Figure 2.

VWF intracellular processing. VWF is transcribed as pre-pro–VWF (nucleus, blue) and is translocated to the ER (green), where it undergoes signal peptide removal, glycosylation, and dimer formation. In the Golgi (yellow) O-linked glycans are added, carbohydrates are processed, VWF is sulfated and multimerized, and propeptide is cleaved from mature VWF but remains noncovalently associated. VWF is trafficked to the regulated secretory pathway (peach) and stored in WPBs or α-granules before release into plasma (red), where VWFpp and VWF dissociate and circulate independently of one another.