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An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
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Neurosciences
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Journal JoVE Neurosciences
An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

DOI:
10.3791/51903-v

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07:45 min

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October 21, 2014

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, , ,
1Department of Neuroscience,University of Connecticut Health Center, 2Department of Anatomy and Cell Biology,University of Illinois at Chicago

Chapitres

  • 00:05Titre
  • 01:16Coverslip Preparation
  • 02:37Globoid Cell Induction in Mixed Glial Cultures
  • 04:30Analysis and Characterization of Globoid Cells in Primary Culture
  • 06:12Results: Representative Microglial Cells After Psychosine and Latex Bead Phagocytosis
  • 07:15Conclusion

Summary

Traduction automatique

Traduction automatique

Globoid cells are a defining pathological feature of Krabbe disease, a leukodystrophy currently lacking an effective long-term therapy. We have developed a cell culture model to study the innate biology and pathogenic potential of activated microglia and their transformation into globoid cells.

Tags

Globoid Cell LeukodystrophyMicrogliaGloboid CellsIn Vitro ModelPrimary Murine Glial CulturePsychosineMatrix Metalloproteinase-3Cellular Pathophysiology

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