Desmoplakin’s Role in Lung Fibrosis in HD AT2 Cells
Official title: Mechanistic studies of the genetic contribution of desmoplakin to pulmonary fibrosis in alveolar type 2 cells
Funded at Boston University Medical Campus · NIH-11377594 · Data updated 2026-11-12
A study in adult human lung cells aims to understand how a genetic change in desmoplakin may contribute to lung fibrosis.
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 | August 15, 2023 |
| Project end | July 31, 2027 |
| Funding year | 2026 |
| Award (this year) | $758,098 |
| NIH project number | 5R01HL166407-04 |
| Project ID | NIH-11377594 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are adults with or at risk for lung fibrosis who could contribute samples or data or benefit from downstream therapies stemming from this research.
✕Less likely to help
Patients without access to genetic testing or lung fibrosis may not directly benefit from this study.
What this research studies
Researchers use patient-derived iPSCs turned into alveolar type 2 (AT2) cells to model how DSP gene variations affect cell connections and signaling. They will examine how lower DSP levels influence Wnt/Tcf signaling and AT2 cell behavior, especially after injury. The goal is to connect a genetic variant to cellular changes that could lead to fibrosis in the lung. Results could point to new strategies for preventing or treating pulmonary fibrosis.
Why it matters
Potential benefit: If successful, the work could reveal how a specific genetic factor drives lung scarring and guide future therapies.
How similar studies have gone: Previous work using iPSCs to model AT2 cell biology has shown promise in connecting genetics to lung disease mechanisms, supporting this approach.
Where this research is happening
-
Boston University Medical Campus
Boston, United States Active
Researchers
- Principal investigator: Wilson, Andrew a — Boston University Medical Campus
- Study coordinator: Wilson, Andrew 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.