vWF is first formed as a prepropeptide. After the
signal peptide is removed, two pro-vWF units
attach to each other at their carboxy ends by
means of numerous disulfide bridges to form a
dimer. The dimers represent the repetitive
units, or protomers, of mature vWF. The provWF
dimers are transported to the Golgi apparatus,
where the pro-vWF (vW antigen II or
vWagII) is removed. Mature vWF and vWagII
are stored inWeibel–Palade bodies in epithelial
cells. The mature subunits and vWagII contain
binding sites for factor VIII, heparin, collagen,
ristocetin + platelets, and thrombin-activated
platelets.
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