Disease-causing mutations were spread along the length of the ADAMTS13 gene. (B) The structure of the ADAMTS13 protein is shown in the red box in the middle of the diagram along with the 29 exons that make up the ADAMTS13 gene encoding for it. (A) The top third of the diagram shows all of the ADAMTS13 variations seen by exon, with novel mutations denoted in bold text. (C) The bottom third shows the age of symptom onset according to mutation location. Red drops denote those presenting in pregnancy. Only patients with mutations confined to a single exon are included. C1 and C2, type 1 and 2 CUB domains; Cys, cysteine rich; Dis, disintegrin; P, propeptide; S, signal peptide. Adapted from Zheng et al.¹⁰