Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis
Publication year
2013Source
Brain, 136, Pt 2, (2013), pp. 471-82ISSN
Publication type
Article / Letter to editor
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Organization
Pathology
Journal title
Brain
Volume
vol. 136
Issue
iss. Pt 2
Page start
p. 471
Page end
p. 82
Subject
ONCOL 3 - Translational research DCN MP - Plasticity and memoryAbstract
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.
This item appears in the following Collection(s)
- Academic publications [245262]
- Faculty of Medical Sciences [93207]
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