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...

Full description

Bibliographic Details
Main Authors: David Gordon, Ruxandra Dafinca, Jakub Scaber, Javier Alegre-Abarrategui, Lucy Farrimond, Connor Scott, Daniel Biggs, Louisa Kent, Peter L. Oliver, Benjamin Davies, Olaf Ansorge, Richard Wade-Martins, Kevin Talbot
Format: Article
Language:English
Published: Elsevier 2019-01-01
Series:Neurobiology of Disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996118306600