Article
An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress.
Neurobiology of disease - 1 Oct 2020
Feneberg Emily, Gordon David, Thompson Alexander G, Finelli Mattéa J, Dafinca Ruxandra, Candalija Ana, Charles Philip D, Mäger Imre, Wood Matthew J, Fischer Roman, Kessler Benedikt M, Gray Elizabeth, Turner Martin R, Talbot Kevin
Abstract excerpt
TDP-43 pathology is a key feature of amyotrophic lateral sclerosis (ALS), but the mechanisms linking TDP-43 to altered cellular function and neurodegeneration remain unclear. We have recently described a mouse model in which human wild-type or mutant TDP-43 are expressed at low levels and where altered stress granule formation is a robust phenotype of TDP-43M337V/- expressing cells. In the present study we use...
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