storage Buffer | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
