How cellular 'folding helpers' keep key eye proteins working
Official title: Mechanisms of chaperone-mediated folding of beta-propeller proteins essential for vision.
Funded at Brigham Young University · NIH-11290774 · Data updated 2026-11-12
This project looks at how cellular chaperonin proteins fold important eye proteins that, when they fail, lead to inherited vision conditions like Bardet‑Biedl syndrome and Leber congenital amaurosis.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Brigham Young University NIH-funded |
| Lab location | 1 site (Provo, United States) |
| Project start | March 1, 2025 |
| Project end | February 28, 2029 |
| Funding year | 2026 |
| Award (this year) | $455,286 |
| NIH project number | 5R01EY036925-02 |
| Project ID | NIH-11290774 on NIH RePORTER |
Who could benefit from this research
✓Could help
People with inherited retinal disorders—particularly those with known mutations in Bardet‑Biedl syndrome genes or genes linked to Leber congenital amaurosis—would be the most relevant candidates to contribute genetic information or samples to related efforts.
✕Less likely to help
Patients whose vision loss is due to non‑genetic causes or entirely unrelated eye conditions are unlikely to receive direct benefit from this basic molecular research.
What this research studies
Researchers will determine the three‑dimensional structures of key visual proteins (Gb1 and Gb5) and their disease-causing variants to see how the CCT chaperonin and its partner PhLP1 guide their folding. Using structural methods and biochemical assays, the team will capture step‑by‑step folding stages and identify where mutations interrupt the normal folding trajectory. By comparing healthy and mutant proteins, they aim to explain how folding failures lead to loss of protein function in the visual system. These molecular insights could suggest ways to stabilize vulnerable proteins or guide future therapeutic strategies.
Why it matters
Potential benefit: If successful, this work could explain why specific genetic mutations cause inherited blindness and point to molecular targets for future treatments.
How similar studies have gone: Previous structural studies of chaperonin–substrate complexes have successfully mapped folding steps for other proteins, so this approach builds on established methods while applying them to new vision‑related proteins.
Where this research is happening
-
Brigham Young University
Provo, United States Active
Researchers
- Principal investigator: Willardson, Barry M — Brigham Young University
- Study coordinator: Willardson, Barry M
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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.