After secretion, ADAMTS13 is either bound to the endothelial surface or free in the blood stream. The heightened shear stress in small- and microvessels alters the 3D-structure of VWF from the contracted globular form to its linear form. The linear VWF has now its active binding sites exposed, that are important to start blood coagulation. These sites bind platelets and blood vessel lesions by interlinking the stretched VWF with one another – a blood clot is formed. In its uncut form, (ultra large) VWF's heightened stickiness and interlinking causes spontaneous platelet binding and blood clotting. The linear VWF exposes the A2 domain, so that in the presence of enough ADAMTS13 activity it gets cut to its normal size. VWF in the normal length loses its heightened stickiness and spontaneous crosslinking activity to only form blood clots when needed.
Upshaw–Schulman syndrome
Haglund's syndrome
Potocki–Shaffer syndrome
Frey's syndrome
Perlman syndrome
Urethral syndrome
Lamb-Shaffer syndrome
Shone's syndrome