ADAMTSbd13

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ADAMTSbd13

An ADAMTSs family enzyme encoded by ADAMTS13 on chromosome 9q34, which is identical to vWFCP (von Willebrand factor-cleaving protease, the name by which it is more commonly known in haematology). ADAMTS13/vWFCP degrades large vWF multimers by cleaving monomeric subunits, the absence of which (due to mutations) causes thrombotic thrombocytopenic purpura.
ADAMTS13 mRNA is expressed in liver, placenta, ovary, various other tissues, and in platelets.
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In cancer-associated TTP, which could be a complication resulting from chemotherapy or a manifestation of cancer itself, (3) ADAMTS-13 level is normal and the condition is likely the result of an increased tumor cell load, which leads to endothelial damage and fragmentation of red blood cells (RBC) as they traverse the injured microvasculature.
3,4 In physiological states, ULVWF released by the endothelium induce platelet aggregation in areas of high shear stress, but this only occurs briefly as VWF is rapidly broken down by ADAMTS-13.
Plasma ADAMTS-13 activity measured by enzyme-linked immunosorbent assay was reduced.
All patients with a clinical diagnosis of thrombotic thrombocytopenic purpura (TTP) with severe ADAMTS-13 deficiency (activity less than 10% of normal) admitted to the Louisiana State University Medical Center from 2008-2010 were included in the study.
Analysis of familial thrombotic thrombocytopenic purpura patients showed that there are mutations in the ADAMTS-13 gene that lead to functional defects in the ADAMTS-13 enzyme.
ADAMTS-13 is the plasma ultra large Von Willebrand Factor (ULVWF)-cleaving metalloprotease that severs the highly adhesive long ULVWF multimeric strings, thus preventing adhesion and aggregation of platelets (Moake, 2007).
The basic pathophysiology is due to a deficient or dysfunctional protease ADAMTS-13.
Another advantage is that the book contains recommendations that are in synch with the most recent practice recommendations, such as when describing management of warfarin overdose or discussing the role of ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) testing for the diagnosis of vWD.