ADAMTS13 is 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 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.
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Protein Aliases: a disintegrin and metalloproteinase; A disintegrin and metalloproteinase with thrombospondin motifs 13; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13; ADAM; ADAM metallopeptidase with thrombospondin type 1 motif, 13; ADAM-TS 13; ADAMs; metalloendopeptidases; von Willebrand factor-cleaving protease; vWF-cleaving protease; vWF-CP
Gene Aliases: ADAM-TS13; ADAMTS-13; ADAMTS13; C9orf8; UNQ6102/PRO20085; vWF-CP; VWFCP
UniProt ID: (Human) Q76LX8
Entrez Gene ID: (Human) 11093
Molecular Function: metalloprotease