What causes tiny airway cilia to fail in lung disease
Regulation of Motile Cilia Assembly in Lung Disease
This project looks at how tiny, hair-like structures (motile cilia) in the airways form and malfunction in people with primary ciliary dyskinesia and some forms of COPD to explain differences in symptom severity.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Washington University NIH-funded |
| Lab location | 1 site (Saint Louis, United States) |
| Project ID | NIH-11333335 on NIH RePORTER |
What this research studies
If you take part, researchers will examine cilia from airway samples using high-resolution imaging (cryo-electron microscopy) and protein analyses to see which parts are missing or misplaced. They will compare genetic variants, including CCDC39 and CCDC40, to understand why some people have much worse lung disease. Lab studies and cell experiments will test how cilia are built and where the assembly process breaks down. Findings from multiple patients will be linked to symptoms to map how specific genetic changes affect lung health.
Who could benefit from this research
Good fit: Ideal participants are people diagnosed with primary ciliary dyskinesia, people with known PCD-related genetic variants (such as CCDC39 or CCDC40), or patients with unexplained bronchiectasis or suspected ciliary dysfunction.
Not a fit: People whose lung disease is clearly caused by other mechanisms (for example, smoking-related COPD without evidence of ciliary defects) or who cannot provide samples are unlikely to receive direct benefit from this project.
Why it matters
Potential benefit: If successful, this work could improve genetic diagnosis and point to ways to protect or restore airway cilia, potentially improving lung clearance and symptoms for affected patients.
How similar studies have performed: Previous work using cryo-EM and proteomics has successfully identified missing ciliary proteins and linked genes like CCDC39/CCDC40 to severe PCD, so this project builds on established, promising methods.
Where this research is happening
Saint Louis, United States
- Washington University — Saint Louis, United States (Active)
Researchers
- Principal investigator: Brody, Steven — Washington University
- Study coordinator: Brody, Steven
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.