Factor I (CFI) Rabbit Polyclonal Antibody

CAT#: TA374680

CFI Rabbit polyclonal Antibody

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB,1:500 - 1:2000
• Reactivities: Human, Mouse
• Modifications: Unmodified
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 19-300 of human CFI (NP_000195.2).
• Formulation: Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
• Concentration: lot specific
• Purification: Affinity purification
• Conjugation: Unconjugated
• Stability: Shelf life: one year from despatch.
• Predicted Protein Size: 65kDa
• Gene Name: complement factor I
• Database Link:
  Entrez Gene 3426 Human
  UniProt ID P05156
• 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.