scholarly journals Defective RAB1B-related megakaryocytic ER-to-Golgi transport in RUNX1 haplodeficiency: impact on von Willebrand factor

2018 ◽  
Vol 2 (7) ◽  
pp. 797-806 ◽  
Author(s):  
Gauthami Jalagadugula ◽  
Lawrence E. Goldfinger ◽  
Guangfen Mao ◽  
Michele P. Lambert ◽  
A. Koneti Rao
Keyword(s):  
Von Willebrand Factor ◽  
Direct Transcriptional Target ◽  
Golgi Transport ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Transcriptional Target

Key Points GTPase RAB1B is a direct transcriptional target of RUNX1 in MK/platelets and is downregulated in RUNX1 haplodeficiency. RUNX1 downregulation is associated with defective RAB1B-related ER-to-Golgi transport and alterations in α-granule vWF.

scholarly journals von Willebrand factor multimerization and the polarity of secretory pathways in endothelial cells

Blood ◽  
2016 ◽  
Vol 128 (2) ◽  
pp. 277-285 ◽  
Author(s):  
Mafalda Lopes da Silva ◽  
Daniel F. Cutler
Keyword(s):  
Endothelial Cells ◽  
Von Willebrand Factor ◽  
Secretory Pathways ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points The 3 endothelial secretory pathways—constitutive, basal, and regulated—release VWF in different multimeric states. Apical- and basolaterally-released VWF follow different secretory pathways, thus releasing differentially multimerized protein.

scholarly journals von Willebrand factor and factor VIII levels after desmopressin are associated with bleeding phenotype in type 1 VWD

2019 ◽  
Vol 3 (24) ◽  
pp. 4147-4154 ◽  
Author(s):  
Ferdows Atiq ◽  
Lisette M. Schütte ◽  
Agnes E. M. Looijen ◽  
Johan Boender ◽  
Marjon H. Cnossen ◽  
...  
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points VWF and FVIII levels after desmopressin, which mimic hemostatic response, are associated with the bleeding phenotype of type 1 VWD patients. Variability in VWF and FVIII response to hemostatic challenges may partly explain heterogeneity in bleeding phenotype of VWD patients.

cAMP-induced secretion of endothelial von Willebrand factor is regulated by a phosphorylation/dephosphorylation switch in annexin A2

Blood ◽  
2013 ◽  
Vol 122 (6) ◽  
pp. 1042-1051 ◽  
Author(s):  
Ines Brandherm ◽  
Jennifer Disse ◽  
Dagmar Zeuschner ◽  
Volker Gerke
Keyword(s):  
Complex Formation ◽  
Von Willebrand Factor ◽  
Annexin A2 ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Secretion

Key Points A calcineurin-like phosphatase dephosphorylates annexin A2 in the course of cAMP-induced Weibel-Palade body exocytosis. Dephosphorylation at serine 11 of annexin A2 triggers complex formation with S100A10 that is required for von Willebrand factor secretion.

scholarly journals High-density lipoprotein modulates thrombosis by preventing von Willebrand factor self-association and subsequent platelet adhesion

Blood ◽  
2016 ◽  
Vol 127 (5) ◽  
pp. 637-645 ◽  
Author(s):  
Dominic W. Chung ◽  
Junmei Chen ◽  
Minhua Ling ◽  
Xiaoyun Fu ◽  
Teri Blevins ◽  
...  
Keyword(s):  
High Density Lipoprotein ◽  
Von Willebrand Factor ◽  
Platelet Adhesion ◽  
Density Lipoprotein ◽  
High Density ◽  
New Approach ◽  
Key Points ◽  
Self Association ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points High-density lipoprotein and its major apolipoprotein ApoA-I prevent von Willebrand factor self-association. Targeting von Willebrand factor self-association could be a new approach to treating thrombotic disorders.

scholarly journals Free hemoglobin increases von Willebrand factor–mediated platelet adhesion in vitro: implications for circulatory devices

Blood ◽  
2015 ◽  
Vol 126 (20) ◽  
pp. 2338-2341 ◽  
Author(s):  
Qi Da ◽  
Miho Teruya ◽  
Prasenjit Guchhait ◽  
Jun Teruya ◽  
John S. Olson ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Platelet Adhesion ◽  
Free Hemoglobin ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points Extracellular Hb alters the GPIbα-VWF interaction.

scholarly journals Human neutrophil peptides inhibit cleavage of von Willebrand factor by ADAMTS13: a potential link of inflammation to TTP

Blood ◽  
2016 ◽  
Vol 128 (1) ◽  
pp. 110-119 ◽  
Author(s):  
Vikram G. Pillai ◽  
Jialing Bao ◽  
Catherine B. Zander ◽  
Jenny K. McDaniel ◽  
Palaniappan S. Chetty ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Human Neutrophil ◽  
Plasma Levels ◽  
Proteolytic Cleavage ◽  
Key Points ◽  
Potential Link ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points HNPs inhibit proteolytic cleavage of VWF by ADAMTS13 by physically blocking VWF-ADAMTS13 interactions. Plasma levels of HNP1, HNP2, and HNP3 are markedly increased in patients with acquired autoimmune TTP.

Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease

Blood ◽  
2013 ◽  
Vol 121 (14) ◽  
pp. 2762-2772 ◽  
Author(s):  
Jiong-Wei Wang ◽  
Eveline A. M. Bouwens ◽  
Maria Carolina Pintao ◽  
Jan Voorberg ◽  
Huma Safdar ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Von Willebrand Factor ◽  
Von Willebrand Disease ◽  
Key Points ◽  
Phenotypic Features ◽  
String Formation ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Multiple Patients

Key Points Isolation of BOECs from multiple patients with VWD is feasible, and the study of BOECs helps explain the pathogenic complexity of VWD. Abnormalities in WPB biogenesis and exocytosis and defects in VWF string formation correlate with the phenotypic features of patients with VWD.

scholarly journals Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status

Blood ◽  
2014 ◽  
Vol 124 (5) ◽  
pp. e1-e3 ◽  
Author(s):  
Adrienne Lee ◽  
Gary Sinclair ◽  
Karen Valentine ◽  
Paula James ◽  
Man-Chiu Poon
Keyword(s):  
Von Willebrand Factor ◽  
Acquired Von Willebrand Syndrome ◽  
Remission Status ◽  
Von Willebrand Factor Antigen ◽  
Key Points ◽  
Relapsing Remitting ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen ◽  
Von Willebrand Factor Propeptide

Key Points Remission status in relapsing-remitting AVWS depends on the balance of VWF clearance by anti-VWF antibody and VWF secretion. VWFpp:Ag ratio is a simple assay that provides information on this balance and predicts remission status in this case of AVWS.

Active von Willebrand factor predicts 28-day mortality in patients with systemic inflammatory response syndrome

Blood ◽  
2014 ◽  
Vol 123 (14) ◽  
pp. 2153-2156 ◽  
Author(s):  
Agon Hyseni ◽  
Hans Kemperman ◽  
Dylan W. de Lange ◽  
Jozef Kesecioglu ◽  
Philip G. de Groot ◽  
...  
Keyword(s):  
Systemic Inflammatory Response Syndrome ◽  
Inflammatory Response ◽  
Von Willebrand Factor ◽  
Systemic Inflammatory Response ◽  
Glycoprotein Ib ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Apache Iv

Key Points von Willebrand factor in its glycoprotein Ib conformation predicts 28-day mortality and could be a useful addition to the APACHE-IV score.

The interaction between factor H and VWF increases factor H cofactor activity and regulates VWF prothrombotic status

Blood ◽  
2014 ◽  
Vol 123 (1) ◽  
pp. 121-125 ◽  
Author(s):  
Julie Rayes ◽  
Lubka T. Roumenina ◽  
Jordan D. Dimitrov ◽  
Yohann Repessé ◽  
Mathieu Ing ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Platelet Aggregation ◽  
Von Willebrand Factor ◽  
Factor H ◽  
Complement Factor H ◽  
Complement Factor ◽  
Key Points ◽  
Cofactor Activity ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points Complement factor H and von Willebrand factor colocalize in the Weibel-Palade bodies of endothelial cells and interact in normal plasma. Formation of the complex enhances FH cofactor activity and VWF-mediated platelet aggregation.

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