Alveolar Cell Dysfunction in Pulmonary Fibrosis: Using SFTPC Mutations to Find New Targets
Official title: Alveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular Targets
Funded at University of Pennsylvania · NIH-11575419 · Data updated 2026-11-12
This grant explores how rare SFTPC mutations in alveolar cells may drive IPF and seeks new molecular targets to improve treatment.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Pennsylvania NIH-funded |
| Lab location | 1 site (Philadelphia, United States) |
| Project start | July 15, 2019 |
| Project end | June 30, 2028 |
| Funding year | 2026 |
| Award (this year) | $223,438 |
| NIH project number | 3R01HL145408-07S1 |
| Project ID | NIH-11575419 on NIH RePORTER |
Who could benefit from this research
✓Could help
Adults with IPF or related alveolar epithelial dysfunction who may provide tissue samples or participate in related translational studies.
✕Less likely to help
Patients without IPF or those not contributing samples or participating in translational studies may not benefit directly.
What this research studies
Idiopathic pulmonary fibrosis (IPF) is a serious lung disease that damages the tiny air sacs where gas exchange happens. The research focuses on alveolar type 2 cells and how SFTPC gene mutations disrupt cell quality control and autophagy, contributing to disease. By studying these human lung cells in their native context, scientists aim to identify molecular changes and test potential therapeutic targets that could slow or halt scarring. The work builds on prior findings showing different mutant pathways activate cellular stress responses and impaired cleanup processes. The ultimate goal is to translate these insights into better therapies for IPF patients.
Why it matters
Potential benefit: If successful, the work could lead to new treatments that target the specific cellular defects driving IPF.
How similar studies have gone: Earlier work demonstrated that SFTPC mutations disrupt epithelial cell quality control and provoke disease-relevant stress responses, supporting a translational path.
Where this research is happening
-
University of Pennsylvania
Philadelphia, United States Active
Researchers
- Principal investigator: Beers, Michael Francis — University of Pennsylvania
- Study coordinator: Beers, Michael Francis
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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.