ADAMTS13

Also known as ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP, ADAM metallopeptidase with thrombospondin type 1 motif 13

ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. It is secreted into the blood and degrades large vWf multimers, decreasing their activity, hence ADAMTS13 acts to reduce thrombus formation.

In the Vinony graph

Vinony's link graph records 162 inbound references to ADAMTS13, and connects out to Q180686, Online Mendelian Inheritance in Man and Ensembl genome database project.

It sits within the topics ADAMTS, EC 3.4.24 and Genes on human chromosome 9.

Vinony links it to 11 Wikipedia language editions.

Gene data

ADAMTS13

Name: ADAM metallopeptidase with thrombospondin type 1 motif 13
Type: protein-coding
Position: 133,414,358–133,459,402 (+)
Aliases: ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP
Ensembl: ENSG00000160323
RefSeq RNA: NM_139025.5, NM_139026.6, NM_139027.6, NM_139028.2, NR_024514.3
RefSeq protein: NP_620594.1, NP_620595.1, NP_620596.2, XP_011516478.1, XP_011516480.1

This gene encodes a member of a family of proteins containing several distinct regions, including a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. The enzyme encoded by this gene specifically cleaves von Willebrand Factor (vWF). Defects in this gene are associated with thrombotic thrombocytopenic purpura. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013].