ADAMTS13
ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif 13, also known as VWFCP or vWF-CP) is a secreted metalloproteinase that cleaves von Willebrand factor multimers in plasma. In doing so it controls vWF-mediated platelet thrombus formation. Browse Assay Genie's current range of ADAMTS13 research reagents below, or read on for the biology behind the target.
ADAMTS13 is a zinc-dependent metalloproteinase that acts on von Willebrand factor (vWF) in plasma. It cleaves vWF multimers into smaller forms, and through this activity it controls vWF-mediated platelet thrombus formation. The NCBI Gene summary describes the enzyme as specifically cleaving vWF, which makes it a focused model of how proteolytic processing regulates haemostasis. The protein is secreted, and its enzymatic activity is classified under EC 3.4.24.87.
The ADAMTS13 gene encodes a member of a family of proteins that share several distinct regions, including a metalloproteinase domain, a disintegrin-like domain and a thrombospondin type 1 (TS) motif. HGNC places it in the group of ADAM metallopeptidases with thrombospondin type 1 motif, alongside other ADAMTS proteases. Alternative splicing of the ADAMTS13 gene results in multiple transcript variants. The mouse ortholog is Adamts13.
The gene has carried several names. Earlier symbols and aliases include VWFCP, vWF-CP, TTP and C9orf8, and the previous gene name was a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13. The TTP alias reflects the disease association: defects in this gene are associated with thrombotic thrombocytopenic purpura. Researchers therefore study ADAMTS13 protein in work on vWF processing, platelet biology and thrombotic disorders.
ADAMTS13 cleaves vWF multimers in plasma into smaller forms, so it is central to studies of how vWF size is regulated.
By controlling vWF-mediated platelet thrombus formation, ADAMTS13 is a key subject in research on platelet adhesion and clot development.
Defects in the ADAMTS13 gene are associated with thrombotic thrombocytopenic purpura, making it a core target in TTP research.
Its metalloproteinase, disintegrin-like and thrombospondin type 1 motif domains make ADAMTS13 a reference point for comparing ADAMTS family proteases.
ADAMTS13 is a secreted metalloproteinase that cleaves von Willebrand factor multimers in plasma into smaller forms. This controls vWF-mediated platelet thrombus formation. The NCBI Gene summary notes that the enzyme specifically cleaves vWF, and its activity is classified as EC 3.4.24.87.
ADAMTS13 is also called VWFCP and vWF-CP, and the aliases TTP, C9orf8, FLJ42993, MGC118899, MGC118900 and DKFZp434C2322 appear in gene records. Its UniProt name is A disintegrin and metalloproteinase with thrombospondin motifs 13. Alternative splicing gives multiple transcript variants.
Defects in the ADAMTS13 gene are associated with thrombotic thrombocytopenic purpura, which explains the TTP alias. A mouse ortholog, Adamts13, exists, so rodent models are used alongside human work. Check the datasheet of any product for its validated reactivity.
Other ADAMTS family metalloproteinases that share the disintegrin-like and thrombospondin type 1 motif architecture with ADAMTS13.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q76LX8; product specifications from Assay Genie datasheets.