Academic Bibliography

 Search 200 years of publications by Ghent University researchers.
Advanced search
1 file | 5.67 MB
Add to list
  1. Search results
  2. Anti-inflammatory mesenchymal stromal...

Anti-inflammatory mesenchymal stromal cell-derived extracellular vesicles improve pathology in Niemann-Pick type C disease

Lien Van Hoecke (UGent) , Caroline Van Cauwenberghe (UGent) , Verena Boerger, Arnout Bruggeman (UGent) , Jonas Castelein (UGent) , Griet Van Imschoot (UGent) , Elien Van Wonterghem (UGent) , Robin Dittrich, Wouter Claeys (UGent) , Junhua Xie, et al.
(2021) BIOMEDICINES. 9(12).
Author
Organization
Project
Abstract
Niemann-Pick type C (NPC) disease is a rare neurovisceral lipid storage disease with progressive neurodegeneration, leading to premature death. The disease is caused by loss-of-function mutations either in the NPC1 or NPC2 gene which results in lipid accumulation in the late endosomes and lysosomes. The involved disease mechanisms are still incompletely understood, making the design of a rational treatment very difficult. Since the disease is characterized by peripheral inflammation and neuroinflammation and it is shown that extracellular vesicles (EVs) obtained from mesenchymal stromal cells (MSCs) provide immunomodulatory capacities, we tested the potential of MSC-EV preparations to alter NPC1 disease pathology. Here, we show that the administration of an MSC-EV preparation with in vitro and in vivo confirmed immune modulatory capabilities is able to reduce the inflammatory state of peripheral organs and different brain regions of NPC1-diseased mice almost to normal levels. Moreover, a reduction of foamy cells in different peripheral organs was observed upon MSC-EV treatment of NPC1(-/-) mice. Lastly, the treatment was able to decrease microgliosis and astrogliosis, typical features of NPC1 patients that lead to neurodegeneration. Altogether, our results reveal the therapeutic potential of MSC-EVs as treatment for the genetic neurovisceral lipid storage disease NPC, thereby counteracting both central and peripheral features.
Keywords
Niemann-Pick type C disease, extracellular vesicles, mesenchymal stem cell, VERSUS-HOST-DISEASE, KUPFFER CELLS, MURINE MODEL, STEM-CELLS, CHOLESTEROL, DEATH, MICE, NEUROINFLAMMATION, PROTECTION, REDUCTION

Downloads

  • Download
    4672 21VanHoecke.pdf
    • full text (Published version)
    • |
    • open access
    • |
    • PDF
    • |
    • 5.67 MB

Citation

Please use this url to cite or link to this publication:

MLA
Van Hoecke, Lien, et al. “Anti-Inflammatory Mesenchymal Stromal Cell-Derived Extracellular Vesicles Improve Pathology in Niemann-Pick Type C Disease.” BIOMEDICINES, vol. 9, no. 12, 2021, doi:10.3390/biomedicines9121864.
APA
Van Hoecke, L., Van Cauwenberghe, C., Boerger, V., Bruggeman, A., Castelein, J., Van Imschoot, G., … Vandenbroucke, R. (2021). Anti-inflammatory mesenchymal stromal cell-derived extracellular vesicles improve pathology in Niemann-Pick type C disease. BIOMEDICINES, 9(12). https://doi.org/10.3390/biomedicines9121864
Chicago author-date
Van Hoecke, Lien, Caroline Van Cauwenberghe, Verena Boerger, Arnout Bruggeman, Jonas Castelein, Griet Van Imschoot, Elien Van Wonterghem, et al. 2021. “Anti-Inflammatory Mesenchymal Stromal Cell-Derived Extracellular Vesicles Improve Pathology in Niemann-Pick Type C Disease.” BIOMEDICINES 9 (12). https://doi.org/10.3390/biomedicines9121864.
Chicago author-date (all authors)
Van Hoecke, Lien, Caroline Van Cauwenberghe, Verena Boerger, Arnout Bruggeman, Jonas Castelein, Griet Van Imschoot, Elien Van Wonterghem, Robin Dittrich, Wouter Claeys, Junhua Xie, Bernd Giebel, and Roosmarijn Vandenbroucke. 2021. “Anti-Inflammatory Mesenchymal Stromal Cell-Derived Extracellular Vesicles Improve Pathology in Niemann-Pick Type C Disease.” BIOMEDICINES 9 (12). doi:10.3390/biomedicines9121864.
Vancouver
1.
Van Hoecke L, Van Cauwenberghe C, Boerger V, Bruggeman A, Castelein J, Van Imschoot G, et al. Anti-inflammatory mesenchymal stromal cell-derived extracellular vesicles improve pathology in Niemann-Pick type C disease. BIOMEDICINES. 2021;9(12).
IEEE
[1]
L. Van Hoecke et al., “Anti-inflammatory mesenchymal stromal cell-derived extracellular vesicles improve pathology in Niemann-Pick type C disease,” BIOMEDICINES, vol. 9, no. 12, 2021.
@article{8734111,
  abstract     = {{Niemann-Pick type C (NPC) disease is a rare neurovisceral lipid storage disease with progressive neurodegeneration, leading to premature death. The disease is caused by loss-of-function mutations either in the NPC1 or NPC2 gene which results in lipid accumulation in the late endosomes and lysosomes. The involved disease mechanisms are still incompletely understood, making the design of a rational treatment very difficult. Since the disease is characterized by peripheral inflammation and neuroinflammation and it is shown that extracellular vesicles (EVs) obtained from mesenchymal stromal cells (MSCs) provide immunomodulatory capacities, we tested the potential of MSC-EV preparations to alter NPC1 disease pathology. Here, we show that the administration of an MSC-EV preparation with in vitro and in vivo confirmed immune modulatory capabilities is able to reduce the inflammatory state of peripheral organs and different brain regions of NPC1-diseased mice almost to normal levels. Moreover, a reduction of foamy cells in different peripheral organs was observed upon MSC-EV treatment of NPC1(-/-) mice. Lastly, the treatment was able to decrease microgliosis and astrogliosis, typical features of NPC1 patients that lead to neurodegeneration. Altogether, our results reveal the therapeutic potential of MSC-EVs as treatment for the genetic neurovisceral lipid storage disease NPC, thereby counteracting both central and peripheral features.}},
  articleno    = {{1864}},
  author       = {{Van Hoecke, Lien and Van Cauwenberghe, Caroline and Boerger, Verena and Bruggeman, Arnout and Castelein, Jonas and Van Imschoot, Griet and Van Wonterghem, Elien and Dittrich, Robin and Claeys, Wouter and Xie, Junhua and Giebel, Bernd and Vandenbroucke, Roosmarijn}},
  issn         = {{2227-9059}},
  journal      = {{BIOMEDICINES}},
  keywords     = {{Niemann-Pick type C disease,extracellular vesicles,mesenchymal stem cell,VERSUS-HOST-DISEASE,KUPFFER CELLS,MURINE MODEL,STEM-CELLS,CHOLESTEROL,DEATH,MICE,NEUROINFLAMMATION,PROTECTION,REDUCTION}},
  language     = {{eng}},
  number       = {{12}},
  pages        = {{13}},
  title        = {{Anti-inflammatory mesenchymal stromal cell-derived extracellular vesicles improve pathology in Niemann-Pick type C disease}},
  url          = {{http://doi.org/10.3390/biomedicines9121864}},
  volume       = {{9}},
  year         = {{2021}},
}
Altmetric
View in Altmetric
Web of Science
Times cited: