Immunoelectron microscopic localization of factor VIII-related antigen in the vascular endothelial cells of a patient with type 1 von Willebrand disease.
The intracellular localization of a factor VIII-related antigen in human umbilical veins derived both from a patient with 'severe Type 1' von Willebrand disease and from a normal full-term baby was investigated by immunoelectron microscopy. In normal vascular endothelial cells, the factor VIII-related antigen was detected in Weibel-Palade bodies. The endothelial cells of von Willebrand disease did not include these structures, but were filled with distended vesicles, in which the factor VIII-related antigen was found immunohistochemically. These results suggest that Type 1 von Willebrand disease causes dysfunction of blood coagulation by suppressing secretion of factor VIII-related antigen into the blood.
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D004730:Endothelium, Vascular / Q000737:chemistry / Q000648:ultrastructure*; D006801:Humans; D007231:Infant, Newborn; D016253:Microscopy, Immunoelectron; D014471:Umbilical Veins; D014842:von Willebrand Diseases / Q000378:metabolism*; D014841:von Willebrand Factor / Q000032:analysis*
D014841:von Willebrand Factor