Generation and validation of small ADAMTS13 fragments for epitope mapping of anti‐ADAMTS13 autoantibodies in immune‐mediated thrombotic thrombocytopenic purpura

摘要

In immune‐mediated thrombotic thrombocytopenic purpura (iTTP), patients develop an immune response against the multidomain enzyme ADAMTS13. ADAMTS13 consists of a metalloprotease (M) and disintegrin‐like (D) domain, 8 thrombospondin type 1 repeats (T1‐T8), a cysteine‐rich (C), a spacer (S), and 2 CUB domains (CUB1‐2). Previous epitope mapping studies have used relatively large overlapping ADAMTS13 fragments.