ArticleBlood2013
Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells.
Article in Blood, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers, 2 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
46 citing papers in PubMed, 2 syntheses or guidelines pooled it, 100 citations in OpenAlex.
- Human endothelial colony-forming cells in regenerative therapy: A systematic review of controlled preclinical animal studies.Stem cells translational medicine · 2020Pooled it
- Standardization of methods to quantify and culture endothelial colony-forming cells derived from peripheral blood: Position paper from the International Society on Thrombosis and Haemostasis SSC.Journal of thrombosis and haemostasis : JTH · 2019Guideline
- Significant gynecological bleeding in women with low von Willebrand factor levels.Blood advances · 2018Trial
- Trial
- Inherited human group IVA cytosolic phospholipase A2 deficiency abolishes platelet, endothelial, and leucocyte eicosanoid generation.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2015Trial
- Structure and multiple functions of von Willebrand factor.Haematologica · 2026Review
- Isolation and Characterization of Endothelial-Colony Forming Cells (ECFC): Studying Endothelial Senescence for Translational Studies and for Personalized Medicine.Methods in molecular biology (Clifton, N.J.) · 2025Article
- Application of genetic testing for the diagnosis of von Willebrand disease.Journal of thrombosis and haemostasis : JTH · 2024Review
- Clusterin knockdown has effects on intracellular and secreted von Willebrand factor in human umbilical vein endothelial cells.PloS one · 2024Article
- What can historical literature on von Willebrand disease teach us?Research and practice in thrombosis and haemostasis · 2023Article
- Experimental Models of Traumatic Injuries: Do They Capture the Coagulopathy and Underlying Endotheliopathy Induced by Human Trauma?International journal of molecular sciences · 2023Review
- APOLD1 loss causes endothelial dysfunction involving cell junctions, cytoskeletal architecture, and Weibel-Palade bodies, while disrupting hemostasis.Haematologica · 2023Article
- Special considerations in GI bleeding in VWD patients.Hematology. American Society of Hematology. Education Program · 2022Review
- Human Endothelial Colony-Forming Cells.Cold Spring Harbor perspectives in medicine · 2022Article
- Dispatch and delivery at the ER-Golgi interface: how endothelial cells tune their hemostatic response.The FEBS journal · 2022Article
- Syntaxin 5 determines Weibel-Palade body size and von Willebrand factor secretion by controlling Golgi architecture.Haematologica · 2022Article
- von Willebrand factor propeptide variants lead to impaired storage and ER retention in patient-derived endothelial colony-forming cells.Journal of thrombosis and haemostasis : JTH · 2022Article
- Structure modeling hints at a granular organization of the Golgi ribbon.BMC biology · 2022Article
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Authors and funding
10 authors at 4 institutions in 2 countries.
Funding
Abstract
Von Willebrand disease (VWD) is a heterogeneous bleeding disorder caused by decrease or dysfunction of von Willebrand factor (VWF). A wide range of mutations in the VWF gene have been characterized; however, their cellular consequences are still poorly understood. Here we have used a recently developed approach to study the molecular and cellular basis of VWD. We isolated blood outgrowth endothelial cells (BOECs) from peripheral blood of 4 type 1 VWD and 4 type 2 VWD patients and 9 healthy controls. We confirmed the endothelial lineage of BOECs, then measured VWF messenger RNA (mRNA) and protein levels (before and after stimulation) and VWF multimers. Decreased mRNA levels were predictive of plasma VWF levels in type 1 VWD, confirming a defect in VWF synthesis. However, BOECs from this group of patients also showed defects in processing, storage, and/or secretion of VWF. Levels of VWF mRNA and protein were normal in BOECs from 3 type 2 VWD patients, supporting the dysfunctional VWF model. However, 1 type 2M patient showed decreased VWF synthesis and storage, indicating a complex cellular defect. These results demonstrate for the first time that isolation of endothelial cells from VWD patients provides novel insight into cellular mechanisms of the disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.