Investigating how a specific gene affects lung health and fibrosis
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-10903825 · Data updated 2026-11-12
This study is looking at how a specific gene called desmoplakin affects lung health in people with pulmonary fibrosis, aiming to learn more about how our lungs repair themselves and find better ways to treat this condition.
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 | 2024 |
| Award (this year) | $764,263 |
| NIH project number | 5R01HL166407-02 |
| Project ID | NIH-10903825 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates for this research include adults with a genetic predisposition to pulmonary fibrosis or related lung diseases.
✕Less likely to help
Patients with lung conditions unrelated to genetic factors or those without a history of pulmonary fibrosis may not benefit from this research.
What this research studies
This research focuses on understanding the role of the desmoplakin gene in pulmonary fibrosis, a condition that affects lung function. By studying how genetic variations influence the behavior of alveolar type 2 cells, which are crucial for lung repair and maintenance, the research aims to uncover the mechanisms behind lung diseases. The team will use advanced techniques to model these cells and analyze how changes in desmoplakin impact their function, particularly in response to injury. This could lead to insights into how to better manage or treat pulmonary fibrosis.
Why it matters
Potential benefit: If successful, this research could lead to new strategies for preventing or treating pulmonary fibrosis, improving lung health for affected patients.
How similar studies have gone: Previous research has shown promising results in understanding genetic contributions to lung diseases, suggesting that this approach could yield valuable insights.
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
Related research
Other NIH-funded projects on the same topics.
-
Molecular clues to progressive lung scarring
Researchers are studying how lung tissue becomes progressively scarred, focusing on cell signals and receptors that may drive fibrosis in people with lung disease.
-
Alveolar Cell Dysfunction in Pulmonary Fibrosis: Using SFTPC Mutations to Find New Targets
This grant explores how rare SFTPC mutations in alveolar cells may drive IPF and seeks new molecular targets to improve treatment.
-
Iron and scar-forming cells in the lungs (IPF)
Researchers are exploring how iron in the lungs may drive the scarring seen in idiopathic pulmonary fibrosis and whether this could lead to new therapies.
-
Repurposing Pyrvinium as an Inhaled Therapy for Idiopathic Pulmonary Fibrosis
A patient-friendly look at a project trying to use an existing drug, pyrvinium, as an inhaled treatment option for idiopathic pulmonary fibrosis (IPF).
-
Screening new compounds to reverse gene signatures in IPF lungs
A researcher hopes to find compounds that reverse harmful gene changes in the lungs of people with idiopathic pulmonary fibrosis (IPF).
-
Role of lactate transporters in lung scarring
Aims to see if blocking lactate transporters can slow or stop scar-forming cells from thickening the lungs in people with fibrotic lung disease.
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.