[Capacity of activation of so-called deficient factors in hereditary blood coagulation disorders].
By factor-specific exchange-experiments it was proved that the lack of factor VIII activity in hemophilia A is not caused by an incapacity of activation or even lack of the factor, but by a defect of the pertaining activator resp. activator system. The same goes for factor IX in hemophilia B as well as factors VII, IX and X in family defect of the prothrombin-complex. In contrast, inactivity in the von Willebrand-Jünrgens-disease is obviously caused by a genuine lack of factor VIII.
D001778:Blood Coagulation Disorders / Q000235:genetics*; D005164:Factor IX / Q000378:metabolism; D005167:Factor VII / Q000378:metabolism; D006467:Hemophilia A / Q000097:blood*; D002836:Hemophilia B / Q000097:blood*; D006801:Humans; D011516:Prothrombin / Q000378:metabolism; D014842:von Willebrand Diseases / Q000097:blood*
D005167:Factor VII; D011516:Prothrombin; D005164:Factor IX
Zur Aktivierbarkeit sog. Mangelfaktoren hereditärer Koagulopathien