How changes in mitochondria and nutrient use may drive lung blood vessel disease in children with congenital heart defects
Mitochondrial network remodeling and the glutamine-proline axis in pulmonary vascular disease associated with CHD.
This work looks at whether altered mitochondrial dynamics and glutamine-proline metabolism contribute to pulmonary blood vessel disease in infants and children with congenital heart defects.
Quick facts
| Grant type | P01 program project |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Florida International University NIH-funded |
| Lab location | 1 site (Miami, United States) |
| Project ID | NIH-11198737 on NIH RePORTER |
What this research studies
From a patient's viewpoint, the team is studying how the tiny powerhouses in blood vessel cells (mitochondria) change shape and how that shifts cell metabolism toward using different nutrients, which can stiffen and damage lung blood vessels. They focus on molecules that increase mitochondrial fission (like Drp1) and decrease fusion (like MFN2), and on a signaling pathway (JNK-Huwe1) that appears to break down MFN2. The researchers use patient-derived samples, cultured pulmonary artery endothelial cells, and animal models to trace how these changes lead to collagen buildup, cell overgrowth, and vessel stiffness. They will also test whether blocking the signaling that destroys MFN2 can restore healthier mitochondrial networks and improve vessel function.
Who could benefit from this research
Good fit: Ideal candidates are infants and children with congenital heart defects who have or are at risk for pulmonary vascular disease and who are followed by pediatric cardiology teams.
Not a fit: This project may not directly help adults without congenital heart defects or patients whose pulmonary vascular disease is already end-stage and not amenable to cell-level interventions.
Why it matters
Potential benefit: If successful, this work could point to new treatments that protect or restore healthy blood vessel cells and reduce pulmonary vascular disease in children with congenital heart defects.
How similar studies have performed: Previous studies have linked mitochondrial fission/fusion balance to vascular health, but targeting the JNK-Huwe1–MFN2 pathway in CHD-associated pulmonary vascular disease is a newer and less-tested approach.
Where this research is happening
Miami, United States
- Florida International University — Miami, United States (Active)
Researchers
- Principal investigator: Wang, Ting — Florida International University
- Study coordinator: Wang, Ting
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.