Factor VIII (aka Factor VIII:C or Antihemophilic Globulin) is a glycoprotein zymogen that circulates in a stabilized non-covalent complex with von Willebrand Factor (vWF). Following activation by thrombin or Factor Xa, Factor VIIIa dissociates from vWF and catalyzes the activation of Factor X by Factor IXa in the amplification phase of coagulation. Factor VIIIa activity is quickly decreased by spontaneous dissociation and proteolytic degradation by activated Protein C, Factor Xa and Factor IXa. Hemophilia A is caused by mutations in the Factor VIII gene; a majority of patients have decreased Factor VIII plasma levels while 5% of patients have normal levels of nonfunctioning protein. The sensitive quantitative measurement of total human Factor VIII antigen in plasma samples is easily performed with this 96 well strip format ELISA kit. The average normal plasma level of Factor VIII is defined as 1.0 IU/ml and the normal range is 0.4-1.8 IU/ml. Hemaophilia A patients are classified by the following Factor VIII levels: 0.05-0.25 IU/ml = mild, 0.01-0.05 IU/ml = moderate, and less than 0.01 IU/ml = severe. The assay measures total human Factor VIII in the 0.0016-0.84 IU/ml range. Samples giving human Factor VIII levels above 0.84 IU/ml should be diluted in blocking buffer before use. 1:8 and 1:16 dilutions for normal plasma, or 1:4 and 1:8 dilutions for Haemophiliac plasma, are suggested for best results.
|Wirt / Host||Human|
|Gene ID|| NCBI (externer Link)|
|Datenblatt|| Auf Anfrage|