Factor VIII alters tubular organization and functional properties of von Willebrand factor stored in Weibel-Palade bodies
Blood, ISSN: 0006-4971, Vol: 118, Issue: 22, Page: 5947-5956
2011
- 18Citations
- 49Captures
Metric Options: CountsSelecting the 1-year or 3-year option will change the metrics count to percentiles, illustrating how an article or review compares to other articles or reviews within the selected time period in the same journal. Selecting the 1-year option compares the metrics against other articles/reviews that were also published in the same calendar year. Selecting the 3-year option compares the metrics against other articles/reviews that were also published in the same calendar year plus the two years prior.
Example: if you select the 1-year option for an article published in 2019 and a metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019. If you select the 3-year option for the same article published in 2019 and the metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019, 2018 and 2017.
Citation Benchmarking is provided by Scopus and SciVal and is different from the metrics context provided by PlumX Metrics.
Example: if you select the 1-year option for an article published in 2019 and a metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019. If you select the 3-year option for the same article published in 2019 and the metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019, 2018 and 2017.
Citation Benchmarking is provided by Scopus and SciVal and is different from the metrics context provided by PlumX Metrics.
Metrics Details
- Citations18
- Citation Indexes18
- 18
- CrossRef16
- Captures49
- Readers49
- 49
Article Description
In endothelial cells, von Willebrand factor (VWF) multimers are packaged into tubules that direct biogenesis of elongated Weibel-Palade bodies (WPBs). WPB release results in unfurling of VWF tubules and assembly into strings that serve to recruit platelets. By confocal microscopy, we have previously observed a rounded morphology of WPBs in blood outgrowth endothelial cells transduced to express factor VIII (FVIII). Using correlative light-electron microscopy and tomography, we now demonstrate that FVIII-containing WPBs have disorganized, short VWF tubules. Whereas normal FVIII and FVIII Y1680F interfered with formation of ultra-large VWF multimers, release of the WPBs resulted in VWF strings of equal length as those from nontransduced blood outgrowth endothelial cells. After release, both WPB-derived FVIII and FVIII Y1680F remained bound to VWF strings, which however had largely lost their ability to recruit platelets. Strings from nontransduced cells, however, were capable of simultaneously recruiting exogenous FVIII and platelets. These findings suggest that the interaction of FVIII with VWF during WPB formation is independent of Y1680, is maintained after WPB release in FVIII-covered VWF strings, and impairs recruitment of platelets. Apparently, intra-cellular and extracellular assembly of FVIII-VWF complex involves distinct mechanisms, which differ with regard to their implications for platelet binding to released VWF strings.
Bibliographic Details
http://www.sciencedirect.com/science/article/pii/S0006497120405907; http://dx.doi.org/10.1182/blood-2011-05-355354; http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=82155178608&origin=inward; http://www.ncbi.nlm.nih.gov/pubmed/21940821; https://ashpublications.org/blood/article/118/22/5947/29200/Factor-VIII-alters-tubular-organization-and; https://dx.doi.org/10.1182/blood-2011-05-355354; https://ashpublications.org/blood/article-pdf/118/22/5947/1345956/zh804811005947.pdf; http://www.bloodjournal.org/content/118/22/5947; http://www.bloodjournal.org/content/118/22/5947.abstract; http://www.bloodjournal.org/content/118/22/5947.full.pdf; http://europepmc.org/abstract/med/21940821; http://www.bloodjournal.org/cgi/doi/10.1182/blood-2011-05-355354
American Society of Hematology
Provide Feedback
Have ideas for a new metric? Would you like to see something else here?Let us know