نتایج جستجو برای: vwf
تعداد نتایج: 3173 فیلتر نتایج به سال:
چکید ه سابقه و هدف تمایل به خونریزی در ناقلین هموفیلی a از سال ها قبل شناخته شده به طوری که برخی حتی با سطح فاکتور 40% تا 60% نیز، دچار خونریزی های غیر طبیعی می شوند. هدف از مطالعه تعیین سطح فاکتور viii و فون ویلبراند و بررسی ارتباط آن ها با علایم بالینی در ناقلین قطعی بود. مواد و روش ها این بررسی توصیفی بر روی 49 زن ناقل قطعی هموفیلی a در سال 1389 انجام شد. آزمایش ptt ، pt ، f. viii و ...
Shear induced platelet activation is responsible for arterial thrombosis. The role of fibrinogen is established at low shear stress, but at high shear stress von Willebrand factor (vWF), not fibrinogen, is essential for platelet adhesion and aggregation. Patients with vWF deficiency exhibit resistance to thrombosis, and raised vWF is a risk factor for acute coronary syndromes. Interpretation of...
In this report we describe the molecular defect underlying partial and severe quantitative von Willebrand factor (VWF) deficiencies in 3 families previously diagnosed with types 1 and 3 Von Willebrand-disease. Analysis of the VWF gene in affected family members revealed a novel C to T transition at nucleotide 1067 of the VWF complemetary DNA (cDNA), predicting substitution of arginine by trypto...
The specific interactions of von Willebrand factor (VWF) with the vessel wall, platelets or other interfaces strongly depend on (a shear-induced) VWF activation. Shear flow has been shown to induce a conformational transition of VWF, but is modulated by its thermodynamic state (state-function relationship). The state in turn is determined by physical (e.g. vessel geometry), physico-chemical (e....
von Willebrand factor (vWF) is a large protein involved in primary hemostasis. A dysfunction in this protein or an insufficient production of the protein leads to improper platelet adhesion/aggregation, resulting in a bleeding phenotype known as von Willebrand disease (vWD). To gain a better understanding of vWF interactions in vivo, the use of zebrafish as a model is ideal because of the trans...
Von Willebrand factor (VWF) is an essential component of hemostasis. However, animal studies using VWF-deficient mice suggest that VWF may also contribute to inflammation. In the present study, we demonstrate that VWF was able to interact with polymorphonuclear leukocytes (PMNs) and monocytes under static and flow conditions. Adhesion under flow was dominated by short-lasting contact with resti...
von Willebrand factor (vWF) secretion by endothelial cells (ECs) is essential for hemostasis and thrombosis; however, the molecular mechanisms are poorly understood. Interestingly, we observed increased bleeding in EC-Gα13(-/-);Gα12(-/-) mice that could be normalized by infusion of human vWF. Blood from Gα12(-/-) mice exhibited significantly reduced vWF levels but normal vWF multimers and impai...
Ultralarge von Willebrand factor (UL-VWF) multimers are thought to play a central role in pathogenesis of the disease thrombotic thrombocytopenic purpura (TTP); however, experimental evidence in support of this hypothesis has been difficult to establish. Therefore, to examine directly the requirement for VWF in TTP pathogenesis, we generated ADAMTS13-deficient mice on a TTP-susceptible genetic ...
BACKGROUND Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD). DESIGN AND METHODS Eight patients from three unrelated families with this mutation were included in the present study who had distinct VWF abnormalities, not described in earlier studies. RESULTS The patients showed notably low levels of...
Von Willebrand Factor (vWF) mRNA was identified in fractionated polyA+ RNA preparations isolated from cultured human endothelial cells. Micro-injection of specific polyA+ RNA fractions in Xenopus laevis oocytes provoked the synthesis of a vWF-like product which could be detected with an immunoradiometric assay relying on Sepharose-linked monoclonal anti-vWF IgG and different radiolabeled monocl...
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