The ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) are a family of proteases that include 19 members. ADAMTS are closely related to the ADAM (a disintegrin and metalloproteinase) subfamily of metalloendopeptidases, sharing similar metalloproteinase domains. ADAMTS are secreted proteases that are involved in matrix proteoglycan cleavage, processing of collagen, and regulating processes such as angiogenesis and hemostasis (Porter et al. 2005).
ADAMTS13 is a protein of 1,427 amino acids with a deduced molecular weight of 190 kDa (Levy et al. 2001). ADAMTS13 was originally identified as a von Willebrand factor (vWF)-cleaving protease (vWF-cp), later discovered to be a novel member of the ADAMTS family. Following genome-wide linkage analysis, the ADAMTS13 gene was located on the human chromosome 9q34 (Levy...
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