AAL17652.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
39,864 Da
NCBI Official Full Name
von Willebrand factor-cleaving protease
NCBI Official Synonym Full Names
ADAM metallopeptidase with thrombospondin type 1 motif, 13
NCBI Official Synonym Symbols
VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13 [Similar Products]
NCBI Protein Information
A disintegrin and metalloproteinase with thrombospondin motifs 13
UniProt Protein Name
A disintegrin and metalloproteinase with thrombospondin motifs 13
UniProt Synonym Protein Names
von Willebrand factor-cleaving protease; vWF-CP; vWF-cleaving protease
UniProt Synonym Gene Names
C9orf8; ADAM-TS 13; ADAM-TS13; ADAMTS-13; vWF-CP; vWF-cleaving protease [Similar Products]
UniProt Entry Name
ATS13_HUMAN
NCBI Summary for ADAMTS13/VWFCP
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]
UniProt Comments for ADAMTS13/VWFCP
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: Secreted; Motility/polarity/chemotaxis; Secreted, signal peptide; EC 3.4.24.87; Calcium-binding; Extracellular matrix; Protease
Chromosomal Location of Human Ortholog: 9q34
Cellular Component: extracellular space; proteinaceous extracellular matrix; cell surface; endoplasmic reticulum lumen
Molecular Function: integrin binding; protein binding; zinc ion binding; metallopeptidase activity; 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; cell-matrix adhesion; response to toxin; protein processing; proteolysis; post-translational protein modification
Disease: Thrombotic Thrombocytopenic Purpura, Congenital
Research Articles on ADAMTS13/VWFCP
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Products associated with ADAMTS13/VWFCP clia kit
Pathways associated with ADAMTS13/VWFCP clia kit
Organs/Tissues associated with ADAMTS13/VWFCP clia kit
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