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Cytoplasmic functions of TDP-43 and FUS and their role in ALS

Birsa, N; Bentham, MP; Fratta, P; (2020) Cytoplasmic functions of TDP-43 and FUS and their role in ALS. Seminars in Cell & Developmental Biology , 99 pp. 193-201. 10.1016/j.semcdb.2019.05.023. Green open access

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Abstract

TAR DNA-binding protein of 43 kDa (TDP-43) and fused in sarcoma (FUS) are RNA binding proteins (RBPs) primarily located in the nucleus, and involved in numerous aspects of RNA metabolism. Both proteins can be found to be depleted from the nucleus and accumulated in cytoplasmic inclusions in two major neurodegenerative conditions, amyotrophic lateral sclerosis and frontotemporal dementia. Recent evidences suggest that, in addition to their nuclear functions, both TDP-43 and FUS are involved in multiple processes in the cytoplasm, including mRNA stability and transport, translation, the stress response, mitochondrial and autophagy regulation. Here, we review the most recent advances in understanding their functions in the cytoplasm and how these are affected in disease.

Type: Article
Title: Cytoplasmic functions of TDP-43 and FUS and their role in ALS
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.semcdb.2019.05.023
Publisher version: https://doi.org/10.1016/j.semcdb.2019.05.023
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: ALS, FUS, RNP, TDP-43, stress granules, translation
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10075956
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