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Factor I rabbit pAb
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ES5825
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50 µL $148.00
100 µL $248.00
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Overview

Product name: Factor I rabbit pAb
Reactivity: Human;Rat;Mouse;
Alternative Names: CFI; IF; Complement factor I; C3B/C4B inactivator
Source: Rabbit
Dilutions: IHC: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
Immunogen: The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490
Storage: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Observed Band: Full lenth:66kD, heavy chain: 50-58kD
GeneID: 3426
Human Swiss-Prot No: P05156
Cellular localization: Secreted, extracellular space. Secreted .
Background: 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