CFI Polyclonal Antibody | E-AB-62790

CFI Polyclonal Antibody | E-AB-62790 | Gentaur UK, US & Europe Distribution

Type: Polyclonal Antibody

Synonyms: CFI, AHUS3, ARMD13, C3BINA, C3b-INA, FI, IF, KAF

Application: IF

Reactivity: Human, Mouse

Host: Rabbit

Isotype: IgG

Reserch Areas: Immunology

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.

Concentration: 1 mg/mL

Storage: Store at -20°C. Avoid freeze / thaw cycles.

Immunogen: Recombinant fusion protein of human CFI (NP_000195.2).

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3

Purification Method: Affinity purification

Dilution: IF 1:50-1:100

Clone: N/A

Conjugation: Unconjugated

Molecular Weight(Calculated): N/A

Molecular Weight(Observed): N/A