|Synonym||von Willebrand-factor cleaving protease (vWF CLP)|
|Molecular mass||153.000 Da|
|Plasma concentration||0.60 - 1.60 µg/ml|
|Normal range||50 - 150%|
The von Willebrand factor cleaving protease belongs to a family of zinc-containing metalloproteases. ADAMTS-13 stands for a desintegrin and metalloprotease with thrombospondin-1-like domains 13. ADAMTS 13 regulates the size and thereby indirectly the biological activity of the von Willebrand factor. Defect in or deficiency of this enzyme leads to unusually large vWF-molecules (UL-vWF) and thereby to an increase in platelet aggregation.
A high concentration of oversized von Willebrand factor molecules can lead to thrombotic microangiopathy. Some examples of such disorders are the thrombotic thrombocytopenic purpura (TTP, Moschcowitz syndrome), the haemolytic-uraemic syndrome and the HELLP-syndrome. These disease states stem from a disturbance in the blood flow in arterioles and capillaries. In modern TTP diagnoses an ADAMTS-13 functional disturbance is considered decisive.
The most common form of idiopathic TTP is an autoimmune disorder of unknown reasons, with autoantibodies directed against ADAMTS-13. Additionally, a hereditary form also exists (Upshaw-Schulman-syndrome), where a genetic defect on chromosome 9 hinders a correct ADAMTS-13 synthesis. Approximately 40 different mutations are known. Further trigging factors for TTP include pregnancy, bone marrow transplantation, pharmaceutical drugs or infectious diseases.
Differential diagnosis for causative determinations of thrombotic microangiopathies.
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