Cleaves the vWF multimers in plasma into smaller forms. Von Willebrand factor cleaving protease, A disintegrin and metalloproteinase with thrombospondin mots 13, A disintegrin like and metalloprotease reprolysin type with thrombospondin type 1 mot 13, A disintegrin like and metalloprotease with thrombospondin type 1 mot 13, ADAM metallopeptidase with thrombospondin type 1 mot 13, ADAM TS 13, ADAM TS13, ADAM-TS 13, ADAM-TS13, ADAMTS 13, ADAMTS-13, ADAMTS13, ADAMTS13 protein, ATS13_HUMAN, C9orf8, TTP, von Willebrand factor-cleaving protease, vWF cleaving protease, vWF CP, vWF-cleaving protease, vWF-CP, vWFCP.
This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene is the von Willebrand Factor (vWF)-cleaving protease, which is responsible for cleaving at the site of Tyr842-Met843 of the vWF molecule. A deficiency of this enzyme is associated with thrombotic thrombocytopenic purpura. Alternative splicing of this gene generates multiple transcript variants encoding different isoforms. [provided by RefSeq, Nov 2008].
