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Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies
TLDR
The consensus defines diagnosis, disease monitoring and response to treatment in thrombotic microangiopathies and TTP and requirements for ADAMTS‐13 are given. Expand
Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity
TLDR
A single-nucleotide polymorphism associated with alterations in VWF-CP activity is reported and it will be important to assess this single- nucleotide SNP as a risk factor for thrombotic disorders. Expand
Localization of ADAMTS13 to the stellate cells of human liver.
TLDR
Results suggest that HSCs may be major cells producing ADAMTS13 in human liver, with positive signals exclusively in perisinusoidal cells with irregularly elongated dendritic processes extending between hepatocytes. Expand
Molecular characterization of L-amino acid oxidase from Agkistrodon halys blomhoffii with special reference to platelet aggregation.
TLDR
Results suggest that M-LAO inhibits the interaction between activated platelet integrin alphaIIb/beta3 and fibrinogen through the continuous generation of H2O2, and may contribute to prolonged bleeding from the vessels at snake bite sites. Expand
VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13.
TLDR
ADAMTS-13 was recently identified as a new hemostatic factor, von Willebrand factor (VWF)-cleaving protease, and data suggested that a minimal region as a functional substrate consisted of 73 amino acid residues from D1596 to R1668 of VWF, designated VWF73, and that further deletion of the E1660-R1668 region led to the loss of cleavage by ADAMts-13. Expand
Novel monoclonal antibody‐based enzyme immunoassay for determining plasma levels of ADAMTS13 activity
TLDR
A unique and highly sensitive enzyme immunoassay (EIA) of ADAMTS13 activity is described and is described as a prerequisite for a differential diagnosis of thrombotic microangiopathies. Expand
Pregnancy‐induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw–Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients
TLDR
Nine women diagnosed with Upshaw–Schulman syndrome who were diagnosed during their first pregnancy had severely deficient ADAMTS13 activity, emphasizing the importance of measuring ADAMts13 activity in the evaluation of thrombocytopenia during childhood and pregnancy. Expand
Natural history of Upshaw–Schulman syndrome based on ADAMTS13 gene analysis in Japan
TLDR
Results indicate that ‘the late‐onset phenotype’ of USS is formed with ethnic specificity, and two of these three males developed sudden overt TTP when they were 55 and 63’years old, respectively. Expand
Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF‐cleaving protease activity
TLDR
Although the survival of patients with TTP has been dramatically improved with plasma exchange (PE), there are still many patients who are refractory to PE and immunosuppressive therapy. Expand
Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.
TLDR
Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13: AC, and rapid ADAMts13:AC assays are therefore prerequisite to appropriately treat T MA patients. Expand
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