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ADAMTS

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AcronymDefinition
ADAMTSA Disintegrin-Like and Metalloproteinase with Thrombospondin


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Joints gradually breaks down and a key component of cartilage called aggrecan, which helps the tissue bear load and resist compression, is chewed up by enzymes of the ADAMTS or aggrecanase family.
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura.
In patients with TTP, absent or severely reduced activity of ADAMTS 13 prevents timely cleavage of unusually large multimers of von Willebrand factor as they are secreted by endothelial cells.
 
 
 
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