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. 2018 Apr 19;103(7):1099–1109. doi: 10.3324/haematol.2016.151407

Figure 4.

Figure 4.

Conformational changes of ADAMTS13. [Mp: metalloprotease (red); Dis: disintegrin-like domain (green); Cys-rich: cysteine-rich domain (orange); TSRs: thrombospondin-type 1 repeats (light and dark gray); CUB1 domain (cyan); CUB2 domain (magenta)]. (A) In the blood circulation of healthy individuals and immune-mediated thrombotic thrombocytopenic purpura (iTTP) patients in remission ADAMTS13 is present in closed conformation with CUB1/2 domains covering the spacer domain. Binding of plasma factors (e.g. activating anti-ADAMTS13 auto-Abs against the TSR5-8-CUB1/2 domains80,82) (orange part of the anti-ADAMTS13 antibodies) results in a conformational change: the opening of the ADAMTS13.83,100 (B) Open conformation of ADAMTS13 exposes the B-cell epitope localized in the spacer domain, making it accessible for additional anti-ADAMTS13 Abs.