CFI Polyclonal Antibody | G-AB-10741

CFI Polyclonal Antibody | G-AB-10741 | Gentaur Antibodies

Overview: 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.

Category Type: Polyclonal Antibody

Research Areas: Immunology

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

Reactivity: Human, Mouse

Host: Rabbit

Isotype: IgG

Gene ID: 3426

Accession #:

Clonality:

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

Clone #:

Conjugation: Unconjugated

Swissprot: P05156

Santa Cruz:

Calculated MW:

Observed MW:

Concentration: 1 mg/mL

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

Purification method: Affinity purification

Application: IF

Dilution: IF 1:50-1:100

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

CFI Polyclonal Antibody DataSheet