Anti-Factor I/CFI Antibody Picoband® Fluoro594 Conjugated

Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. 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.

E. coli-derived human Factor I recombinant protein (Position: K19-D220) . Human Factor I shares 70.7% and 71.2% amino acid (aa) sequence identity with mouse and rat Factor I, respectively.

1. Entrez Gene: complement factor I. 2. Goldberger G, Bruns GA, Rits M, Edge MD, Kwiatkowski DJ (Jul 1987) . Human complement factor I: analysis of cDNA-derived primary structure and assignment of its gene to chromosome 4. The Journal of Biological Chemistry 262 (21) : 10065–71.