Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy
Open accessAbstract
Glial proliferation and activation are associated with disease progression in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia. In this study, we describe a unique platform to address the question of cell autonomy in tran s active response DNA-binding protein (TDP-43) proteinopathies. We generated functional astroglia from human induced pluripotent stem cells carrying an ALS-causing TDP-43 mutation and show that mutant astrocytes exhibit increased levels of TDP-43, subcellular mislocalization of TDP-43, and decreased cell survival. We then performed coculture experiments to evaluate the effects of M337V astrocytes on the survival of wild-type and M337V TDP-43 motor neurons, showing that mutant TDP-43 astrocytes do not adversely affect survival of cocultured neurons. These observations reveal a significant and previously unrecognized glial cell-autonomous pathological phenotype associated with a pathogenic mutation in TDP-43 and show that TDP-43 proteinopathies do not display an astrocyte non-cell-autonomous component in cell culture, as previously described for SOD1 ALS. This study highlights the utility of induced pluripotent stem cell-based in vitro disease models to investigate mechanisms of disease in ALS and other TDP-43 proteinopathies.
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TARDBPM337V increases the risk of death in iPSC-derived astrocytes.
“but also increases the risk of death in iPSC-derived astrocytes [299].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [299]
Expression of TARDBPM337V in astrocytes exerts cell autonomous toxicity.
“expression of TARDBPM337V in astrocytes exerts cell autonomous toxicity [299].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [299]
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- Authors
- Serio, A., Bilican, B., Barmada, S. J., Ando, D. M., Zhao, C., Siller, R., Burr, K., Haghi, G., Story, D., Nishimura, A. L., Carrasco, M. A., Phatnani, H. P., Shum, C., Wilmut, I., Maniatis, T., Shaw, C. E., Finkbeiner, S., & Chandran, S.
- Affiliations
- MRC Centre for Regenerative Medicine (GB)
- Gladstone Institutes (US)
- Euan MacDonald Centre for Motor Neurone Disease Research, Centre for Neuroregeneration, and Medical Research Council Centre for Regenerative Medicine, University of Edinburgh, Edinburgh EH16 4UU, United Kingdom;
- King's College London (GB)
- Department of Biochemistry and Molecular Biophysics, Columbia University, New York, NY 10032
- Columbia University (US)
- ORCID
- Venue
- Proceedings of the National Academy of Sciences
- Publisher
- National Academy of Sciences
- Published
- 2013-02-11
- Volume
- 110
- Issue
- 12
- Pages
- 4697-4702
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- PMC
- PMC3607024
- OpenAlex
- W1987426179
- Cited by
- 345
- References
- 47
- Citations by year
- 2026: 9 · 2025: 20 · 2024: 24 · 2023: 22 · 2022: 24 · 2021: 37 · 2020: 29 · 2019: 30 · 2018: 15 · 2017: 32 · 2016: 24 · 2015: 34 · 2014: 26 · 2013: 17
- FWCI
- 21.38
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Proceedings of the National Academy of Sciences (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Research Portal (King's College London) (submittedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
- Queen Mary Research Online (Queen Mary University of London) (submittedVersion)
- Columbia Academic Commons (Columbia University)
- Funders
- Fidelity Foundation
- Wellcome Trust (089701)
- Motor Neurone Disease Association (Chandran/Mar10/6751)
- National Institutes of Health
- Medical Research Council (MC_G1000733)
- National Institute of General Medical Sciences (T32GM008568)
- National Institute of Neurological Disorders and Stroke (K08NS072233, DP1NS082099, R01NS039074, R01NS083390)
- National Center for Chronic Disease Prevention and Health Promotion (8DP1NS082099-06)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Pluripotent Stem Cells Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- TDP-43 proteinopathy, astrocytes, cell autonomy, amyotrophic lateral sclerosis, frontotemporal lobar dementia, induced pluripotent stem cells, astrocyte-neuron co-culture, motor neuron survival, TDP-43 mislocalization, glial activation, in vitro disease model
- MeSH terms
- Amyotrophic Lateral Sclerosis, Astrocytes, Cell Line, Cell Survival, DNA-Binding Proteins, Humans, Male, Middle Aged, Motor Neurons, Mutation, Coculture Techniques, Cell Proliferation, Induced Pluripotent Stem Cells
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