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Article

Oligodendrocyte pathology exceeds axonal pathology in white matter in human amyotrophic lateral sclerosis.

The Journal of pathology - 1 Jul 2020

Lorente Pons Alejandro, Higginbottom Adrian, Cooper-Knock Johnathan, Alrafiah Aziza, Alofi Ebtisam, Kirby Janine, Shaw Pamela J, Wood Jonathan D, Highley J Robin

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. The majority of cases are sporadic (sALS), while the most common inherited form is due to C9orf72 mutation (C9ALS). A high burden of inclusion pathology is seen in glia (including oligodendrocytes) in ALS, especially in C9ALS. Myelin basic protein (MBP) messenger RNA (mRNA) must be transported to oligodendrocyte processes for myelination, a...

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PMID: 32391572
DOI: 10.1002/path.5455

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