Cell stress response to misfolded rhodopsin in retinitis pigmentosa
Unfolded Protein Response in Drosophila models of Retinitis Pigmentosa
This project looks at how cells cope with misfolded rhodopsin to help people with retinitis pigmentosa keep their vision.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | New York University School of Medicine NIH-funded |
| Lab location | 1 site (New York, United States) |
| Project ID | NIH-11169873 on NIH RePORTER |
What this research studies
Researchers use fruit flies that carry a mutant rhodopsin gene (ninaEG69D) which causes protein misfolding and age-related retinal degeneration similar to human retinitis pigmentosa. They focus on the cell's unfolded protein response (UPR), especially the PERK→ATF4 signaling branch that helps cells fold or clear bad proteins in the endoplasmic reticulum. By changing genes and following eye degeneration and molecular markers in the flies, they test which pathways slow or speed vision loss. The long-term aim is to turn genetic and cellular findings into targets that could be explored for treatments in people.
Who could benefit from this research
Good fit: Adults with retinitis pigmentosa, especially those with known rhodopsin mutations, are the most relevant group for future applications of this work.
Not a fit: People whose vision loss is due to causes unrelated to rhodopsin misfolding or those with very advanced retinal degeneration are less likely to receive direct benefit.
Why it matters
Potential benefit: Could identify molecular targets to slow or prevent vision loss in people with rhodopsin-related retinitis pigmentosa.
How similar studies have performed: Previous work in fruit fly models has shown that modifying UPR pathways can change retinal degeneration, but translating those findings to human therapies remains unproven.
Where this research is happening
New York, United States
- New York University School of Medicine — New York, United States (Active)
Researchers
- Principal investigator: Ryoo, Hyung D — New York University School of Medicine
- Study coordinator: Ryoo, Hyung D
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.