Schematic structure of VWF.

Linear VWF multimers are built from dimers as smallest repeating subunits, which are linked in a head-to-head fashion via the disulfide bonds Cys1099-Cys1099’ and Cys1142-Cys1142’ between their N-terminal D`D3 assemblies. Dimers consist of two monomers–comprising the domains D`D3, A1, A2, A3, D4, C1-C6, and CK–that are linked in a tail-to-tail fashion via the disulfide bonds Cys2771-Cys2773’, Cys2773-Cys2771’, and Cys2811-Cys2811’ between their C-terminal CK domains.