Evaluation of Weibel-Palade bodies role in von Willebrand Disease

Background and ObjectivesWeibel-Palade bodies (WPBs) are specific storage granules of vascular endothelial cells to which they are unique and for whose physiology they might play an important role. Materials and MethodsWe have reviewed the most recent literature published regarding the function of WPBs and their related proteins. ResultsDiscrimination of WPBs from lysosomes drew scientist's attention to their biological importance. Furthermore, localization of the important proteins such as von Willebrand factor (vWF), P-selectin, and Osteoprotegerin has confirmed the critical role played by these organelles. The content of WPBs can be very quickly available in case of vascular injuries suggesting that they could act as regulators of rapid vascular responses. Since the biogenesis of these granules depends on the presence of vWF, it would be interesting to know how the absence of vWF and WPBs would affect the function of other stored proteins. This situation which is present in von Willebrand Disease needs to be evaluated. ConclusionsThis review contributes to a better knowledge of WPBs biology and function. At the same time, further investigations have yet to be done to reveal the roles these organelles can play.