Understanding how Huntington disease disrupts protein production in brain cells
Official title: Mechanisms of Translational Dysregulation in Huntington Disease
Funded at Florida Atlantic University · NIH-11126038 · Data updated 2026-11-12
Researchers are studying Huntington disease to learn how a faulty protein blocks ribosomes and slows protein-making in brain cells, which may contribute to movement and thinking problems.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Florida Atlantic University NIH-funded |
| Lab location | 1 site (Boca Raton, United States) |
| Project start | July 1, 2024 |
| Project end | May 31, 2029 |
| Funding year | 2026 |
| Award (this year) | $381,250 |
| NIH project number | 5R01NS138278-03 |
| Project ID | NIH-11126038 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are individuals with Huntington disease or carriers with brain involvement who could contribute samples or be considered for future translational studies.
✕Less likely to help
People without Huntington disease or without involvement of ribosome dysfunction in HD would not directly benefit.
What this research studies
This project looks at why ribosomes stall during protein synthesis in brain cells affected by Huntington disease. Using advanced ribosome profiling and biochemical techniques, the team will map how mutant huntingtin interacts with RNA and ribosome-binding proteins to slow or stop production of other proteins. The study aims to connect molecular findings with the cognitive and motor symptoms seen in HD, potentially revealing new targets to slow disease progression. Results could inform future therapies that restore normal protein production in the brain.
Why it matters
Potential benefit: If successful, the work could identify new molecular targets to restore protein synthesis in HD, potentially slowing neurodegeneration and cognitive decline.
How similar studies have gone: Preliminary findings and prior work suggest ribosome interactions with mutant huntingtin are linked to impaired protein synthesis, supporting feasibility.
Where this research is happening
-
Florida Atlantic University
Boca Raton, United States Active
Researchers
- Principal investigator: Subramaniam, Srinivasa — Florida Atlantic University
- Study coordinator: Subramaniam, Srinivasa
Related research
Other NIH-funded projects on the same topics.
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Harnessing neuron-derived vesicles to find Huntington’s disease biomarkers
A research project aims to identify brain-derived biomarkers in blood that reflect Huntington disease activity, using neuronal extracellular vesicles as the signal source.
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Understanding how blood flow problems in the brain affect Huntington’s disease
A study at a major hospital is looking at how blood vessels in the brain may contribute to symptoms and brain changes in Huntington’s disease.
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Rhes‑SUMO pathway's role in Huntington's disease
This work looks at whether the Rhes‑SUMO protein system increases toxic huntingtin and helps it spread in the brain, which may explain early striatal damage in people with Huntington's disease.
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Understanding how ribosome stalling affects Huntington disease
This study is looking at how a problem with protein production in the brain might contribute to Huntington disease, which could help us find new ways to treat the condition.
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Rhes-SUMO Pathway and Huntington Disease in Mice
A study in mouse models explores how the Rhes-SUMO pathway may drive brain and nerve cell vulnerability in Huntington disease.
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Investigating the role of the Rhes-SUMO pathway in Huntington's disease
This study is looking at how a specific pathway in the brain might affect the worsening of Huntington's disease, with the hope of finding new ways to help manage the symptoms and slow down the disease for those living with it.
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.