AMM03651G

antibody from Leading Biology

Targeting: CFI C3b-INA, FI, IF, KAF

Western blot (Recommended by provider)

Antibody Data

Product number

AMM03651G

Provider

Leading Biology

Product name

IF Antibody (monoclonal) (M01)

Antibody type

Monoclonal

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 uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.

Reactivity

Human

Host

Mouse

Isotype

IgG

Antibody clone number

1B3

Storage

Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.