This is a synthetic peptide designed for use in combination with anti-CFI Antibody. It may block above mentioned antibody from binding to its target protein in western blot and/or immunohistochecmistry under proper experimental settings. There is no guarantee for its use in other applications.
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Format
Lyophilized
Reconstitution
Add 100 μL of sterile PBS. Final peptide concentration is 1 mg/mL in PBS.
Stock
-20 °C
Stockage commentaire
For longer periods of storage, store at -20°C. Avoid repeat freeze-thaw cycles.
Antigène
Complement Factor I (CFI)
Synonymes
cfi Peptide, MGC53615 Peptide, Cfi Peptide, factor I Peptide, IF Peptide, gb:ai721528 Peptide, ahus3 Peptide, c3b-ina Peptide, c3bc4bi Peptide, c3bina Peptide, kaf Peptide, CFI Peptide, AHUS3 Peptide, C3BINA Peptide, C3b-INA Peptide, FI Peptide, KAF Peptide, complement factor I S homeolog Peptide, complement factor I L homeolog Peptide, complement factor I Peptide, complement component factor i Peptide, cfi.S Peptide, cfi.L Peptide, CFI Peptide, cfi Peptide, Cfi Peptide
Sujet
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.