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.

scholarly journals Human Complement Factor H Is a Reductase for Large Soluble von Willebrand Factor Multimers—Brief Report

2013 ◽  
Vol 33 (11) ◽  
pp. 2524-2528 ◽  
Author(s):  
Leticia Nolasco ◽  
Jennifer Nolasco ◽  
Shuju Feng ◽  
Vahid Afshar-Kharghan ◽  
Joel Moake
Keyword(s):  
Von Willebrand Factor ◽  
Factor H ◽  
Complement Factor H ◽  
Complement Factor ◽  
Human Complement ◽  
Von Willebrand ◽  
Willebrand Factor

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 Analysis focusing on plasma von Willebrand factor in pachychoroid neovasculopathy and age-related macular degeneration

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hiromasa Hirai ◽  
Mariko Yamashita ◽  
Masanori Matsumoto ◽  
Masaki Hayakawa ◽  
Kazuya Sakai ◽  
...  
Keyword(s):  
Macular Degeneration ◽  
Von Willebrand Factor ◽  
Factor H ◽  
Age Related Macular Degeneration ◽  
Complement Factor ◽  
Clinical Criteria ◽  
Age Related ◽  
Pachychoroid Neovasculopathy ◽  
Von Willebrand ◽  
Willebrand Factor

AbstractPachychoroid neovasculopathy (PNV) is a new concept of macular disorder. Some cases diagnosed as age-related macular degeneration (AMD) have been re-diagnosed as PNV. However, the biological features of PNV are still uncertain. The purpose of this study was to compare PNV and AMD by analyses focusing on von Willebrand factor (VWF) and complement factor H (CFH). Ninety-seven patients who were previously diagnosed with treatment naïve AMD were enrolled in this study. They were re-classified as either PNV or AMD based on the clinical criteria and 33 patients were classified as PNV and 64 patients as AMD. We examined the clinical data, analyzed VWF multimer and two genetic polymorphisms (I62V and Y402H) in the CFH. PNV group was significantly younger than AMD group (P = 0.001). In both I62V and Y402H, there were no significant differences between PNV and AMD while the recessive homozygous (AA) was found only in PNV group in I62V. The presence of unusually large VWF multimers (UL-VWFMs) and subretinal hemorrhages were significantly higher in PNV than in AMD (P = 0.045, P = 0.020, respectively). Thus, the residual UL-VWFMs may result in platelet thrombosis and hemorrhages in the choriocapillaris of PNV. In conclusion, our results suggest the biological differences between PNV and AMD.

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 von Willebrand factor is a cofactor in complement regulation

Blood ◽  
2015 ◽  
Vol 125 (6) ◽  
pp. 1034-1037 ◽  
Author(s):  
Shuju Feng ◽  
Xiaowen Liang ◽  
Michael H. Kroll ◽  
Dominic W. Chung ◽  
Vahid Afshar-Kharghan
Keyword(s):  
Von Willebrand Factor ◽  
Complement Activation ◽  
Complement Regulation ◽  
Factor I ◽  
Normal Plasma ◽  
Key Points ◽  
Cofactor Activity ◽  
Von Willebrand ◽  
Willebrand Factor

Key Points Normal plasma VWF multimers act as a cofactor in the factor I–mediated cleavage of C3b to iC3b and inhibit complement activation. Large VWF multimers, including ultra-large VWF multimers, do not have factor I cofactor activity and permit complement activation.

scholarly journals Ibrutinib treatment affects collagen and von Willebrand factor-dependent platelet functions

Blood ◽  
2014 ◽  
Vol 124 (26) ◽  
pp. 3991-3995 ◽  
Author(s):  
Marie Levade ◽  
Elodie David ◽  
Cédric Garcia ◽  
Pierre-Alexandre Laurent ◽  
Sarah Cadot ◽  
...  
Keyword(s):  
Platelet Aggregation ◽  
Shear Rate ◽  
Platelet Activation ◽  
Von Willebrand Factor ◽  
Bleeding Diathesis ◽  
Key Points ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Platelet Functions

Key Points Ibrutinib affects collagen and VWF-mediated platelet activation. The bleeding diathesis correlates with defects in collagen-induced platelet aggregation and firm adhesion on VWF at arterial shear rate.

Von Willebrand Factor interacts with Factor H and enhances its cofactor activity

2013 ◽  
Vol 56 (3) ◽  
pp. 251
Author(s):  
J. Rayes ◽  
L. Roumenina ◽  
Y. Repressé ◽  
J. Dimitrov ◽  
M. Ing ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Factor H ◽  
Cofactor Activity ◽  
Von Willebrand ◽  
Willebrand Factor

scholarly journals Adhesion of Staphylococcus aureus to the vessel wall under flow is mediated by von Willebrand factor–binding protein

Blood ◽  
2014 ◽  
Vol 124 (10) ◽  
pp. 1669-1676 ◽  
Author(s):  
Jorien Claes ◽  
Thomas Vanassche ◽  
Marijke Peetermans ◽  
Laurens Liesenborghs ◽  
Christophe Vandenbriele ◽  
...  
Keyword(s):  
Staphylococcus Aureus ◽  
Endothelial Cells ◽  
Von Willebrand Factor ◽  
Vessel Wall ◽  
Binding Protein ◽  
Factor Binding ◽  
Key Points ◽  
Platelet Aggregates ◽  
Von Willebrand ◽  
Willebrand Factor

Key PointsvWbp mediates adhesion of S aureus under flow to activated endothelial cells and the subendothelium via VWF. vWbp activates prothrombin and triggers the formation of bacteria–fibrin–platelet aggregates, which enhance adhesion to vessels under flow.

scholarly journals Analysis Focusing on Plasma Von Willebrand Factor in Pachychoroid Neovasculopathy and Age-related Macular Degeneration

2021 ◽  
Author(s):  
Hiromasa Hirai ◽  
Mariko Yamashita ◽  
Masanori Matsumoto ◽  
Masaki Hayakawa ◽  
Kazuya Sakai ◽  
...  
Keyword(s):  
Macular Degeneration ◽  
Von Willebrand Factor ◽  
Factor H ◽  
Age Related Macular Degeneration ◽  
Complement Factor ◽  
Clinical Criteria ◽  
Age Related ◽  
Pachychoroid Neovasculopathy ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract Pachychoroid neovasculopathy (PNV) is a new disease concept. Some cases diagnosed as age-related macular degeneration (AMD) have been re-diagnosed as PNV. However, the biological features of PNV are still uncertain. The purpose of this study was to compare PNV and AMD by an analysis focusing on von Willebrand factor (VWF) and complement factor H (CFH). Ninety-seven patients who were previously diagnosed with treatment naïve AMD were enrolled in this study. They were re-classified PNV and AMD based on the clinical criteria and 33 patients were classified as PNV and 64 patients as AMD. We examined the clinical data and performed VWF multimer analysis and genetic polymorphisms in the CFH. PNV group was significantly younger than AMD group. The presence of unusually large VWF multimers (UL-VWFMs) and subretinal hemorrhage was significantly higher in PNV than in AMD. In the CFH I62V, allele A tended to be more frequent in PNV group and recessive homozygous (AA) was found only in PNV group. Thus, the genetic alternations in CFH may result in the residual UL-VWFMs and platelet thrombosis in the choriocapillaris of PNV. In conclusion, our results suggest the biological differences between PNV and AMD.

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