Von Willebrand factor (VWF) is a multifunctional plasma protein that plays
a prominent role in haemostasis. In endothelial cells, processing of its pr
ecursor pro-VWF results in the formation of two large polypeptides, mature
VWF and a propeptide. These proteins are co-secreted on an equimolar basis
but are cleared from the circulation at different rates. VWF levels are fre
quently elevated in response to vascular disorders. Similarly, propeptide l
evels are increased under these conditions, although primarily in fulminant
vascular disease, such as thrombotic thrombocytopenic purpura and septicem
ia. In chronic vascular disease, e.g. diabetes or peripheral vascular disea
se, propeptide levels are much less elevated. The differential response of
VWF and propeptide levels to vascular disease could provide a means to asse
ss the extent and time course of endothelial cell activation. After secreti
on, the propeptide may play a role in modulating cellular adhesion processe
s. Thus, enhanced propeptide secretion seems not to be of merely diagnostic
significance.