Figure 1.
Figure 1. Structure of ADAMTS13 and mutations in congenital thrombotic thrombocytopenic purpura. / The ADAMTS13 gene (A) contains 29 exons in ∼ 37 kb on chromosome 9q34. Dashed lines show the relationship of exons to the ADAMTS13 protein (B). Structural domains include signal peptide (S), propeptide (P), metalloprotease, disintegrin domain (Dis), thrombospondin 1 repeats (numbered 1-8), cysteine-rich domain (Cys), spacer domain, and CUB domains. Mutations in patients with inherited TTP are shown (C). / Reproduced with permission from Zheng X, Majerus EM, Sadler JE. ADAMTS13 and TTP. Curr Opin Hematol. 2002;9:389-394.

Structure of ADAMTS13 and mutations in congenital thrombotic thrombocytopenic purpura.

The ADAMTS13 gene (A) contains 29 exons in ∼ 37 kb on chromosome 9q34. Dashed lines show the relationship of exons to the ADAMTS13 protein (B). Structural domains include signal peptide (S), propeptide (P), metalloprotease, disintegrin domain (Dis), thrombospondin 1 repeats (numbered 1-8), cysteine-rich domain (Cys), spacer domain, and CUB domains. Mutations in patients with inherited TTP are shown (C).

Reproduced with permission from Zheng X, Majerus EM, Sadler JE. ADAMTS13 and TTP. Curr Opin Hematol. 2002;9:389-394.

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