“STRESSED OUT”: The role of FUS and TDP-43 in amyotrophic lateral sclerosis

“STRESSED OUT”: The role of FUS and TDP-43 in amyotrophic lateral sclerosis.

Full text not available from this repository.
Item Type: Article
Status: Published
Official URL: https://doi.org/10.1016/j.biocel.2020.105821
Journal or Publication Title: The International Journal of Biochemistry & Cell Biology
Volume: 126
Page Range: p. 105821
Date: 2020
Divisions: Directed Evolution
Depositing User: General Admin
Identification Number: 10.1016/j.biocel.2020.105821
ISSN: 13572725
Date Deposited: 04 Jan 2021 03:40
Abstract:

Mutations in fused-in-sarcoma (FUS) and TAR DNA binding protein-43 (TDP-43; TARDBP) are known to cause the severe adult-onset neurodegenerative disorder amyotrophic lateral sclerosis (ALS). Proteinopathy caused by cellular stresses such as endoplasmic reticulum (ER) stress, oxidative stress, mitochondrial stress and proteasomal stress and the formation of stress granules (SGs), cytoplasmic aggregates and inclusions is a hallmark of ALS. FUS and TDP-43, which are DNA/RNA binding proteins that regulate transcription, RNA homeostasis and protein translation are implicated in ALS proteinopathy. Disease-causing mutations in FUS and TDP-43 cause sequestration of these proteins and their interacting partners in the cytoplasm, which leads to aggregation. This mislocalization and formation of aggregates and SGs is cytotoxic and a contributor to neuronal death. We explore how loss-of-nuclear-function and gain-of-cytoplasmic function mechanisms that affect FUS and TPD-43 localization can generate a ‘stressed out’ neuronal pathology and proteinopathy that drives ALS progression.

Creators:
Creators
Email
Aksoy, Yagiz Alp
UNSPECIFIED
Deng, Wei
UNSPECIFIED
Stoddart, Jack
UNSPECIFIED
Chung, Roger
UNSPECIFIED
Guillemin, Gilles
UNSPECIFIED
Cole, Nicholas James
UNSPECIFIED
Neely, Graham Gregory
UNSPECIFIED
Hesselson, Daniel
UNSPECIFIED
Last Modified: 04 Jan 2021 03:40
URI: https://eprints.centenary.org.au/id/eprint/734

Actions (login required)

View Item View Item