<i>In vivo</i> proteolysis of VWF mutants.

<p>FLAG tagged, plasma VWF, <i>A-E</i>; 10ng load, F; 25ng load, were examined for proteolysis in 129X1/Sv <i>Adamts13 +/+</i> or <i>Adamts13</i>-/- mice by reducing SDS-PAGE and western blotting. Each lane represents an individual mouse with the VWF mutation(s) and VWF concentration in PPP as measured by ELISA (ug/mL). 10 ng of VWF was loaded per lane. Several forms of VWF were observed: proVWF (i.e., propeptide bound; gray arrowhead), uncleaved mature VWF (black arrowhead), Adamts13-mediated proteolysis (black arrow), and Adamts13-independent proteolysis (gray arrow). In the 2 left most lanes of <i>B</i>-<i>F</i>, wtVWF and R1597W are the same as the 1^st wtVWF and 2^nd R1597W of <i>A</i>, respectively.