Identification and characterization of the elusive mutation causing the historical von Willebrand Disease type IIC Miami.

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Identification and characterization of the elusive mutation causing the historical von Willebrand Disease type IIC Miami.

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http://www.wikidata.org/entity/Q39655365

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2016 nî lūn-bûn

@nan

2016年の論文

@ja

2016年論文

@yue

2016年論文

@zh-hant

2016年論文

@zh-hk

2016年論文

@zh-mo

2016年論文

@zh-tw

2016年论文

@wuu

2016年论文

@zh

2016年论文

@zh-cn

name

Identification and characteriz ...... ebrand Disease type IIC Miami.

@en

Identification and characteriz ...... ebrand Disease type IIC Miami.

@nl

type

Item

label

Identification and characteriz ...... ebrand Disease type IIC Miami.

@en

Identification and characteriz ...... ebrand Disease type IIC Miami.

@nl

prefLabel

Identification and characteriz ...... ebrand Disease type IIC Miami.

@en

Identification and characteriz ...... ebrand Disease type IIC Miami.

@nl

P1476

Identification and characteriz ...... ebrand Disease type IIC Miami.

@en

P2093

F Oyen

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J E Sadler

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M Ledford-Kraemer

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R Marschalek

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R R Montgomery

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R Schneppenheim

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S Schneppenheim

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T Obser

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U Budde

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P2860

Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP)

The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF.

Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor.

von Willebrand factor is dimerized by protein disulfide isomerase.

Quantitative assay of a plasma factor deficient in von Willebrand's disease that is necessary for platelet aggregation. Relationship to factor VIII procoagulant activity and antigen content

Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE).

Endothelial cell synthesis of von Willebrand antigen II, von Willebrand factor, and von Willebrand factor/von Willebrand antigen II complex.

Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease

Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity.

Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC)

P304

1725-1735

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scholarly article

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10.1111/JTH.13398

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Cecile Denis

Maria A Brehm

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2016-06-25T00:00:00Z

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27344059

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5035592

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