Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 7, Pages -Publisher
MDPI
DOI: 10.3390/ijms22073426
Keywords
oligodendrocyte; demyelination; axonal degeneration
Funding
- French Amyotrophic Lateral Sclerosis Association [RAK19097LLP]
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Motor neurons and their axons reaching the skeletal muscle have long been considered the primary targets of degeneration in ALS, but recent research suggests that glial cells, particularly oligodendrocytes, also play a significant role. Oligodendrocytes are now recognized to be critically involved in axonal injury and are affected by toxic effects of degenerative neurons.
Motor neurons and their axons reaching the skeletal muscle have long been considered as the best characterized targets of the degenerative process observed in amyotrophic lateral sclerosis (ALS). However, the involvement of glial cells was also more recently reported. Although oligodendrocytes have been underestimated for a longer time than other cells, they are presently considered as critically involved in axonal injury and also conversely constitute a target for the toxic effects of the degenerative neurons. In the present review, we highlight the recent advances regarding oligodendroglial cell involvement in the pathogenesis of ALS. First, we present the oligodendroglial cells, the process of myelination, and the tight relationship between axons and myelin. The histological abnormalities observed in ALS and animal models of the disease are described, including myelin defects and oligodendroglial accumulation of pathological protein aggregates. Then, we present data that establish the existence of dysfunctional and degenerating oligodendroglial cells, the chain of events resulting in oligodendrocyte degeneration, and the most recent molecular mechanisms supporting oligodendrocyte death and dysfunction. Finally, we review the arguments in support of the primary versus secondary involvement of oligodendrocytes in the disease and discuss the therapeutic perspectives related to oligodendrocyte implication in ALS pathogenesis.
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