ITEM METADATA RECORD
Title: Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis
Authors: Philips, Thomas
Abreu Bento, André
Nonneman, Annelies
Haeck, Wanda
Staats, Kim A
Geelen, Veerle
Hersmus, Nicole
Küsters, Benno
Van Den Bosch, Ludo
Van Damme, Philip
Richardson, William D
Robberecht, Wim # ×
Issue Date: Feb-2013
Publisher: Macmillan
Series Title: Brain vol:136 issue:Pt 2 pages:471-82
Abstract: Oligodendrocytes are well known targets for immune-mediated and infectious diseases, and have been suggested to play a role in neurodegeneration. Here, we report the involvement of oligodendrocytes and their progenitor cells in the ventral grey matter of the spinal cord in amyotrophic lateral sclerosis, a neurodegenerative disease of motor neurons. Degenerative changes in oligodendrocytes were abundantly present in human patients with amyotrophic lateral sclerosis and in an amyotrophic lateral sclerosis mouse model. In the mouse model, morphological changes in grey matter oligodendrocytes became apparent before disease onset, increasingly so during disease progression, and oligodendrocytes ultimately died. This loss was compensated by increased proliferation and differentiation of oligodendrocyte precursor cells. However, these newly differentiated oligodendrocytes were dysfunctional as suggested by their reduced myelin basic protein and monocarboxylate transporter 1 expression. Mutant superoxide dismutase 1 was found to directly affect monocarboxylate transporter 1 protein expression. Our data suggest that oligodendroglial dysfunction may be a contributor to motor neuron degeneration in amyotrophic lateral sclerosis.
URI: 
ISSN: 0006-8950
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory for Neurobiology (Vesalius Research Center)
× corresponding author
# (joint) last author

Files in This Item:

There are no files associated with this item.

Request a copy

 




All items in Lirias are protected by copyright, with all rights reserved.

© Web of science