How a mitochondrial protein (pp60Src) affects lung blood vessels in children born with heart defects
Mitochondrial pp60Src and cellular metabolism in pulmonary vascular disease associated with CHD
This project looks at whether changes in a mitochondrial protein called pp60Src change energy use in lung blood-vessel cells of children born with congenital heart defects that cause extra blood flow to the lungs.
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-11198736 on NIH RePORTER |
What this research studies
From a patient perspective, the research team studies cells that line the lung blood vessels and animal models that mimic the extra blood flow caused by certain congenital heart defects to understand why those vessels become abnormal. They focus on a mitochondrial protein called pp60Src and how chemical changes to mitochondrial proteins alter the assembly and activity of the cell’s energy machinery (Complex I of the electron transport chain). The group uses lab experiments on endothelial cells, biochemical mapping of phospho-tyrosine sites on mitochondrial proteins, and a lamb model of pulmonary over-circulation to link these molecular changes to vessel function. The goal is to reveal mechanisms that could point toward new ways to prevent or treat pulmonary vascular disease in children with these heart defects.
Who could benefit from this research
Good fit: Children (infants and young children) born with congenital heart defects that cause pulmonary over-circulation and related pulmonary endothelial dysfunction would be the most relevant candidates for related clinical studies or sample donation.
Not a fit: Children without congenital heart defects or those whose heart defects do not cause extra lung blood flow are unlikely to benefit directly from this project.
Why it matters
Potential benefit: If successful, this work could point to new treatments that protect or restore healthy lung blood-vessel function in children with congenital heart defects and reduce long-term pulmonary vascular complications.
How similar studies have performed: The research team’s prior lab and animal data show that altering pp60Src changes mitochondrial Complex I activity and endothelial function, but translating these molecular findings into human therapies is still early and largely untested.
Where this research is happening
Miami, United States
- Florida International University — Miami, United States (Active)
Researchers
- Principal investigator: Black, Stephen M — Florida International University
- Study coordinator: Black, Stephen M
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.