Cultured aortic endothelial cells from pigs with von Willebrand disease: in vitro model for studying the molecular defect(s) of the disease.
Aortic endothelial cells from normal pigs and pigs with von Willebrand disease have been established in long-term cultures. Both cultures appeared similar in terms of general growth characteristics, morphologic features and ultrastructure. Immunofluorescent staining of these cultures with chicken (or rabbit) antiporcine ristocetin-Willebrand factor sera (or IgG) resulted in extensive perinuclear staining of the cells in both cultures. Additionally, staining of semiconfluent cultures of normal cells for ristocetin-Willebrand factor revealed an extensive meshwork of distinct, immunologically identifiable ristocetin-Willebrand factor-containing filaments between cells. Immunoreactive material was considerably decreased and more diffuse between cells in semiconfluent cultures from affected pigs. Through immunocytochemical staining with peroxidase-coupled antiserum, the filaments (of indeterminate length) were found to have a diameter of approximately 300 A. Finally, washed porcine platelets interacted extensively with scrape-damaged cultures of affected endothelial cells. This interaction of platelets with damaged normal cultures was abolished by pretreatment of the cultures with rabbit antiporcine ristocetin-Willebrand factor IgG.
D000818:Animals; D001011:Aorta / Q000473:pathology; D001792:Blood Platelets / Q000502:physiology; D002455:Cell Division; D002478:Cells, Cultured; D004727:Endothelium / Q000473:pathology; D008854:Microscopy, Electron; D012310:Ristocetin / Q000378:metabolism; D013552:Swine; D014842:von Willebrand Diseases / Q000378:metabolism / Q000473:pathology*; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D012310:Ristocetin