Results 1 to 10 of about 8,401,904 (201)

A novel approach to laboratory assessment and reporting of platelet von Willebrand factor

Platelets, 2022
The interaction of platelets with von Willebrand factor is essential for primary hemostasis. Concentration and activity of plasma von Willebrand factor are routine parameters in the assessment of hemostasis disorders. In addition to plasma von Willebrand
Thorsten Kragh, Isabell Pekrul, Helmut W. Ott, Michael Spannagl, Patrick Möhnle   +4 more
doaj   +1 more source

Cryptic non-canonical splice site activation is part of the mechanism that abolishes multimer organization in the c.2269_2270del von Willebrand factor

Haematologica, 2020
We report a new pathogenic mechanism in von Willebrand disease involving the use of a non-canonical splicing site. The proband, carrying the homozygous c.2269_2270del mutation previously classified as a type 3 mutation, showed severely reduced plasma and
Viviana Daidone, Eva Galletta, Luigi De Marco, Alessandra Casonato   +3 more
doaj   +1 more source

Evaluation of a new semi-automated Hydragel 11 von Willebrand factor multimers assay kit for routine use [PDF]

Journal of Medical Biochemistry, 2021
Background: Accurate diagnosis and classification of von Willebrand disease (VWD) are essential for optimal management. The von Willebrand factor multimers analysis (VWF:MM) is an integral part of the diagnostic process in the phenotypic classification ...
Pikta Marika, Szanto Timea, Viigimaa Margus, Lejniece Sandra, Balode Dārta, Saks Kadri, Banys Valdas   +6 more
doaj   +1 more source

Platelet-independent adhesion of calcium-loaded erythrocytes to von Willebrand factor. [PDF]

PLoS ONE, 2017
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage.
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk   +7 more
doaj   +1 more source

von Willebrand factor binds to the surface of dendritic cells and modulates peptide presentation of factor VIII

Haematologica, 2016
It has been proposed that von Willebrand factor might affect factor VIII immunogenicity by reducing factor VIII uptake by antigen presenting cells. Here we investigate the interaction of recombinant von Willebrand factor with immature monocyte-derived ...
Nicoletta Sorvillo, Robin B. Hartholt, Esther Bloem, Magdalena Sedek, Anja ten Brinke, Carmen van der Zwaan, Floris P. van Alphen, Alexander B. Meijer, Jan Voorberg   +8 more
doaj   +1 more source

An apparently silent nucleotide substitution (c.7056C>T) in the von Willebrand factor gene is responsible for type 1 von Willebrand disease

Haematologica, 2011
Background Nucleotide variations not changing protein sequences are considered silent mutations; accumulating data suggest that they can, however, be important in human diseases.Design and Methods We report an altered splicing process induced by a silent
Viviana Daidone, Lisa Gallinaro, Maria Grazia Cattini, Elena Pontara, Antonella Bertomoro, Antonio Pagnan, Alessandra Casonato   +6 more
doaj   +1 more source

Biogenesis of Weibel-Palade bodies in von Willebrand’s disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation

Haematologica, 2012
Background Mutations of cysteine residues in von Willebrand factor are known to reduce the storage and secretion of this factor, thus leading to reduced antigen levels. However, one cysteine mutation, p.Cys2773Ser, has been found in patients with type 2A(
Jiong-Wei Wang, Dafna J. Groeneveld, Guy Cosemans, Richard J. Dirven, Karine M. Valentijn, Jan Voorberg, Pieter H. Reitsma, Jeroen Eikenboom   +7 more
doaj   +1 more source

Principles of care for the diagnosis and treatment of von Willebrand disease

Haematologica, 2013
Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in ...
Giancarlo Castaman, Anne Goodeve, Jeroen Eikenboom   +2 more
doaj   +1 more source

The Molecular Genetics of von Willebrand Disease

Turkish Journal of Hematology, 2012
Quantitative and/or qualitative deficiency of von Willebrand factor (vWF) is associated with the most common inherited bleeding disease von Willebrand disease (vWD). vWD is a complex disease with clinical and genetic heterogeneity.
Ergül Berber
doaj   +1 more source

VWF/ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma

BMC Gastroenterology, 2019
Background To investigate the von Willebrand factor to ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma (HCC) in cirrhosis.
Hiroaki Takaya, Tadashi Namisaki, Mitsuteru Kitade, Kosuke Kaji, Keisuke Nakanishi, Yuki Tsuji, Naotaka Shimozato, Kei Moriya, Kenichiro Seki, Yasuhiko Sawada, Soichiro Saikawa, Shinya Sato, Hideto Kawaratani, Takemi Akahane, Ryuichi Noguchi, Masanori Matsumoto, Hitoshi Yoshiji   +16 more
doaj   +1 more source