ADAMTS13 and hemolytic-uremic syndrome: Subsequently, the classification evolved toward a molecular basis of the disease (68): a disintegrin and metalloprotease with thrombospondin type one repeats, member 13 (ADAMTS13) deficiency is associated with thrombotic thrombocytopenic purpura; and HUS is classified as typical or atypical depending on whether shiga toxin is present or absent (HUS and aHUS, respectively).