The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
生理活性Specific activity: 500-1800 units/mg.
Specific activity is determined by titration of Thrombomodulin in a Human protein C activation assay. One unit of activity is the amount of Thrombomodulin required to maximize the activation of human protein C by one NIH unit of thrombin.
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Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
This product is an active protein and may elicit a biological response in vivo, handle with caution.
機能Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated.
組織特異性Endothelial cells are unique in synthesizing thrombomodulin.
関連疾患Defects in THBD are the cause of thrombophilia due to thrombomodulin defect (THR-THBD) [MIM:188040]. A hemostatic disorder characterized by a tendency to thrombosis. Defects in THBD are a cause of susceptibility to hemolytic uremic syndrome atypical type 6 (AHUS6) [MIM:612926]. 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. Note=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.