Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation. The enzyme cleaves the von Willebrand factor at bond 842-Tyr-|-Met-843 within the A2 domain. Binds 1 zinc ion per subunit. Binds 4 Ca(2+) ions. Zinc and calcium ions cooperatively modulate enzyme activity. The cleavage of the pro-domain is not required for protease activity. Dependence on calcium for proteolytic activity is mediated by the high affinity site. Secretion enhanced by O-fucosylation of TSP type-1 repeats. Plasma. Expressed primarily in liver. The pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency. The globular cysteineless spacer domain adopts a jelly-roll topology, and is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction. Glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion. The precursor is processed by a furin endopeptidase which cleaves off the pro-domain. Genetic variations in ADAMTS13 coding region influence plasmatic ADAMTS13 activity levels. Dependent on the sequence context, the same polymorphisms might be either positive or negative modifiers of gene expression, thereby altering the phenotype of ADAMTS13 deficiency. The disease is caused by variants affecting the gene represented in this entry. Truncated N-terminus. Truncated N-terminus. ADAMTS13 entry Leiden Open Variation Database (LOVD)