Deficient activity of von Willebrand factor-cleaving protease in patients with Upshaw-Schulman syndrome (Q33338779)

25 July 2017

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

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25 July 2017

Y Sasahara

1

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25 July 2017

S Kumaki

2

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25 July 2017

Y Ohashi

3

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25 July 2017

M Minegishi

4

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25 July 2017

H Kano

5

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25 July 2017

F Bessho

6

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25 July 2017

S Tsuchiya

7

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25 July 2017

1 July 2001

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International Journal of Hematology

25 July 2017

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25 July 2017

74

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25 July 2017

1

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25 July 2017

109-114

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https://scigraph.springernature.com/pub.10.1007/bf02982559

25 July 2017

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cites work

Binding of factor VIII to von willebrand factor is enabled by cleavage of the von Willebrand factor propeptide and enhanced by formation of disulfide-linked multimers.

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12 December 2020

Thrombotic thrombocytopenic purpura: understanding a disease no longer rare

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12 December 2020

Inducible secretion of large, biologically potent von Willebrand factor multimers

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12 December 2020

The collagen-platelet interaction

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12 December 2020

Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.

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12 December 2020

von Willebrand factor proteolysis is deficient in classic, but not in bone marrow transplantation-associated, thrombotic thrombocytopenic purpura.

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12 December 2020

Shear stress enhances the proteolysis of von Willebrand factor in normal plasma

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12 December 2020

Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation

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12 December 2020

Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura

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12 December 2020

Studies on thrombopoiesis. I. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency

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12 December 2020

Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura

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12 December 2020

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

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12 December 2020

Congenital microangiopathic hemolytic anemia: report of a Japanese girl

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12 December 2020

von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

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12 December 2020

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

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12 December 2020

Chronic relapsing thrombotic thrombocytopenic purpura in infants with large von Willebrand factor multimers during remission

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12 December 2020

Triplet structure of von Willebrand factor reflects proteolytic degradation of high molecular weight multimers

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12 December 2020

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

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12 December 2020

Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis.

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12 December 2020

Chronic relapsing thrombotic thrombocytopenic purpura: three case reports and update of the pathogenesis and therapeutic modalities

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF02982559

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Dimensions Publication ID

25 July 2017

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