Antithrombin III is the most important inhibitor of thrombin and other coagulation proteinases. It belongs to the serine proteinase inhibitor (serpin) superfamily of inhibitors and structurally related proteins, which contain reactive centers that have evolved to attract and entrap certain proteinases. Inherited antithrombin III deficiency (AT3D) is a risk factor for the early development of venous thromboembolism (THPH7). Antithrombin III regulates clot formation both by inhibiting thrombin activity directly and by interfering with earlier stages of the clotting cascade. By in situ hybridization and quantitative analysis of DNA dosage in carriers of chromosome 1 deletions, AT3 is mapped to 1q23-q25. The AT3 gene has 7 exons. It contains 9 complete and 1 partial repetitive ALU sequence elements, which occur in the introns of the gene at a higher frequency (about 22% of the intron sequence) than in the genome as a whole (about 5%). Sample Type: Cell culture supernates, serum, plasma (heparin, EDTA, citrate) and urine Intended Use: For quantitative detection of human Serpin C1 in cell culture supernates, serum, plasma (heparin, EDTA, citrate) and urine. Sensitivity: <10pg/ml Range: 312-20,000pg/ml Specificity: Natural and recombinant human Serpin C1 Test Principle: This human Serpin C1 BioAssay(TM) ELISA Kit was based on standard sandwich enzyme-linked immune-sorbent assay technology. A monoclonal antibody from mouse specific for Serpin C1 has been precoated onto 96-well plates. Standards (NSO, H33-K464) and test samples are added to the wells, a biotinylated detection polyclonal antibody from goat specific for Serpin C1 is added subsequently and then followed by washing with PBS or TBS buffer. Avidin-Biotin-Peroxidase Complex was added and unbound conjugates were washed away with PBS or TBS buffer. HRP substrate TMB was used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the human Serpin C1 amount of sample captured in plate. Kit Components: 96-well plate precoated with human Serpin C1 antibody, 1 ST0000-20, Lyophilized recombinant human Serpin C1 standard, 20ng/tube*2 Biotinylated human Serpin C1 antibody, 130ul (dilution 1:100) Avidin-Biotin-Peroxidase Complex (ABC), 130ul (dilution 1:100) Sample diluent buffer, 30ml Antibody diluent buffer, 12ml ABC diluent buffer, 12ml TMB color developing agent, 10ml TMB stop solution, 10ml Storage and Stability: Store powder at 4C liquid at -20C. Store other components at 4C. Stable for at least 6 months For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
