[HTML][HTML] von Willebrand factor arginine 1205 substitution results in accelerated macrophage‐dependent clearance in vivo

O Rawley, JM O'sullivan, A Chion, S Keyes… - Journal of Thrombosis …, 2015 - Elsevier
Summary Background Enhanced von Willebrand factor (VWF) clearance is important in the
etiology of type 1 and type 2 von Willebrand disease (VWD). More than 20 different VWF
point mutations have already been reported in patients with enhanced clearance. These
include the VWD‐Vicenza variant, which is characterized by an Arg1205His substitution in
the VWF D3 domain. Critically, however, the molecular mechanisms through which single
amino acid substitutions in VWF result in enhanced clearance of this complex multimeric …