Search Results - "Moake, Joel"
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Factor VIII Is Synthesized in Human Endothelial Cells, Packaged in Weibel-Palade Bodies and Secreted Bound to ULVWF Strings
Published in PloS one (16-10-2015)“…The cellular synthesis site and ensuing storage location for human factor VIII (FVIII), the coagulation protein deficient in hemophilia A, has been elusive…”
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Thrombotic Microangiopathies
Published in The New England journal of medicine (22-08-2002)“…This article reviews the substantial progress made in understanding the thrombotic microangiopathies. The mechanisms of both thrombotic thrombocytopenic…”
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3
Acquired von Willebrand syndrome associated with left ventricular assist device
Published in Blood (23-06-2016)“…Left ventricular assist devices (LVAD) provide cardiac support for patients with end-stage heart disease as either bridge or destination therapy, and have…”
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4
Thrombotic thrombocytopenic purpura
Published in Nature reviews. Disease primers (06-04-2017)“…Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia,…”
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5
Heat-inactivated Factor B inhibits alternative pathway fluid-phase activation and convertase formation on endothelial cell-secreted ultra-large von Willebrand factor strings
Published in Scientific reports (08-04-2023)“…Defective regulation of the alternative complement pathway (AP) causes excessive activation and promotes the inflammation and renal injury observed in atypical…”
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6
A new therapeutic strategy for atypical HUS
Published in Blood (20-07-2017)Get full text
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7
Brain microvascular endothelial cells exhibit lower activation of the alternative complement pathway than glomerular microvascular endothelial cells
Published in The Journal of biological chemistry (11-05-2018)“…Atypical hemolytic uremic syndrome (aHUS) and bone marrow transplantation-associated thrombotic microangiopathy (TA-TMA) are associated with excessive…”
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8
Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis
Published in PloS one (29-03-2013)“…Vascular endothelial cells (ECs) express and release protein components of the complement pathways, as well as secreting and anchoring ultra-large von…”
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9
Production and control of coagulation proteins for factor X activation in human endothelial cells and fibroblasts
Published in Scientific reports (06-02-2020)“…Human endothelial cells (ECs) synthesize, store, and secrete von Willebrand factor multimeric strings and coagulation factor (F) VIII. It is not currently…”
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10
TNF Regulates Essential Alternative Complement Pathway Components and Impairs Activation of Protein C in Human Glomerular Endothelial Cells
Published in The Journal of immunology (1950) (15-01-2016)“…Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy with severe renal injury secondary to an overactive alternative complement pathway…”
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11
Human endothelial cells and fibroblasts express and produce the coagulation proteins necessary for thrombin generation
Published in Scientific reports (08-11-2021)“…In a previous study, we reported that human endothelial cells (ECs) express and produce their own coagulation factors (F) that can activate cell surface FX…”
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12
N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice
Published in The Journal of clinical investigation (01-02-2011)“…Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterized by systemic microvascular thrombosis caused by adhesion of platelets to…”
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13
Thrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies
Published in Best practice & research. Clinical haematology (01-12-2009)“…Thrombotoic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterised by systemic platelet aggregation, organ ischaemia, profound…”
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14
Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage
Published in Blood (03-12-2009)“…Human umbilical vein endothelial cell (HUVEC)–released ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin repeats) and HUVEC-secreted von…”
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15
Laminin Peptide-Immobilized Hydrogels Modulate Valve Endothelial Cell Hemostatic Regulation
Published in PloS one (19-06-2015)“…Valve endothelial cells (VEC) have unique phenotypic responses relative to other types of vascular endothelial cells and have highly sensitive hemostatic…”
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Effects of inflammatory cytokines on the release and cleavage of the endothelial cell–derived ultralarge von Willebrand factor multimers under flow
Published in Blood (01-07-2004)“…ADAMTS13 cleaves ultralarge and hyperreactive von Willebrand factor (ULVWF) freshly released from activated endothelial cells to smaller and less active forms…”
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Thrombotic Microangiopathies: Multimers, Metalloprotease, and Beyond
Published in Clinical and translational science (01-10-2009)“…The pathophysiology of various types of thrombotic microangiopathies is coming progressively into focus. Therapeutic advances are likely to follow at a…”
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Treatment of refractory thrombotic thrombocytopenic purpura with N-acetylcysteine: a case report
Published in Transfusion (Philadelphia, Pa.) (01-05-2014)“…Background Thrombotic thrombocytopenic purpura (TTP) is a life‐threatening disease resulting in systemic microvascular thrombosis. The disease is caused by…”
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Regulatory components of the alternative complement pathway in endothelial cell cytoplasm, factor H and factor I, are not packaged in Weibel-Palade bodies
Published in PloS one (24-03-2015)“…It was recently reported that factor H, a regulatory component of the alternative complement pathway, is stored with von Willebrand factor (VWF) in the…”
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20
Correlating Conformational Dynamics with the Von Willebrand Factor Reductase Activity of Factor H Using Single Molecule Force Measurements
Published in The journal of physical chemistry. B (29-11-2018)“…Activation of proteins often involves conformational transitions, and these switches are often difficult to characterize in multidomain proteins. Full-length…”
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