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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None of these four patients had detectable ADAMTS-13 antibodies.
The moderately low level of plasma ADAMTS-13 reported in some of these patients as well as the predominance of renal disease is perhaps more suggestive of atypical HUS rather than classical TTP.
Plasma ADAMTS-13 activity measured by enzyme-linked immunosorbent assay was reduced.
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.
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.
The basic pathophysiology is due to a deficient or dysfunctional protease ADAMTS-13.
The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency.
vWF-CP is a member of the ADAMTS family of metalloprotease enzymes and is designated ADAMTS-13.
4-6) Unlike our case, two previously reported cases had low ADAMTS-13 activity and increased ADAMTS13 inhibitor level, (4,5) while in the two other cases ADAMTS-13 levels were not determined.
ADAMTS-13 activity, microangiopathic haemolytic anaemia and thrombocytopaenia following snake bite envenomation.
However, the disorders appear to be different entities, with TTP associated with a severe deficiency of the von Willebrand factor cleaving protease ADAMTS-13, and familial and some sporadic cases of HUS associated with a deficiency of complement regulatory proteins.
Detection of the proteolytic enzyme ADAMTS-13 may be used to differentiate between the forms of thrombotic microangiopathy (TMA), thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS).