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: EC 22.214.171.124; EC 3.4.24.-; Secreted, signal peptide; Protease; Motility/polarity/chemotaxis; Secreted; Calcium-binding protein; Extracellular matrix
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
SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.