Journal
BRAIN
Volume 136, Issue -, Pages 471-482Publisher
OXFORD UNIV PRESS
DOI: 10.1093/brain/aws339
Keywords
motor neuron disease; ALS; NG2 cell; oligodendrocyte; dysfunction
Categories
Funding
- Fund for Scientific Research Flanders, University of Leuven [GOA/11/014]
- Interuniversity Attraction Poles program of the Belgian Federal Science Policy Office [[P7/16]
- European Community [259867]
- E. von Behring Chair for Neuromuscular and Neurodegenerative Disorders
- UK Medical Research Council
- Wellcome Trust
- MRC [G9708005, G0800575] Funding Source: UKRI
- Medical Research Council [G9708005, G0800575] Funding Source: researchfish
- Wellcome Trust [100269/Z/12/Z] Funding Source: researchfish
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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.
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