Nucleolus dysfunction and ALS: understanding motor neuron disease at the cellular level
Official title: The Nucleolus as a Nexus of Disease in Amyotrophic Lateral Sclerosis
Funded at Montana State University -Billings · NIH-11359779 · Data updated 2026-11-12
Aims to understand how problems in the neuron’s nucleolus contribute to ALS and how key proteins behave in models to guide future therapies for people with ALS.
Quick facts
| Grant type | R15 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Montana State University -Billings NIH-funded |
| Lab location | 1 site (Billings, United States) |
| Project start | September 1, 2015 |
| Project end | June 30, 2029 |
| Funding year | 2026 |
| Award (this year) | $484,014 |
| NIH project number | 2R15NS090384-04 |
| Project ID | NIH-11359779 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are individuals with ALS or those with a genetic/molecular predisposition studied through model systems or biomarker analyses linked to nucleolar biology.
✕Less likely to help
People without ALS or those not connected to nucleolar pathways may not directly benefit.
What this research studies
This project investigates why the nucleolus inside motor neurons malfunctions in ALS and how this may drive disease. Researchers will study the behavior of TDP-43 and a Cronos protein isoform in mouse and yeast models to map their roles in nucleolar biology. They plan to characterize the RNA and regulatory interactions that affect nucleolar TDP-43 and to determine what Cronos does when removed from motor neurons. Findings could reveal new targets for therapies that protect motor neurons in ALS. The work is conducted using established animal and yeast models to mirror human disease mechanisms.
Why it matters
Potential benefit: If successful, the work could identify new molecular targets to slow or halt motor neuron degeneration in ALS.
How similar studies have gone: Previous studies have linked TDP-43 and nucleolar dysfunction to ALS, supporting a promising avenue for new therapies but exact mechanisms remain under investigation.
Where this research is happening
-
Montana State University -Billings
Billings, United States Active
Researchers
- Principal investigator: George, Lynn D — Montana State University -Billings
- Study coordinator: George, Lynn D
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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.