Factor I Polyclonal Antibody

Ab type

Primary antibody

Background

CFI 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.

Reactivity

Human

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Immunogen

The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

65720

synonyms

CFI; IF; Complement factor I; C3B/C4B inactivator