title: An in vitro model for the study of cellular pathophysiology in globoid cell leukodystrophy.
abstract: The precise function of multi-nucleated microglia, called globoid cells, that are uniquely abundant in the central nervous system of globoid cell leukodystrophy (GLD) is unclear. This gap in knowledge has been hindered by the lack of an appropriate in vitro model for study. Herein, we describe a primary murine glial culture system in which treatment with psychosine results in multinucleation of microglia resembling the characteristic globoid cells found in GLD. Using this novel system, we defined the conditions and modes of analysis for study of globoid cells. The potential use of this model system was validated in our previous study, which identified a potential role for matrix metalloproteinase (MMP)-3 in GLD. This novel in vitro system may be a useful model in which to study the formation and function, but also the potential therapeutic manipulation, of these unique cells.
authors.affiliation: Department of Neuroscience, University of Connecticut Health Center.
authors.forename: Kumiko I
authors.initials: KI
authors.lastname: Claycomb
authors.affiliation: Department of Neuroscience, University of Connecticut Health Center.
authors.forename: Kasey M
authors.initials: KM
authors.lastname: Johnson
authors.affiliation: Department of Anatomy and Cell Biology, University of Illinois at Chicago.
authors.forename: Ernesto R
authors.initials: ER
authors.lastname: Bongarzone
authors.affiliation: Department of Neuroscience, University of Connecticut Health Center; crocker@uchc.edu.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Crocker
mesh_terms: D000818:Animals; D018929:Cell Culture Techniques / Q000379:methods*; D004195:Disease Models, Animal; D007965:Leukodystrophy, Globoid Cell / Q000473:pathology*; D051379:Mice; D017628:Microglia / Q000473:pathology*