NP_620596.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
NCBI Official Full Name
A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 2 preproprotein
NCBI Official Synonym Full Names
ADAM metallopeptidase with thrombospondin type 1 motif, 13
NCBI Official Synonym Symbols
TTP; VWFCP; C9orf8; vWF-CP; FLJ42993; MGC118899; MGC118900; DKFZp434C2322 [Similar Products]
NCBI Protein Information
A disintegrin and metalloproteinase with thrombospondin motifs 13; ADAM-TS13; ADAMTS-13; ADAM-TS 13; OTTHUMP00000022496; OTTHUMP00000022497; OTTHUMP00000022498; vWF-cleaving protease; von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13
UniProt Protein Name
Von Willebrand factor-cleaving protease
UniProt Entry Name
Q6QNA4_HUMAN
NCBI Summary for ADAMTS13
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]
UniProt Comments for ADAMTS13
ADAMTS13: Cleaves the vWF multimers in plasma into smaller forms. Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP); also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Extracellular matrix; EC 3.4.24.87; Protease; Motility/polarity/chemotaxis; Calcium-binding; Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 9q34
Cellular Component: extracellular space; proteinaceous extracellular matrix; cell surface; endoplasmic reticulum lumen
Molecular Function: integrin binding; protein binding; metallopeptidase activity; zinc ion binding; metalloendopeptidase activity; calcium ion binding
Biological Process: integrin-mediated signaling pathway; protein amino acid O-linked glycosylation; platelet activation; cellular protein metabolic process; glycoprotein metabolic process; peptide catabolic process; response to toxin; cell-matrix adhesion; protein processing; proteolysis; post-translational protein modification
Disease: Thrombotic Thrombocytopenic Purpura, Congenital
Research Articles on ADAMTS13
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Products associated with anti-ADAMTS13 antibody
Organs/Tissues associated with anti-ADAMTS13 antibody
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