Alveolar Cell Dysfunction in Pulmonary Fibrosis: Using SFTPC Mutations to Find New Treatments
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-11365117 · Data updated 2026-11-12
A study focused on a specific lung cell defect linked to a common scarring lung disease (IPF) to uncover new targets for therapy.
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) | $632,964 |
| NIH project number | 5R01HL145408-07 |
| Project ID | NIH-11365117 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are adults with IPF or related lung conditions, and healthy volunteers may be included for comparison.
✕Less likely to help
Patients without IPF or related alveolar epithelial dysfunction may not directly benefit.
What this research studies
This project looks at how mutations in the SFTPC gene affect alveolar type 2 cells, which may drive IPF. Researchers will use human cells and model systems to understand how misfolded SP-C proteins disrupt cell quality control and lead to lung damage. The goal is to identify actionable molecular targets that could guide future therapies for IPF patients. The work builds on prior findings showing two distinct cell phenotypes related to SFTPC mutations and the unfolded protein response. By translating these insights into testable interventions, the study aims to move toward more effective treatments for IPF.
Why it matters
Potential benefit: If successful, the research could reveal new drug targets and help develop therapies that slow or halt lung scarring in IPF.
How similar studies have gone: Similar studies have shown that correcting cellular pathways affected by SFTPC mutations can improve cell function in disease models.
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.