Understanding a Gene's Role in Lung Scarring
Mechanistic studies of the genetic contribution of desmoplakin to pulmonary fibrosis in alveolar type 2 cells
This project explores how a specific gene called desmoplakin contributes to lung scarring, also known as pulmonary fibrosis, by affecting important lung cells.
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 ID | NIH-11139420 on NIH RePORTER |
What this research studies
Our bodies have many genes, and sometimes small changes in these genes can make us more likely to develop certain health conditions. This project looks at a gene called desmoplakin (DSP), which helps keep lung cells strong and healthy. We believe that a specific change in the DSP gene might weaken these lung cells, called alveolar type 2 cells, making them more prone to scarring. To understand this better, we are using special lab-grown human lung cells to see how this gene change affects their function and contributes to pulmonary fibrosis.
Who could benefit from this research
Good fit: This research is relevant for adults aged 21 and older who have or are at risk for pulmonary fibrosis, especially those with genetic factors contributing to the condition.
Not a fit: Patients whose pulmonary fibrosis is not linked to genetic factors or the specific desmoplakin gene may not directly benefit from this particular line of research.
Why it matters
Potential benefit: If successful, this work could uncover new ways to prevent or treat pulmonary fibrosis by targeting the specific genetic pathways involved.
How similar studies have performed: Previous large-scale genetic analyses have successfully identified genes linked to pulmonary fibrosis, and lab models using human cells are increasingly used to understand these genetic connections.
Where this research is happening
Boston, United States
- Boston University Medical Campus — Boston, United States (Active)
Researchers
- Principal investigator: Wilson, Andrew a — Boston University Medical Campus
- Study coordinator: Wilson, Andrew a
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.