How cells build the inner membrane of mitochondria
BIOGENESIS OF THE MITOCHONDRIAL INNER MEMBRANE
This project looks for small molecules that change how mitochondria bring proteins inside to help people with nerve and muscle diseases linked to mitochondrial problems.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of California Los Angeles NIH-funded |
| Lab location | 1 site (Los Angeles, United States) |
| Project ID | NIH-11190833 on NIH RePORTER |
What this research studies
Researchers will study how proteins are moved into mitochondria using yeast, zebrafish, and human cells to learn what goes wrong in disease. They will run a cell-based screen in mammalian cells to find chemical probes that alter mitochondrial protein import. Promising molecules will be tested for effects on mitochondrial stress responses and selective removal of damaged mitochondria (mitophagy). Findings from the model organisms will be compared to human-cell results to connect the basic biology to nerve and muscle disorders.
Who could benefit from this research
Good fit: People with mitochondrial disorders or neurodegenerative or neuromuscular conditions linked to mitochondrial dysfunction, such as certain forms of ataxia, would be most relevant to this work.
Not a fit: Patients whose conditions are unrelated to mitochondrial protein import or caused by defects outside mitochondrial pathways are unlikely to benefit from these findings.
Why it matters
Potential benefit: If successful, this work could produce drug-like molecules that correct mitochondrial protein import and slow or reverse degeneration in some nerve and muscle diseases.
How similar studies have performed: Basic studies have mapped mitochondrial protein import for years, but finding specific small-molecule modulators in mammalian cells is relatively new and not yet proven clinically.
Where this research is happening
Los Angeles, United States
- University of California Los Angeles — Los Angeles, United States (Active)
Researchers
- Principal investigator: Koehler, Carla M — University of California Los Angeles
- Study coordinator: Koehler, Carla 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.