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Oligodendrocytes in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: the New Players on Stage

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Specialty Molecular Biology
Date 2024 Apr 8
PMID 38585368
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Abstract

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal adult-onset neurodegenerative disorders that share clinical, neuropathological and genetic features, which forms part of a multi-system disease spectrum. The pathological process leading to ALS and FTD is the result of the combination of multiple mechanisms that operate within specific populations of neurons and glial cells. The implication of oligodendrocytes has been the subject of a number of studies conducted on patients and related animal models. In this review we summarize our current knowledge on the alterations specific to myelin and the oligodendrocyte lineage occurring in ALS and FTD. We also consider different ways by which specific oligodendroglial alterations influence neurodegeneration and highlight the important role of oligodendrocytes in these two intrinsically associated neurodegenerative diseases.

References
1.
Mackenzie I, Ansorge O, Strong M, Bilbao J, Zinman L, Ang L . Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation. Acta Neuropathol. 2011; 122(1):87-98. PMC: 3319073. DOI: 10.1007/s00401-011-0838-7. View

2.
Ling S, Polymenidou M, Cleveland D . Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron. 2013; 79(3):416-38. PMC: 4411085. DOI: 10.1016/j.neuron.2013.07.033. View

3.
Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar H, Mackenzie I . A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain. 2009; 132(Pt 11):2922-31. PMC: 2768659. DOI: 10.1093/brain/awp214. View

4.
Stadelmann C, Timmler S, Barrantes-Freer A, Simons M . Myelin in the Central Nervous System: Structure, Function, and Pathology. Physiol Rev. 2019; 99(3):1381-1431. DOI: 10.1152/physrev.00031.2018. View

5.
Antonioni A, Raho E, Lopriore P, Pace A, Latino R, Assogna M . Frontotemporal Dementia, Where Do We Stand? A Narrative Review. Int J Mol Sci. 2023; 24(14). PMC: 10380352. DOI: 10.3390/ijms241411732. View