{"title":"15266-ADAMTS-13","description":"","products":[{"product_id":"adamts-13-human-mab-20a5","title":"ADAMTS-13, Human, mAb 20A5","description":"\u003cul\u003e\n\u003cli\u003eThe monoclonal antibody 20A5 recognizes human ADAMTS-13, A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13. ADAMTS-13 is produced by hepatic stellate cells and in smaller amounts by human endothelial cells, and is present in plasma at a concentration of approximately 1 ug\/ml. ADAMTS-13 is a zinc-containing metalloprotease belonging to the ADAMTS family characterized by a protease domain, an adjacent disintegrin-like domain, one or more thrombospondin type 1 repeats, a cystein-rich domain and a typical spacer region. ADAMTS-13 is composed of a series of domains (amino to carboxy terminal): metalloprotease, disintegrin-like, central thrombospondin-1 (TSP-1), cysteine-rich, spacer, seven additional TSP-1 domains and two unique CUB domains. ADAMTS-13 has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane. The apparent molecular weight is 170 or 190 kDa on non-reducing or reducing SDS-PAGE, respectively. ADAMTS-13 has an important function in haemostasis, where it catalyzes the cleavage of the peptide bond between tyrosine-1605 and methionine-1606 in the A2 domain of von Willebrand Factor (VWF), resulting in 2 electrophoretic reduced fragments of 176 and 140 kDa, respectively. This process renders large multimers less adhesive and hence less reactive in the setting of thrombus formation. ADAMTS-13 is therefore said to be a natural anti-thrombotic agent. Severe ADAMTS-13 deficiency is associated with systemic microvascular thrombosis in familial or acquired thrombotic thrombocytopenic purpura (TTP). The accumulation of non-cleaved large VWF multimers causes spontaneous systemic platelet aggregation blocking oxygen supply to vital organs. This life-threatening disorder can lead to ischemic disease with (multiple) organ failure. The monoclonal antibody 20A5 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).\u003c\/li\u003e\n\u003cli\u003eApplications: Immunoassays , Immunofluorescence , Western Blot\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Hycult Biotech","offers":[{"title":"Default Title","offer_id":53614150746403,"sku":"HYBHM2226100UG","price":726.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0926\/3870\/8003\/files\/Hycult_20800x800_20100dpi_f611a91d-1e3d-4f67-ac24-e4f8e06a8aee.png?v=1753031798"},{"product_id":"adamts-13-human-mab-20a5-20-ug","title":"ADAMTS-13, Human, mAb 20A5, 20 µg","description":" ","brand":"Hycult Biotech","offers":[{"title":"Default Title","offer_id":53614150779171,"sku":"HYBHM222620UG","price":265.9,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0926\/3870\/8003\/files\/Hycult_20800x800_20100dpi_f28d12b8-bc11-4681-baee-26328186b975.png?v=1753031802"}],"url":"https:\/\/chromspec.com\/collections\/15266-adamts-13.oembed","provider":"Chromatographic Specialties Inc.","version":"1.0","type":"link"}