How copper gets into the cell's powerhouses (mitochondria)
Mitochondrial Copper Biology
This project looks at ways to deliver copper into mitochondria to help people with inherited copper‑handling problems like Menkes disease and some mitochondrial disorders.
Quick facts
| Grant type | NIH-funded research |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Texas A&m Agrilife Research NIH-funded |
| Lab location | 1 site (College Station, UNITED STATES) |
| Project ID | NIH-11254935 on NIH RePORTER |
What this research studies
From a patient's viewpoint, researchers are working to learn exactly how cells move and store copper so mitochondria can make energy. They use cells and animal models (including zebrafish) to find the proteins and pathways that control copper delivery to cytochrome c oxidase. The team has identified a drug candidate, elesclomol, that can carry copper to mitochondrial enzymes and restore energy production in lab models. The goal is to turn those basic discoveries into leads that could be tested as treatments for lethal infantile copper‑deficiency disorders.
Who could benefit from this research
Good fit: People with inherited copper‑transport disorders such as Menkes disease or mitochondrial conditions linked to copper deficiency would be the most relevant candidates for future clinical testing based on this work.
Not a fit: Patients whose conditions do not involve copper handling or cytochrome c oxidase dysfunction are unlikely to benefit from the findings of this research.
Why it matters
Potential benefit: If successful, this work could point to therapies that restore mitochondrial energy production in people with Menkes disease and related copper‑deficiency mitochondrial disorders.
How similar studies have performed: Preclinical lab and animal studies have shown that elesclomol can deliver copper to mitochondria and restore respiration, but clinical benefit in patients has not yet been demonstrated.
Where this research is happening
College Station, UNITED STATES
- Texas A&m Agrilife Research — College Station, United States (Active)
Researchers
- Principal investigator: Gohil, Vishal Mahendrasingh — Texas A&m Agrilife Research
- Study coordinator: Gohil, Vishal Mahendrasingh
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.