ArticleArteriosclerosis, thrombosis, and vascular biology2013
Endothelial Von Willebrand factor promotes blood-brain barrier flexibility and provides protection from hypoxia and seizures in mice.
Article in Arteriosclerosis, thrombosis, and vascular biology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 52 papers.
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Who cites it
52 citing papers in PubMed, 86 citations in OpenAlex.
- ABO and RhD Blood Groups in Epilepsy: A Comprehensive Case-Control Analysis.Journal of clinical medicine · 2026Article
- Increased VWF-to-ADAMTS13 ratio is associated with large cerebral white matter hyperintensities.Blood vessels, thrombosis & hemostasis · 2026Article
- Photobiomodulation restores blood-brain barrier integrity after hypoxia via endothelial von Willebrand factor modulation in a humanised tricellular transwell model.The Journal of physiology · 2026Article
- Modulation of the Receptor Tyrosine Kinase TIE2/International journal of molecular sciences · 2026Article
- The interplay between neuroinflammation and endothelial dysfunction in cerebral small vessel disease.Frontiers in immunology · 2026Review
- Neutrophil extracellular traps aggravate blood-brain barrier disruption via ZBP1/FSP1-mediated ferroptosis after traumatic brain injury.Fluids and barriers of the CNS · 2025Article
- Unraveling Predominantly Inattentive ADHD (ADHD-PI): Insights from Proteomic Analysis of the Striatum of Thyroid Hormone-Responsive Protein (THRSP)-Overexpressing Mice.Molecular neurobiology · 2025Article
- Novel purine derivatives mitigate hypoxia ischemia related brain injury through agrin, zyxin and synaptotagmin proteins.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2025Article
- Neuroprotective and Anti-Inflammatory Activity ofMarine drugs · 2025Article
- Spatiotemporal Angiogenic Patterns in the Development of the Mouse Fetal Blood-Brain Barrier System During Pregnancy.International journal of molecular sciences · 2025Article
- Blood biomarkers of vascular dysfunction in small vessel disease progression: Insights from a longitudinal neuroimaging study.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Engineering extracellular vesicles to transiently permeabilize the blood-brain barrier.Journal of nanobiotechnology · 2024Article
- Endothelial Myosin IIA Is Required for the Maintenance of Blood-Brain Barrier Integrity.Cells · 2024Article
- Gene-SGAN: discovering disease subtypes with imaging and genetic signatures via multi-view weakly-supervised deep clustering.Nature communications · 2024Article
- Cadmium, von Willebrand factor and vascular aging.npj aging · 2023Review
- Plasma inflammatory biomarkers in cerebral small vessel disease: A review.CNS neuroscience & therapeutics · 2023Review
- circ_0001274 Competitively Binds miR-143-3p to Upregulate VWF Expression to Improve Acute Traumatic Coagulopathy.Oxidative medicine and cellular longevity · 2023Article
- Cerebral Microbleeds During Transcatheter Aortic Valve Replacement: A Prospective Magnetic Resonance Imaging Cohort.Circulation · 2022Observational
- The Intriguing Connections between von Willebrand Factor, ADAMTS13 and Cancer.Healthcare (Basel, Switzerland) · 2022Review
- Biomarkers for Transient Ischemic Attack: A Brief Perspective of Current Reports and Future Horizons.Journal of clinical medicine · 2022Review
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
objectiveAberrant blood-brain barrier (BBB) permeability is a hallmark pathology of many central nervous system diseases. von Willebrand factor (VWF) is stored in endothelial Weibel-Palade bodies from where it is released on activation into plasma and basement membrane. The role of VWF in endothelial homeostasis is unclear. The goal of this study was to assess the role of VWF in disease models associated with increased BBB permeability. APPROACH AND
resultsWe did not find any differences in BBB permeability to Evans blue dye at baseline between wild-type and VWF(-/-) animals. We next used 2 models presenting with increased BBB permeability, hypoxia/reoxygenation and pilocarpine-induced status epilepticus, to assess the response of VWF(-/-) animals. In both models, VWF(-/-) mice maintained a tighter BBB than wild-type mice. VWF(-/-) mice fared worse in both conditions, with ≈ 100% of VWF(-/-) mice dying within 120 minutes after pilocarpine administration, whereas >80% of wild-type animals survived. Investigation into the status of tight junction proteins revealed that VWF(-/-) mice expressed more claudin-5 at baseline. In vitro work confirmed that the presence of subendothelial VWF is inhibitory to claudin-5 expression.
conclusionsVWF deficiency confers partial preservation of BBB integrity after hypoxia/reoxygenation and seizures. Surprisingly, this decrease in BBB permeability did not result in protection of animals because they demonstrated more severe pathology in both models compared with wild-type animals. These data suggest that a rigid BBB is detrimental (to the organism) during certain disease states and that VWF release may provide desired flexibility under stress.
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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.