Nerve-cell excitability in TDP-43 linked ALS and frontotemporal dementia
Excitability dysfunction mechanisms underlying the TDP43-dependent ALS and FTD pathogenesis
['FUNDING_R01'] · WRIGHT STATE UNIVERSITY · NIH-11164543
Researchers are looking at how TDP-43–related changes in nerve‑cell activity might harm motor neurons in ALS and frontotemporal dementia.
Quick facts
| Phase | ['FUNDING_R01'] |
|---|---|
| Study type | Nih_funding |
| Sex | All |
| Sponsor | WRIGHT STATE UNIVERSITY (nih funded) |
| Locations | 1 site (DAYTON, UNITED STATES) |
| Trial ID | NIH-11164543 on ClinicalTrials.gov |
What this research studies
This project uses a newer rNLS8 mouse model that develops TDP‑43 protein changes like those seen in most people with ALS and many with frontotemporal dementia. The team will measure motor neuron excitability with electrophysiology and cellular analyses to see whether neurons become overactive, underactive, or both. They will compare findings to the common SOD1 mouse model where excitability results have conflicted, to clarify which changes are harmful or protective. The work is preclinical (mouse and lab-based) and does not currently enroll human participants.
Who could benefit from this research
Good fit: People with ALS or frontotemporal dementia linked to TDP‑43 pathology would be most relevant to future clinical work informed by this research.
Not a fit: Patients with rare ALS forms that lack TDP‑43 pathology, such as some SOD1 mutation cases, may be less likely to benefit directly from these findings.
Why it matters
Potential benefit: If successful, this work could reveal targets to correct nerve‑cell activity and slow or prevent motor neuron loss in ALS and related dementias.
How similar studies have performed: Prior studies using SOD1 mouse models have produced mixed results on neuron excitability, so applying these methods to a TDP‑43 model is relatively novel and intended to resolve those inconsistencies.
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.
Conditions: Amyotrophic Lateral Sclerosis Motor Neuron Disease