Type 2A VWD variants enhance the rate of ADAMTS13 proteolysis.

<p>VWF A2 FRET variants (50nM) and 50nM ADAMTS13 or 5nM ADAMTS13 were separately pre-incubated in 1.25mM CaCl₂ ± 0.5M urea for at 37°C for 45mins before being combined to initiate proteolysis. Emission scans were taken at Ex₅₀₅ every 0.5mins for 120mins, after 2mins ADAMTS13 was added. FRET_obs data was converted to Fraction Cleaved (Frac Cleaved). (A) Selected exponential rise fits are shown for type 2A VWD variants with either 0M (upper panel) or 0.5M urea (lower panel) and 50nM (solid lines) and 5nM (dashed lines) ADAMTS13. (B) Half-lives were calculated and are displayed for type 2A VWD variants (grouped by 0M and 0.5M urea in ascending order, various coloured bars) and WT (0.5M, 1M, 1.5M, 2M and 2.5M urea, black diagonal lined bars) in ascending order.