Why people with osteogenesis imperfecta have lung problems
Investigating pulmonary complications due to abnormal collagen/ER stress in Osteogenesis Imperfecta
This project looks at whether faulty type I collagen and ongoing cell stress cause lung damage and breathing problems in people with osteogenesis imperfecta.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of California Los Angeles NIH-funded |
| Lab location | 1 site (Los Angeles, United States) |
| Project ID | NIH-11309113 on NIH RePORTER |
What this research studies
Researchers will use two mouse models that carry common type I collagen mutations seen in OI to mimic how the disease may affect the lungs. They will study lung structure, cell types, and cell-to-cell communication at multiple stages of development and after lung injury. The team will measure abnormal collagen secretion, signs of chronic endoplasmic reticulum (ER) stress in collagen-producing cells, and changes in signaling pathways. Results will be compared to known OI lung problems to help point toward ways to monitor or protect lungs in people with OI.
Who could benefit from this research
Good fit: People with osteogenesis imperfecta, particularly those with known type I collagen mutations or with respiratory symptoms, would be most relevant to this research.
Not a fit: People without OI or whose lung disease is caused by unrelated conditions are unlikely to benefit directly from this specific project.
Why it matters
Potential benefit: If successful, this work could identify mechanisms behind lung problems in OI and suggest targets for monitoring or future treatments to protect breathing.
How similar studies have performed: Previous lab and animal work has suggested abnormal collagen and ER stress affect tissues in OI, but applying those findings to lung structure and therapies remains largely untested.
Where this research is happening
Los Angeles, United States
- University of California Los Angeles — Los Angeles, United States (Active)
Researchers
- Principal investigator: Zieba, Jennifer — University of California Los Angeles
- Study coordinator: Zieba, Jennifer
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.