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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. Note: This description may include information from UniProtKB.
Protein type: Calcium-binding; EC; Extracellular matrix; Motility/polarity/chemotaxis; Protease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 9q34.2
Cellular Component: endoplasmic reticulum lumen; proteinaceous extracellular matrix
Molecular Function: calcium ion binding; integrin binding; metallopeptidase activity; protein binding; zinc ion binding
Biological Process: peptide catabolic process; protein processing; proteolysis
Disease: Thrombotic Thrombocytopenic Purpura, Congenital
Reference #:  Q76LX8 (UniProtKB)
Alt. Names/Synonyms: A disintegrin and metalloproteinase with thrombospondin motifs 13; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13; ADAM metallopeptidase with thrombospondin type 1 motif, 13; ADAM-TS 13; ADAM-TS13; ADAMTS-13; ADAMTS13; ATS13; C9orf8; DKFZp434C2322; FLJ42993; MGC118899; MGC118900; TTP; von Willebrand factor-cleaving protease; vWF-cleaving protease; vWF-CP; VWFCP
Gene Symbols: ADAMTS13
Molecular weight: 153,604 Da
Basal Isoelectric point: 6.96  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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