Nerve cell excitability changes in ALS and frontotemporal dementia linked to TDP‑43
Excitability dysfunction mechanisms underlying the TDP43-dependent ALS and FTD pathogenesis
Researchers will look at how TDP‑43 related changes alter motor neuron electrical activity in models of ALS and frontotemporal dementia to better understand causes of nerve cell loss.
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 ID | NIH-11377866 on NIH RePORTER |
What this research studies
This project uses mouse models that mirror the TDP‑43 changes seen in most ALS and many FTD patients to study how motor neurons change their electrical behavior over time. The team will compare a newer rNLS8 TDP‑43 model with the established G93A SOD1 model to see when and where neurons become overactive or underactive. They will record electrical signals from motor neurons and examine brain and spinal cord tissue to link excitability changes with TDP‑43 inclusions and neuron loss. Results aim to clarify whether excitability shifts are harmful or protective during disease.
Who could benefit from this research
Good fit: People living with ALS or frontotemporal dementia, especially those with suspected TDP‑43–linked disease or interested in contributing samples to related research, would be most relevant.
Not a fit: Patients seeking immediate therapies or those with genetic ALS forms not linked to TDP‑43 may not directly benefit from this basic animal-focused research.
Why it matters
Potential benefit: If successful, this work could clarify when and how motor neuron electrical changes drive degeneration, pointing to timing and targets for future treatments to protect neurons in ALS and FTD.
How similar studies have performed: Previous work in SOD1 mouse models has shown mixed hyperexcitability and hypoexcitability results, and excitability has not yet been characterized in the rNLS8 TDP‑43 model.
Where this research is happening
Dayton, United States
- Wright State University — Dayton, United States (Active)
Researchers
- Principal investigator: Elbasiouny, Sherif M — Wright State University
- Study coordinator: Elbasiouny, Sherif M
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 below.