L’anticorps Lapin Polyclonal anti-Complement Factor I a été validé pour WB. Il convient pour détecter Complement Factor I dans des échantillons de Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Cheval, Porc et Lapin.
N° du produit ABIN5514688
Aperçu rapide pour Complement Factor I anticorps (C-Term) (ABIN5514688)
CFI
Reactivité: Humain
WB
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Format
Liquid
Buffer
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Stock
-20 °C
Stockage commentaire
For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
Antigène
Complement Factor I (CFI)
Autre désignation
CFI
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.