Article
ALS-linked mutant TDP-43 in oligodendrocytes induces oligodendrocyte damage and exacerbates motor dysfunction in mice.
Acta neuropathologica communications - 27 Nov 2024
Horiuchi Mai, Watanabe Seiji, Komine Okiru, Takahashi Eiki, Kaneko Kumi, Itohara Shigeyoshi, Shimada Mayuko, Ogi Tomoo, Yamanaka Koji
Abstract excerpt
Nuclear clearance and cytoplasmic aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) are pathological hallmarks of amyotrophic lateral sclerosis (ALS) and its pathogenic mechanism is mediated by both loss-of-function and gain-of-toxicity of TDP-43. However, the role of TDP-43 gain-of-toxicity in oligodendrocytes remains unclear. To investigate the impact of excess TDP-43 on oligodendrocytes, we established...
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