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Description for Protein ADAMTS13

ADAM metallopeptidase with thrombospondin type 1 motif, 13
1 total interacting proteins; 1 platelet interacting proteins
Icon Book Platelet Evidence (proteome studies/others : 0/1)
(undefined: 1)
Summary:
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] (PubMed Links)
Domains and Motifs:
  • TSP1: Thrombospondin type 1 repeats
  • ACR: ADAM Cysteine-Rich Domain
  • REP: Replication protein
  • SP: Signal Peptide
    • Gene Ontology:
    Gene Ontology Annotations
    KEGG - Enzyme ID(s):
    3.4.24.-
    KEGG - Orthology:
    K08627
    KEGG - Pathway(s):
    None Available
    Nomenclature / Alternative Names:
    A disintegrin like and metalloprotease with thrombospondin type 1 motif, 13; Von Willebrand factor cleaving protease; VWFCP; A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13 isoform 2 preproprotein; A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13 isoform 3 preproprotein; ADAM metallopeptidase with thrombospondin type 1 motif, 13 isoform 1 preproprotein
    Approved Symbol:
    ADAMTS13
    (De-) Phosphorylations:
    Total (de-) phosphorylation sites: 0
    No human (de-) phosphorylation sites; No platelet phosphorylation sites
    Phosphorylation Targets:
    Total phosphorylation targets: 0
    Human phosphorylation targets: 0;Predicted platelet targets: 0
    		Protein Characteristics:
    Isoform-specific Information
    Icon DrugsAssociated Drugs (DrugBank Accession):

    None Available

    Associated Genetic Diseases:

  • Thrombotic thrombocytopenic purpura, congenital(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 4 : 1
  • Isoform 3 : 1
  • Isoform 2 : 1
  • Isoform 1 : 1
    • Additional Identifiers:

    HPRD: 04994 Entrez Gene ID: 11093 OMIM ID: 604134 Swissprot Accession: Q76LX8; B3KWF7;