Description
Complement Factor I Antibody / CFI | R32399 | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water
Format: Antigen affinity purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human, Rat
Application: WB, FACS
Buffer: N/A
Limitation: This Factor I antibody is available for research use only.
Purity: Antigen affinity
Description: Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. 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.
Immunogen: Amino acids K19-D220 were used as the immunogen for the Factor I antibody.
Storage: After reconstitution, the Factor I antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20 °C. Avoid repeated freezing and thawing.
