New roles for a mitochondrial RNA modifier in building ribosomes
Official title: New Mechanisms of the Pseudouridine Synthase Module in Mitoribosome Assembly
Funded at State University of New York at Buffalo · NIH-11401749 · Data updated 2026-11-12
A researcher team at SUNY Buffalo will study how a mitochondrial RNA modifying enzyme helps assemble ribosomes and how mutations linked to mitochondrial diseases disrupt this process.
Quick facts
| Grant type | NIH-funded research |
|---|---|
| Study type | NIH-funded research |
| Funding institution | State University of New York at Buffalo NIH-funded |
| Lab location | 1 site (Amherst, United States) |
| Project start | August 5, 2022 |
| Project end | June 30, 2027 |
| Funding year | 2026 |
| Award (this year) | $400,250 |
| NIH project number | 5R35GM147123-06 |
| Project ID | NIH-11401749 on NIH RePORTER |
Who could benefit from this research
✓Could help
Patients with mitochondrial diseases caused by defective ribosome assembly or rRNA modification may be relevant for future translational studies.
✕Less likely to help
Individuals without mitochondrial disease are unlikely to directly benefit from this basic research.
What this research studies
This project focuses on how the RPUSD4 pseudouridine synthase, with helper factors, guides the precise modification of mitochondrial rRNA during ribosome assembly. By combining structural biology, genomics, and biochemistry, the team will map how this enzyme achieves site-specific action and why disease-causing mutations disturb it. The work aims to reveal signals that coordinate RNA modification with ribosome formation, which could illuminate mechanisms behind complex mitochondrial diseases. Findings could identify new targets for understanding or treating conditions caused by mitochondrial dysfunction.
Why it matters
Potential benefit: Understanding these mechanisms could lead to better insight into mitochondrial diseases and potentially identify new avenues for diagnosis or therapy.
How similar studies have gone: Previous studies show that RNA modifications and ribosome assembly are linked to disease, supporting potential translational relevance, though this specific mechanism is being explored.
Where this research is happening
-
State University of New York at Buffalo
Amherst, United States Active
Researchers
- Principal investigator: Pillon, Monica — State University of New York at Buffalo
- Study coordinator: Pillon, Monica
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.