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. 2016 Feb 10;52(5-6):319–335. doi: 10.3233/BIR-15061

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Fig. 1. — Mature VWF. (A) Mature VWF is a multi-domain protein. It contains the A1, A2 and A3 domains that are flanked by two D-domain assemblies. These D-domains include the VWD, cysteine-8(C8)-fold, trypsin inhibitor-like (TIL) structure and E-modules. Following this, the protein contains six VW C-domains followed by the C-terminal cysteine knot (CTCk). Mature VWF includes 10 O-linked glycans depicted by open circle and 13 N-linked glycans depicted by filled circles. Thirteen percent of the N-glycans carry ABO(H) blood group determinants. VWF has a number of binding partners that are shown below the schematic. The A2-domain is also cleaved by the enzyme ADAMTS13. (B) Approximate representation of VWF based on existing structural knowledge. (C) Though the carbohydrates of VWF are usually represented by small lollipops, their physical dimension can be fairly large, in particular the N-linked glycans. (Colors are visible in the online version of the article; http://dx.doi.org/10.3233/BIR-15061.)