Von Willebrand Disease: From In Vivo to In Vitro Disease Models.

摘要: Von Willebrand factor (VWF) plays an essential role in primary hemostasis and is exclusively synthesized stored endothelial cells megakaryocytes. Upon vascular injury, VWF released into the circulation where this multimeric protein required for platelet adhesion. Defects of lead to most common inherited bleeding disorder von disease (VWD). Three different types VWD exist, presenting with varying degrees tendencies. The pathophysiology can be investigated by examining synthesis, storage secretion producing cells. These either or transfected heterologous cell models. For many years have been used successfully elucidate aspects synthesis. However, those do not fully reflect characteristics Obtaining megakaryocytes a phenotype, requires invasive procedures, such as vessel collection bone marrow biopsy. A more recent promising development isolation colony forming (ECFCs) from peripheral blood true-to-nature model. Alternatively, various animal models are available but limiting, therefore, new approaches needed study other disorders. potential versatile source could induced pluripotent stem (iPSCs). This review gives overview that will discuss novel considered improve understanding structural functional mechanisms underlying disease.