Cat#:EIA-619C;Product Name:Human ADAMTS13 ELISA Kit;Size:96T;Sample:plasma;Intended use:RUO;Synonym:ADAMTS13; ADAM metallopeptidase with thrombospondin type 1 motif, 13; 13; TTP; VWFCP; C9orf8; vWF-CP; ADAM-TS; FLJ42993; ADAM-TS13; ADAMTS-13; MGC118899; MGC118900; DKFZp434C2322; A disintegrin and metalloproteinase with thrombospondin motifs 13; ADAM-TS 13; OTTHUMP00000022496; OTTHUMP00000022497; OTTHUMP00000022498; vWF-cleaving protease; von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13; EC 3.4.24.87; EC 3.4.24.14; EC 3.4.24.82;Background:ADAMTS13: A Disintegrin-like And Metalloprotease with ThromboSpondin type 1 repeats is a von Willebrand factor-cleaving metalloproteinase. The level of ADAMTS13 in normal plasma is estimated 900 ng/ml. Deficiency of ADAMTS13 results in the development of clinical entity Thrombotic Thrombocytopenic Purpura (TTP) and may play a role in Hemolytic Uremic Syndrome. Von Willebrand’s Factor is synthesized by endothelial cells and stored in the sub-endothelial space as very large multimeric structures. These ULVWf avidly bind platelets when they are exposed in the setting of endothelial disruption and lead to platelet clumping and closure of the endothelial rent. Any ULVWf that escapes into circulation is rapidly degraded by ADAMTS13. Deficiency of ADAMTS13 (congenital or acquired) leads to the accumulation of ULVWf in the plasma with resultant systemic platelet aggregation and clumping. This leads to the clinical syndrome of TTP characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic deficits, renal abnormalities and fever.Congenital deficiency of ADAMTS13 (Upshaw-Schulman Syndrome) is characterized by ADAMTS13 enzymatic activity of 2-7% or normal. Modest reductions in ADAMTS13 have been noted in a variety of conditions, such as severe sepsis, disseminated intravascular coagulation, heparin-induced thrombocytopenia, cardiac surgery and cirrhosis.;Species Reactivity:Human;Application:quantitate;Storage:Unopened Kit: Store at 2 - 8°C. Do not use past kit expiration date. Opened/Reconstituted Reagents: TMB Solution A; TMB Solution B; TMB Stop Solution; Wash Buffer; HRP-conjugate antibody The above mentioned reagents should be stored for up to 1 month at 2 - 8°C. Microplate Wells: Return unused wells to the foil pouch containing the desiccant pack, reseal along entire edge of zip-seal. May be stored for up to 1 month at 2 - 8°C.;Usage:For Lab Research Use Only;Detection Principle:Sandwich ELISA;