How TGF-β and SOX18 affect lung blood vessel stiffness in congenital heart defect–related lung disease
TGF-ß-SOX18-Collagen metabolism in pulmonary vascular disease associated with CHD
This research looks at whether the signaling proteins TGF-β and SOX18 change collagen and make lung blood vessels stiffer in people born with certain 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-11198735 on NIH RePORTER |
What this research studies
If your child was born with a heart defect that increases blood flow to the lungs, researchers are using an animal model that mimics this condition to see how lung blood vessels remodel. They isolate lung endothelial cells and study changes in cell metabolism, focusing on SOX18 and TGF-β signaling and how these pathways alter collagen production and artery stiffness. The team combines tissue studies, cellular experiments, and molecular analyses to trace how metabolic shifts lead to vessel stiffening. Their goal is to identify molecular steps that could be targeted to prevent harmful remodeling.
Who could benefit from this research
Good fit: Ideal candidates are children born with left-to-right congenital heart defects that cause increased pulmonary blood flow and early pulmonary vascular changes.
Not a fit: Patients without congenital heart defects or those with long-standing, end-stage pulmonary hypertension unrelated to increased pulmonary blood flow are unlikely to benefit directly from this work.
Why it matters
Potential benefit: If successful, this work could point to new ways to prevent or reverse pulmonary hypertension and artery stiffening in people with congenital heart defects.
How similar studies have performed: Preclinical animal and cell studies have shown metabolic rewiring and upregulation of SOX18 in this model, but translating these findings into human therapies remains 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.