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A microglial hypothesis of globoid cell leukodystrophy pathology
Author(s) -
Nicaise Alexandra M.,
Bongarzone Ernesto R.,
Crocker Stephen J.
Publication year - 2016
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.23773
Subject(s) - leukodystrophy , microglia , giant cell , krabbe disease , inflammation , multinucleate , phenotype , oligodendrocyte , pathology , cytotoxic t cell , disease , cell , biology , myelin , multiple sclerosis , demyelinating disease , neuroscience , immunology , microbiology and biotechnology , medicine , central nervous system , in vitro , genetics , gene
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.