Domain organizations of VWF and ADAMTS13. (A) Domain organization of VWF. Bottom: Major ligand-binding sites in VWF are highlighted. Top: Location of the ADAMTS13 cleavage site in the A2 domain is highlighted. (B) Domain organization of ADAMTS13. Bottom: Highlighted functional aspects of ADAMTS13 domains. The N-terminal domains recognize/bind to unraveled VWF, whereas the C-terminal domains interact with both globular and unraveled VWF.