Washington University in St. Louis (WU)
My work is focused on understanding the etiologies of thrombotic microangiopathies to effect a treatment. We have studied the structure function relationship of von Willebrand Factor and ADAMTS13. Deficiency of ADAMTS13 activity leads to a thrombotic microangiopathy. We have identified a small polypeptide of ADAMTS13 that will enhance cleavage of von Willebrand Factor by full-length ADAMTS13. We have also participated in the clinical trials of eculizumab in the treatment of atypical hemolytic uremic syndrome, another thrombotic microangiopathy that results from deficient complement regulation.