TDP-43 linked nerve cell electrical problems in ALS and frontotemporal dementia
Official title: Excitability Dysfunction Mechanisms Underlying the TDP43-Dependent ALS and FTD Pathogenesis
Funded at Wright State University · NIH-11377867 · Data updated 2026-11-12
This project looks at how TDP-43-related changes in nerve cell electrical activity may lead to ALS and frontotemporal dementia, using mouse models that mirror most patients' disease.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Wright State University NIH-funded |
| Lab location | 1 site (Dayton, United States) |
| Project start | August 1, 2023 |
| Project end | July 31, 2028 |
| Funding year | 2025 |
| Award (this year) | $79,238 |
| NIH project number | 3R01NS131816-03S2 |
| Project ID | NIH-11377867 on NIH RePORTER |
Who could benefit from this research
✓Could help
People with ALS or frontotemporal dementia—especially sporadic cases where TDP-43 protein clumps are present—are the groups most likely to benefit from these findings.
✕Less likely to help
Patients whose disease is driven by SOD1 mutations or other non–TDP-43 mechanisms may be less likely to benefit directly from results focused on TDP-43-related changes.
What this research studies
From a patient's point of view, researchers are using a newer TDP-43 mouse model (rNLS8) together with the well-known SOD1 model to see whether motor neurons become too active, not active enough, or both during disease. They will record electrical signals from motor neurons over time and compare those patterns with TDP-43 protein clumps and neuron loss. The team aims to sort out conflicting past results to learn whether excitability changes help or harm neurons at different stages. That clearer picture could guide future treatments that try to normalize nerve cell activity in ALS and related frontotemporal dementia.
Why it matters
Potential benefit: If successful, this work could identify time windows and targets for therapies that protect motor neurons in ALS and related frontotemporal dementia.
How similar studies have gone: Prior work in SOD1 mouse models has shown mixed results about motor neuron excitability, and studying excitability in the rNLS8 TDP-43 model is a relatively new approach.
Where this research is happening
-
Wright State University
Dayton, United States Active
Researchers
- Principal investigator: Elbasiouny, Sherif M — Wright State University
- Study coordinator: Elbasiouny, Sherif M
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About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page.