A microglial hypothesis of globoid cell leukodystrophy pathology

J Neurosci Res. 2016 Nov;94(11):1049-61. doi: 10.1002/jnr.23773.


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.

Keywords: globoid cell leukodystrophy; globoid cells; microglia.

Publication types

  • Review
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Humans
  • Leukodystrophy, Globoid Cell / pathology*
  • Microglia / metabolism
  • Microglia / pathology*