(Q33396314)

26 July 2017

1 reference

title

Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan (English)

1 reference

26 July 2017

1 reference

Y Fujimura

1

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26 July 2017

M Matsumoto

2

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26 July 2017

A Isonishi

3

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26 July 2017

H Yagi

4

1 reference

26 July 2017

K Kokame

5

1 reference

26 July 2017

K Soejima

6

1 reference

26 July 2017

M Murata

7

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26 July 2017

T Miyata

8

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26 July 2017

1 July 2011

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Journal of Thrombosis and Haemostasis

26 July 2017

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26 July 2017

9 Suppl 1

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26 July 2017

283-301

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Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome

26 July 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

aHUS caused by complement dysregulation: new therapies on the horizon

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Clinical practice. Thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Thrombotic Microangiopathies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Partial amino acid sequence of purified von Willebrand factor-cleaving protease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Pathophysiology of thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Fibronectin levels in congenital thrombocytopenia: Schulman's syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Association between ADAMTS13 polymorphisms and risk of cardiovascular events in chronic coronary disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Absence of Pro475Ser polymorphism in ADAMTS-13 in Caucasians

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

ADAMTS13 P475S polymorphism causes a lowered enzymatic activity and urea lability in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Plasma of patients with Upshaw-Schulman syndrome, a congenital deficiency of von Willebrand factor-cleaving protease activity, enhances the aggregation of normal platelets under high shear stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Efficacy of several plasma components in a young boy with chronic thrombocytopenia and hemolytic anemia who responds repeatedly to normal plasma infusions

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Congenital microangiopathic hemolytic anemia: report of a Japanese girl

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Deficient activity of von Willebrand factor-cleaving protease in patients with Upshaw-Schulman syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

ADAMTS13 gene mutation in congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Congenital thrombotic thrombocytopenic purpura associated with unilateral moyamoya disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

Quantitative Western blot analysis of plasma ADAMTS13 antigen in patients with Upshaw-Schulman syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1538-7836.2011.04341.X

10.1111/J.1538-7836.2011.04341.X

21 January 2018

Identifiers

DOI

1 reference

26 July 2017

1 reference