TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration
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From the earliest stages of embryogenesis and throughout life, transcriptional regulation is carefully orchestrated in order to generate, shape, and reshape the central nervous system (CNS). TAR DNA-binding protein 43 (TDP-43) is identified as a regulator of essential transcriptional events in the CNS. Evidence for its importance comes from the identification of TDP-43 protein aggregates and genetic mutations in patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Efforts are being made to learn more about the biological function of TDP-43 and gain a better understanding of its role in neurodegeneration. TDP-43 RNA targets and protein interactions have now been identified, and in vivo evidence shows that TDP-43 is essential in CNS development and function. This review will highlight aspects of these findings.
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TDP-43 is an RNA-binding protein that regulates RNA metabolism, including mRNA splicing, stability, and transport; after acute traumatic injury in animal models, TDP-43 expression is upregulated and TDP-43 relocates from the neuronal nucleus to accumulate in the neuronal cytoplasm.
“TDP-43 is an RNA-binding protein that regulates RNA metabolism, including mRNA splicing, stability, and transport [167]. After acute traumatic injury in animal models, TDP-43 expression is upregulated and TDP-43 relocates from the neuronal nucleus to accumulate in the neuronal cytoplasm [134, 135]”
From The neuropathology of sport (2014) · cites this paper as [167]
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- Authors
- Sephton, C. F., Cenik, B., Cenik, B. K., Herz, J., & Yu, G.
- Affiliations
- The University of Texas Southwestern Medical Center (US)
- The University of Texas at Dallas (US)
- Corresponding author
- Sephton, Chantelle F.
- Yu, Gang
- Venue
- bchm
- Publisher
- Walter de Gruyter GmbH
- Published
- 2012-07-01
- Volume
- 393
- Issue
- 7
- Pages
- 589-594
- ISSN
- 1431-6730 (ISSN-L)
- 1437-4315
- PMC
- PMC3537500
- OpenAlex
- W138705543
- Cited by
- 86
- References
- 48
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 4 · 2023: 8 · 2022: 7 · 2021: 20 · 2020: 7 · 2019: 7 · 2018: 5 · 2017: 3 · 2016: 2 · 2015: 6 · 2014: 8 · 2013: 4
- Type
- conference abstract
- Language
- en
- Open access
- green
- europepmc.org
- Locations
- Biological Chemistry (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- American Health Assistance Foundation
- National Institute on Aging (R01AG029547)
- National Heart, Lung, and Blood Institute (R01HL063762, R37HL063762, P01HL020948)
- National Institute of Neurological Disorders and Stroke (RC1NS068697)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Keywords
- TDP-43, central nervous system development, transcriptional regulation, neurodegeneration, amyotrophic lateral sclerosis, frontotemporal lobar degeneration, protein-protein interaction, embryonic development
- MeSH terms
- Animals, Brain, DNA-Binding Proteins, Humans, Ribonucleoproteins, RNA, Spinal Cord, Neurodegenerative Diseases
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