Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

Abstract Nucleolar stress has been implicated in the pathology and disease pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) from repeat expansions of GGGGCC in C9orf72 (C9-ALS/FTLD) but not in sporadic ALS (SALS). Previously we reported that antisense...

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Bibliographic Details
Main Authors: Olubankole Aladesuyi Arogundade, Sandra Nguyen, Ringo Leung, Danielle Wainio, Maria Rodriguez, John Ravits
Format: Article
Language:English
Published: BMC 2021-02-01
Series:Acta Neuropathologica Communications
Online Access:https://doi.org/10.1186/s40478-021-01125-6