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CFI Polyclonal Antibody-BS70683
CFI Polyclonal Antibody-BS70683
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CFI Polyclonal Antibody
Sizes: 50µl, 100µl
Catalogue Numbers: BS70683-50, BS70683-100
Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Swiss-Prot: P05156
Host: Rabbit
Reactivity: Human, Mouse
Applications: WB
All Applications: WB,1:500 - 1:2000
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.
Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: Polyclonal Antibodies
Bioworld Molecular Weight: 60kDa
Note: For research use only, not for use in diagnostic procedure.
Extra Notes: Western Blot analysis of extracts of Human serum, using CFI antibody at 1:500 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% non-fat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 5s.
Alternative Name: CFI; AHUS3; ARMD13; C3BINA; C3b-INA; FI; IF; KAF
Immunogen: Recombinant fusion protein of human CFI(NP_000195.2).
Conjugate: Unconjugated
Modification: Unmodified