APA
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Stan, R., Tse, D., Deharvengt, S., Smits, N., Xu, Y., Luciano, M., … Carrière, C. (2012). The diaphragms of fenestrated endothelia – gatekeepers of vascular permeability and blood composition. Developmental Cell.
Chicago/Turabian
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Stan, R., D. Tse, S. Deharvengt, N. Smits, Yan Xu, M. Luciano, C. McGarry, et al. “The Diaphragms of Fenestrated Endothelia – Gatekeepers of Vascular Permeability and Blood Composition.” Developmental Cell (2012).
MLA
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Stan, R., et al. “The Diaphragms of Fenestrated Endothelia – Gatekeepers of Vascular Permeability and Blood Composition.” Developmental Cell, 2012.
BibTeX Click to copy
@article{r2012a,
title = {The diaphragms of fenestrated endothelia – gatekeepers of vascular permeability and blood composition},
year = {2012},
journal = {Developmental Cell},
author = {Stan, R. and Tse, D. and Deharvengt, S. and Smits, N. and Xu, Yan and Luciano, M. and McGarry, C. and Buitendijk, M. and Nemani, Krishnamurthy V. and Elgueta, R. and Kobayashi, Takashi and Shipman, Samantha L. and Moodie, K. and Daghlian, C. and Ernst, P. and Lee, Hong‐Kee and Suriawinata, A. and Schned, A. and Longnecker, D. and Fiering, S. and Noelle, R. and Gimi, B. and Shworak, N. and Carrière, C.}
}
SUMMARY Fenestral and stomatal diaphragms are endothelial subcellular structures of unknown function that form on organelles implicated in vascular permeability: fenestrae, transendothelial channels and caveolae. PV1 protein is required for diaphragm formation in vitro. Here, we report that deletion of the PV1-encoding Plvap gene in mice results in the absence of diaphragms and decreased survival. Loss of diaphragms did not affect the fenestrae and transendothelial channels formation but disrupted the barrier function of fenestrated capillaries causing a major leak of plasma proteins. This disruption results in early death of animals due to severe non-inflammatory protein loosing enteropathy. Deletion of PV1 in endothelium, but not the hematopoietic compartment, recapitulates the phenotype of global PV1 deletion, whereas endothelial reconstitution of PV1 rescues the phenotype. Taken together, these data provide genetic evidence for the critical role of the diaphragms in fenestrated capillaries in the maintenance of blood composition.