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Domain structure of ADAMTS13. The consensus sequence for zinc binding, potential N- and O-glycosylation sites, and the regions sufficient for VWF cleaving activity and for interaction with IgG antibodies of TTP are marked. Truncation upstream of the spacer domain markedly decreases the cleaving activity against either VWF multimers or VWF fragments of the A2 domain, while truncation downstream of the spacer domain decreases the cleaving activity against VWF multimers.
