Search Results - "Voorberg, J."

An open conformation of ADAMTS‐13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura by Roose, E., Schelpe, A. S., Joly, B. S., Peetermans, M., Verhamme, P., Voorberg, J., Greinacher, A., Deckmyn, H., De Meyer, S. F., Coppo, P., Veyradier, A., Vanhoorelbeke, K.

“…Essentials Conformational changes in ADAMTS‐13 are part of its mode‐of‐action. The murine anti‐ADAMTS‐13 antibody 1C4 discriminates between folded and open…”
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Antibodies against recombinant alpha-galactosidase A in Fabry disease: Subclass analysis and impact on response to treatment by van der Veen, S.J., van Kuilenburg, A.B.P., Hollak, C.E.M., Kaijen, P.H.P., Voorberg, J., Langeveld, M.

“…Treatment of Fabry disease (FD) with recombinant alpha-galactosidase A (r-αGAL A) is complicated by the formation of anti-drug antibodies in the majority of…”
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Prevalence and Incidence of Non-neutralizing Antibodies in Congenital Hemophilia A- A Systematic Review and Meta-Analysis by Abdi, A, Bordbar, M R, Hassan, S, Rosendaal, F R, van der Bom, J G, Voorberg, J, Fijnvandraat, K, Gouw, S C

“…In hemophilia A the presence of non-neutralizing antibodies (NNAs) against Factor VIII (FVIII) may predict the development of neutralizing antibodies…”
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von Willebrand factor remodeling during exocytosis from vascular endothelial cells by Mourik, M. J., Valentijn, J. A., Voorberg, J., Koster, A. J., Valentijn, K. M., Eikenboom, J.

“…Summary Background In vascular endothelial cells, high molecular weight multimers of von Willebrand factor (VWF) are folded into tubular structures for storage…”
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Identification of N‐linked glycosylation and putative O‐fucosylation, C‐mannosylation sites in plasma derived ADAMTS13 by Sorvillo, N., Kaijen, P. H., Matsumoto, M., Fujimura, Y., Zwaan, C., Verbij, F. C., Pos, W., Fijnheer, R., Voorberg, J., Meijer, A. B.

“…Summary Background Acquired deficiency of ADAMTS13 causes a rare and life‐threatening disorder called thrombotic thrombocytopenic purpura (TTP). Several…”
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Angiogenic characteristics of blood outgrowth endothelial cells from patients with von Willebrand disease by Groeneveld, D. J., Bekkum, T., Dirven, R. J., Wang, J.‐W., Voorberg, J., Reitsma, P. H., Eikenboom, J.

“…Summary Background Endothelial von Willebrand factor (VWF) inhibits angiogenesis. Accordingly, blood outgrowth endothelial cells (BOECs) isolated from von…”
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Early cellular interactions and immune transcriptome profiles in human factor VIII‐exposed hemophilia A mice by Lai, J. D., Cartier, D., Hartholt, R. B., Swystun, L. L., van Velzen, A. S., den Haan, J. M. M., Hough, C., Voorberg, J., Lillicrap, D.

“…Essentials Initial immune cell interactions leading to factor (F) VIII immunity are not well characterized. We assessed cellular interactions and expression…”
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Dangerous liaisons: how the immune system deals with factor VIII by WROBLEWSKA, A., REIPERT, B. M., PRATT, K. P., VOORBERG, J.

“…Only a fraction of patients with hemophilia A develop a neutralizing antibody (inhibitor) response to therapeutic infusions of factor VIII. Our present…”
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Variation in baseline factor VIII concentration in a retrospective cohort of mild/moderate hemophilia A patients carrying identical F8 mutations by Loomans, J. I., Velzen, A. S., Eckhardt, C. L., Peters, M., Mäkipernaa, A., Holmstrom, M., Brons, P. P., Dors, N., Haya, S., Voorberg, J., Bom, J. G., Fijnvandraat, K.

“…Essentials Factor VIII levels vary in mild and moderate hemophilia A (MHA) patients with the same mutation. We aimed to estimate the variation and determinants…”
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Evaluation of the role of the GPIb-IX-V receptor complex in development of the platelet storage lesion by Rijkers, M., van der Meer, P. F., Bontekoe, I. J., Daal, B. B., de Korte, D., Leebeek, F. W. G., Voorberg, J., Jansen, A. J. G.

“…Background and Objectives In mice, loss of sialic acid resulting in shedding of glycoprotein (GP) Ibα and GPV has been linked to platelet survival. The aim of…”
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Residues Arg568 and Phe592 contribute to an antigenic surface for anti-ADAMTS13 antibodies in the spacer domain by POS, Wouter, SORVILLO, Nicoletta, FIJNHEER, Rob, FEYS, Hendrik B, KAIJEN, Paul H. P, VIDARSSON, Gestur, VOORBERG, Jan

“…The majority of patients diagnosed with thrombotic thrombocytopenic purpura have autoantibodies directed towards the spacer domain of ADAMTS13. In this study…”
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Enhanced uptake of blood coagulation factor VIII containing immune complexes by antigen presenting cells by Hartholt, R. B., Wroblewska, A., Herczenik, E., Peyron, I., ten Brinke, A., Rispens, T., Nolte, M. A., Slot, E., Claassens, J. W., Nimmerjahn, F., Verbeek, J. S., Voorberg, J.

“…Essentials Anti‐factor (F) VIII antibody formation is a major complication in the treatment of hemophilia A. We investigated uptake of FVIII and FVIII immune…”
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Humoral immune response to ADAMTS13 in acquired thrombotic thrombocytopenic purpura by POS, W., LUKEN, B. M., SORVILLO, N., HOVINGA, J. A. KREMER, VOORBERG, J.

“…The apparently spontaneous development of autoantibodies to ADAMTS13 in previously healthy individuals is a major cause of thrombotic thrombocytopenic purpura…”
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VH1‐69 germline encoded antibodies directed towards ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura by POS, W., LUKEN, B. M., KREMER HOVINGA, J. A., TURENHOUT, E. A. M., SCHEIFLINGER, F., DONG, J.‐F., FIJNHEER, R., VOORBERG, J.

“…Background: Autoantibodies directed towards ADAMTS13 are present in the majority of patients with acquired thrombotic thrombocytopenic purpura (TTP). Analysis…”
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Formation of platelet‐binding von Willebrand factor strings on non‐endothelial cells by WANG, J. W., VALENTIJN, J. A., VALENTIJN, K. M., DRAGT, B. S., VOORBERG, J., REITSMA, P. H., EIKENBOOM, J.

“…Background and Objective:  Von Willebrand factor (VWF) forms strings on activated vascular endothelial cells that recruit platelets and initiate clot…”
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T‐cell responses in two unrelated hemophilia A inhibitor subjects include an epitope at the factor VIII R593C missense site by JAMES, E. A., VAN HAREN, S. D., ETTINGER, R. A., FIJNVANDRAAT, K., LIBERMAN, J. A., KWOK, W. W., VOORBERG, J., PRATT, K. P.

“…Background: Development of neutralizing anti‐factor (F)VIII antibodies (‘inhibitors’) is a serious clinical problem in hemophilia A. Increased inhibitor risk…”
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Phosphatidylinositol‐3,4,5‐triphosphate‐dependent Rac exchange factor 1 regulates epinephrine‐induced exocytosis of Weibel–Palade bodies by Hooren, K. W. E. M., Breevoort, D., Fernandez‐Borja, M., Meijer, A. B., Eikenboom, J., Bierings, R., Voorberg, J.

“…Summary Background Weibel–Palade bodies (WPBs) function as storage vesicles for von Willebrand factor (VWF) and a number of other bioactive compounds,…”
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Covalent regulation of ULVWF string formation and elongation on endothelial cells under flow conditions by LI, Y., CHOI, H., ZHOU, Z., NOLASCO, L., POWNALL, H. J., VOORBERG, J., MOAKE, J. L., DONG, J.‐F.

“…Background and Objectives: The adhesion ligand von Willebrand factor (VWF) is a multimeric glycoprotein that mediates platelet adhesion to exposed…”
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Multiple B‐cell clones producing antibodies directed to the spacer and disintegrin/thrombospondin type‐1 repeat 1 (TSP1) of ADAMTS13 in a patient with acquired thrombotic thrombocytopenic purpura by LUKEN, B. M., KAIJEN, P. H. P., TURENHOUT, E. A. M., KREMER HOVINGA, J. A., VAN MOURIK, J. A., FIJNHEER, R., VOORBERG, J.

“…Background: The cysteine‐rich/spacer domains of ADAMTS13 contain a major binding site for antibodies in patients with acquired thrombotic thrombocytopenic…”
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Proteolytic cleavage of factor VIII heavy chain is required to expose the binding‐site for low‐density lipoprotein receptor‐related protein within the A2 domain by BOVENSCHEN, N., VAN STEMPVOORT, G., VOORBERG, J., MERTENS, K., MEIJER, A. B.

“…Background: Low‐density lipoprotein receptor‐related protein (LRP) is an endocytic receptor that contributes to the clearance of coagulation factor (F) VIII…”
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