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Article

Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43G298S transgenic mice.

Experimental neurology - 1 Jan 2021

Sieverding Kirsten, Ulmer Johannes, Bruno Clara, Satoh Takashi, Tsao William, Freischmidt Axel, Akira Shizuo, Wong Philip C, Ludolph Albert C, Danzer Karin M, Lobsiger Christian S, Brenner David, Weishaupt Jochen H

Abstract excerpt

Mutations in the genes TARDBP (encoding the TDP-43 protein) and TBK1 can cause familial ALS. Neuronal cytoplasmatic accumulations of the misfolded, hyperphosphorylated RNA-binding protein TDP-43 are the pathological hallmark of most ALS cases and have been suggested to be a key aspect of ALS pathogenesis. Pharmacological induction of autophagy has been shown to reduce mutant TDP-43 aggregates and alleviate motor...

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PMID: 33038415
DOI: 10.1016/j.expneurol.2020.113496

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