Porcine Complement Factor I ELISA Kit | CFI elisa kit
Porcine Complement Factor I ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
INTENDED USE This CFI ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Porcine CFI. This ELISA kit for research use only!
PRINCIPLE OF THE ASSAY CFI ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-CFI antibody and an CFI-HRP conjugate. The assay sample and buffer are incubated together with CFI-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the CFI concentration since CFI from samples and CFI-HRP conjugate compete for the anti-CFI antibody binding site. Since the number of sites is limited, as more sites are occupied by CFI from the sample, fewer sites are left to bind CFI-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The CFI concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
CFI: Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3). An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency). CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections. Belongs to the peptidase S1 family.
Protein type: Secreted; Protease; EC 3.4.21.45; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 4q25
Cellular Component: extracellular space; membrane; extracellular region; nucleus
Molecular Function: metal ion binding; serine-type endopeptidase activity; scavenger receptor activity
Biological Process: receptor-mediated endocytosis; regulation of complement activation; innate immune response; proteolysis; complement activation, classical pathway
Disease: Macular Degeneration, Age-related, 13; Complement Factor I Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 3
