Figure 11.

ADAMTS13 and VWF interaction. (A) Models of the ADAMTS13-MDTCS shown at 90 degree rotations. The residues important for the interaction with VWF are shown in red and other potential VWF-binding sites are shown in orange. Domains are colored as in Figure 7A. (B) Amino acid sequence of VWF (D1596-R1668) and the ADAMTS13-interacting sites are schematically represented. (C) Close-up view of the exosite-3 in the S domain, the hydrophobic cluster surrounded by arginine residues. The residues important for VWF-binding are indicated with red letters. (D) Close-up view of the VWF segment (D1653-C1670) in an α-helical conformation observed in the crystal structure of the VWF A2 domain [153]. The figure was created in reference to the original drawing by de Groot et al. [152].