Upon binding to the D4‐CK domains of globular VWF, ADAMTS‐13 is induced to adopt an ‘open’ conformation exposing the cryptic spacer domain exosites (this conformational change also exposes the autoantibody epitopes recognized in thrombotic thrombocytopenic purpura) 22. Here, ADAMTS13 is linked to thrombotic thrombocytopenic purpura.