Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits

Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits

Abstract Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of the same disease spectrum of adult-onset neurodegenerative diseases that affect the motor and cognitive functions, respectively. Multiple common genetic loci such as fused in sarcoma (FUS) have been...

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Bibliographic Details
Main Authors: Wan Yun Ho, Ira Agrawal, Sheue-Houy Tyan, Emma Sanford, Wei-Tang Chang, Kenneth Lim, Jolynn Ong, Bernice Siu Yan Tan, Aung Aung Kywe Moe, Regina Yu, Peiyan Wong, Greg Tucker-Kellogg, Edward Koo, Kai-Hsiang Chuang, Shuo-Chien Ling
Format: Article
Language:English
Published: BMC 2021-01-01
Series:Acta Neuropathologica Communications
Subjects:
Amyotrophic lateral sclerosis (ALS)
Frontotemporal dementia (FTD)
FUS (fused in sarcoma)
Auto-regulation
Nonsense-mediated decay (NMD)
Protein homeostasis
Online Access:https://doi.org/10.1186/s40478-020-01111-4

Internet

https://doi.org/10.1186/s40478-020-01111-4

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