Understanding Lung Cell Problems in Pulmonary Fibrosis
Alveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular Targets
This research explores how specific genetic changes in lung cells contribute to pulmonary fibrosis, aiming to find new ways to help patients.
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 ID | NIH-11137775 on NIH RePORTER |
What this research studies
Idiopathic Pulmonary Fibrosis (IPF) is a serious lung disease where scar tissue forms, making it hard to breathe. We know that certain changes in special lung cells, called AT2 cells, play a big role in IPF. This project looks at how specific genetic errors in these AT2 cells cause them to malfunction, leading to the disease. By understanding these cell problems, we hope to discover new targets for future treatments that could slow or stop the scarring.
Who could benefit from this research
Good fit: This foundational research is relevant to adults diagnosed with Idiopathic Pulmonary Fibrosis, especially those with genetic mutations in the SFTPC gene.
Not a fit: Patients without pulmonary fibrosis or those whose condition is not linked to AT2 cell dysfunction may not directly benefit from this specific line of research.
Why it matters
Potential benefit: If successful, this work could lead to the discovery of new medications or therapies that specifically target the root causes of pulmonary fibrosis, offering better outcomes for patients.
How similar studies have performed: Previous work has shown that SFTPC mutations disrupt AT2 cell quality control, providing a strong foundation for this continued investigation into disease mechanisms.
Where this research is happening
Philadelphia, United States
- University of Pennsylvania — Philadelphia, United States (Active)
Researchers
- Principal investigator: Beers, Michael Francis — University of Pennsylvania
- Study coordinator: Beers, Michael Francis
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 below.