作者
Paquita Nurden, Giuliana Gobbi, Alan Nurden, Jocelyne Enouf, Ibtissam Youlyouz-Marfak, Cecilia Carubbi, Silvia La Marca, Margherita Punzo, Luciano Baronciani, Luigi De Marco, Marco Vitale, Augusto B Federici
发表日期
2010/4/1
期刊
Blood, The Journal of the American Society of Hematology
卷号
115
期号
13
页码范围
2649-2656
出版商
American Society of Hematology
简介
von Willebrand factor (VWF) is an essential mediator of platelet adhesion to the vessel wall, but little is known about its role in megakaryocytopoiesis. VWF and its platelet receptor, glycoprotein Ibα (GPIbα), are both expressed during megakaryocyte (MK) maturation. This study was designed to evaluate whether the enhanced VWF-GPIbα interactions typical of patients with von Willebrand disease type 2B (VWD2B) modify platelet production. Platelets from 9 patients with VWD2B with 7 different gain-of-function mutations were examined by electron microscopy (EM) and immunofluorescence labeling. For the patients with VWD2B, EM characteristically showed variable numbers of structurally abnormal giant platelets, sometimes in agglutinates. Cultures of MKs from controls performed with or without purified VWF confirmed a positive influence of VWF on platelet production with specific inhibition by an antibody …
学术搜索中的文章
P Nurden, G Gobbi, A Nurden, J Enouf… - Blood, The Journal of the American Society of …, 2010