CHCHD10 and its role in dementia and ALS
Official title: CHCHD10 Frontotemporal Dementia, Molecular Mechanisms and Therapeutic Strategies
Funded at Weill Medical Coll of Cornell Univ · NIH-11380813 · Data updated 2026-11-12
Researchers are studying how mutations in CHCHD10 may cause brain diseases like frontotemporal dementia and ALS, with the goal of uncovering how the protein misfolds and forms damaging structures.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Weill Medical Coll of Cornell Univ NIH-funded |
| Lab location | 1 site (New York, United States) |
| Project start | August 22, 2024 |
| Project end | July 31, 2029 |
| Funding year | 2026 |
| Award (this year) | $808,996 |
| NIH project number | 5R01NS139141-03 |
| Project ID | NIH-11380813 on NIH RePORTER |
Who could benefit from this research
✓Could help
People with frontotemporal dementia, ALS, or Alzheimer’s-type symptoms who have CHCHD10-related pathology or mutations.
✕Less likely to help
Individuals without CHCHD10 mutations or related pathology may not benefit directly.
What this research studies
This project will look at how CHCHD10 mutations affect the protein’s shape and interactions inside mitochondria, using advanced biochemical and structural techniques. The team will examine mouse brain models and human brain tissue to see if mutant CHCHD10 forms amyloid aggregates in real life. They aim to determine if different mutations create distinct structural patterns that drive disease processes, including mitochondrial stress and neuronal damage. The results could reveal new targets for therapies that stabilize protein folding or prevent harmful aggregation. Ultimately, the work seeks to connect molecular changes to the progression of dementia and ALS-related pathology.
Why it matters
Potential benefit: If successful, this research could lead to new therapeutic strategies that prevent or reduce neuronal damage caused by CHCHD10 mutations.
How similar studies have gone: Previous findings show mutant CHCHD10 forms amyloid aggregates, suggesting a plausible path to therapy, but detailed structures and brain-confirmed relevance remain to be clarified.
Where this research is happening
-
Weill Medical Coll of Cornell Univ
New York, United States Active
Researchers
- Principal investigator: Fujita, Hibiki — Weill Medical Coll of Cornell Univ
- Study coordinator: Fujita, Hibiki
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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.