title: A microglial hypothesis of globoid cell leukodystrophy pathology.
abstract: Globoid cell leukodystrophy (GLD), also known as Krabbe's disease, is a fatal demyelinating disease accompanied by the formation of giant, multinucleated cells called globoid cells. Previously believed to be a byproduct of inflammation, these cells can be found early in disease before evidence of any damage. The precise mechanism by which these globoid cells cause oligodendrocyte dysfunction is not completely understood, nor is their cell type defined. This Review outlines the idea that microglial cells are transformed into an unknown and undefined novel M3 phenotype in GLD, which is cytotoxic to oligodendrocytes, leading to disease progression. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Neuroscience, University of Connecticut School of Medicine, Farmington, Connecticut.
authors.forename: Alexandra M
authors.initials: AM
authors.lastname: Nicaise
authors.affiliation: Department of Anatomy and Cell Biology, University of Illinois at Chicago, Chicago, Illinois.
authors.forename: Ernesto R
authors.initials: ER
authors.lastname: Bongarzone
authors.affiliation: Department of Neuroscience, University of Connecticut School of Medicine, Farmington, Connecticut. crocker@uchc.edu.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Crocker
mesh_terms: D000818:Animals; D006801:Humans; D007965:Leukodystrophy, Globoid Cell / Q000473:pathology*; D017628:Microglia / Q000378:metabolism / Q000473:pathology*
keywords: globoid cell leukodystrophy; globoid cells; microglia