Lang, R., Hodgson, R.E. and Shelkovnikova, T.A. orcid.org/0000-0003-1367-5309 (2024) TDP-43 in nuclear condensates: where, how, and why. Biochemical Society Transactions, 52 (4). pp. 1809-1825. ISSN 0300-5127
Abstract
TDP-43 is an abundant and ubiquitously expressed nuclear protein that becomes dysfunctional in a spectrum of neurodegenerative diseases. TDP-43's ability to phase separate and form/enter biomolecular condensates of varying size and composition is critical for its functionality. Despite the high density of phase-separated assemblies in the nucleus and the nuclear abundance of TDP-43, our understanding of the condensate-TDP-43 relationship in this cellular compartment is only emerging. Recent studies have also suggested that misregulation of nuclear TDP-43 condensation is an early event in the neurodegenerative disease amyotrophic lateral sclerosis. This review aims to draw attention to the nuclear facet of functional and aberrant TDP-43 condensation. We will summarise the current knowledge on how TDP-43 containing nuclear condensates form and function and how their homeostasis is affected in disease.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2024 The Author(s). This is an open access article published by Portland Press Limited on behalf of the Biochemical Society and distributed under the Creative Commons Attribution License 4.0 (CC BY) https://creativecommons.org/licenses/by/4.0/ |
Keywords: | ALS; TDP-43; condensate; neurodegeneration; nuclear body; paraspeckle; stress response; Humans; DNA-Binding Proteins; Cell Nucleus; Amyotrophic Lateral Sclerosis; Biomolecular Condensates; Animals; Neurodegenerative Diseases |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > School of Medicine and Population Health |
Funding Information: | Funder Grant number BIOTECHNOLOGY AND BIOLOGICAL SCIENCES RESEARCH COUNCIL BB/V014110/1 BIOTECHNOLOGY AND BIOLOGICAL SCIENCES RESEARCH COUNCIL BB/X018393/1 |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 17 Oct 2024 10:21 |
Last Modified: | 17 Oct 2024 10:21 |
Status: | Published |
Publisher: | Portland Press Ltd. |
Refereed: | Yes |
Identification Number: | 10.1042/bst20231447 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:218318 |