Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction

Mutations in the gene encoding the RNA-binding protein TDP-43 cause amyotrophic lateral sclerosis (ALS), clinically and pathologically indistinguishable from the majority of ‘sporadic’ cases of ALS, establishing altered TDP-43 function and distribution as a primary mechanism of neurodegeneration. Tr...

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Main Authors: Gordon, D, Dafinca, R, Scaber, J, Alegre-Abarrategui, J, Farrimond, L, Scott, C, Biggs, D, Kent, L, Oliver, P, Davies, B, Ansorge, O, Wade-Martins, R, Talbot, K
格式: Journal article
出版: Elsevier 2018