Disease in a dish: Modelling Von Willebrand disease with patient-specific induced pluripotent stem cells

Blood outgrowth endothelial cells (BOECs), also indicated as endothelial colony forming cells (ECFCs), are being used in our lab and by others as an endothelial cell model to study Von Willebrand disease (VWD) and Von Willebrand factor (VWF). To overcome some of the shortcomings of this model, among which the low success rate of isolation of BOECs and their phenotypic variability, we aim to develop a more reliable disease model. We will generate induced pluripotent stem cells (iPSCs) from peripheral blood mononuclear cells. The iPSCs will be differentiated into iPSC derived endothelial cells and also megakaryocytes to further study the pathophysiology of VWD.