Understanding how inherited prion diseases affect the brain
Official title: Mechanisms of Neurodegeneration in Hereditary Prion Diseases
Funded at Boston University Medical Campus · NIH-11168162 · Data updated 2026-11-12
This study is looking at how certain gene changes related to hereditary prion diseases affect brain cells, using special lab-grown cells to better understand how these changes might lead to problems like Alzheimer's disease.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Boston University Medical Campus NIH-funded |
| Lab location | 1 site (Boston, United States) |
| Project start | June 1, 2025 |
| Project end | May 31, 2030 |
| Funding year | 2025 |
| Award (this year) | $869,478 |
| NIH project number | 1R01NS143202-01 |
| Project ID | NIH-11168162 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates for this research include individuals with a family history of hereditary prion diseases or specific mutations in the PRNP gene.
✕Less likely to help
Patients with sporadic forms of prion diseases or those without genetic mutations related to prion disorders may not benefit from this research.
What this research studies
This research investigates the cellular and molecular mechanisms behind hereditary prion diseases, particularly focusing on how mutations in the PRNP gene lead to neurodegeneration. By utilizing innovative induced pluripotent stem cell (iPSC) technology, the study aims to analyze the effects of mutant prion proteins on human neurons and glial cells. The goal is to uncover the toxic properties of these proteins and their interactions within the brain, which could provide insights into the progression of related conditions like Alzheimer's disease.
Why it matters
Potential benefit: If successful, this research could lead to new therapeutic strategies for treating neurodegenerative diseases linked to prion mutations.
How similar studies have gone: Previous research has shown promise in understanding prion diseases through animal models, but this approach using human-derived cells is relatively novel.
Where this research is happening
-
Boston University Medical Campus
Boston, United States Active
Researchers
- Principal investigator: Harris, David a — Boston University Medical Campus
- Study coordinator: Harris, David a
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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.