TBX4-Driven Pulmonary Hypertension: Understanding Causes and Finding New Therapies
Official title: Tbx4-Driven Pulmonary Hypertension: Mechanisms and Therapeutic Targets
Funded at Vanderbilt University Medical Center · NIH-11340870 · Data updated 2026-11-12
A patient-focused look at how TBX4 gene changes can cause a form of high blood pressure in the lungs, especially in children, and how new models and tools might lead to better treatments.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Vanderbilt University Medical Center NIH-funded |
| Lab location | 1 site (Nashville, United States) |
| Project start | June 10, 2026 |
| Project end | March 31, 2028 |
| Funding year | 2026 |
| Award (this year) | $1,774,674 |
| NIH project number | 1R01HL185347-01 |
| Project ID | NIH-11340870 on NIH RePORTER |
Who could benefit from this research
✓Could help
Children with TBX4-related PAH or those with similar genetic risk factors may be eligible for future studies or trials.
✕Less likely to help
Patients whose PAH is not linked to TBX4 mutations may not benefit directly from these findings.
What this research studies
This grant studies how variants in TBX4 lead to pulmonary arterial hypertension by using three complementary tools: identifying TBX4 targets, creating a mouse model, and analyzing single-cell gene expression. The work aims to build a complete model of how loss of TBX4 function disrupts blood vessel formation and function, particularly through effects on pericytes and angiogenesis. Findings could point to new therapeutic targets that help patients, including children, with TBX4-related PAH. The project combines genetic, cellular, and animal experiments to translate basic biology into potential treatments. The research is conducted at Vanderbilt University Medical Center under the leadership of James D. West.
Why it matters
Potential benefit: If successful, the research could reveal new targets and pathways to treat or manage TBX4-related PAH, potentially improving outcomes for patients.
How similar studies have gone: Prior work using genetic models and single-cell analysis has shown promise in clarifying PAH mechanisms, with ongoing translation efforts.
Where this research is happening
-
Vanderbilt University Medical Center
Nashville, United States Active
Researchers
- Principal investigator: West, James D — Vanderbilt University Medical Center
- Study coordinator: West, James D
Related research
Other NIH-funded projects on the same topics.
-
Understanding how CD4 T cells fuel schistosomiasis-related lung disease
A study explores how specific immune cells and inflammatory signals in schistosomiasis may contribute to pulmonary hypertension in people affected by the disease.
-
CD4 T Cells, Schistosoma, and Pulmonary Hypertension: Understanding How Immune Cells Drive Disease
A human-health focused study uses a mouse model and human samples to explore how immune cells and inflammation may contribute to a lung blood vessel disease linked to schistosomiasis.
-
CD4 T Cells and Schistosoma-Related Pulmonary Hypertension
A study to understand how specific immune cells behave in a lung complication caused by schistosomiasis, with the goal of finding targets to prevent or treat it.
-
Clinical CT Imaging to identify and subtype pulmonary hypertension in COPD
A new CT imaging approach aims to help doctors detect and classify pulmonary hypertension in people with COPD.
-
Right heart remodeling in pulmonary hypertension
In people with pulmonary hypertension, researchers are studying how the right side of the heart changes and why it fails, with the goal of finding new treatments.
-
TEMPO-PH: How the right heart adapts in pulmonary hypertension over time
A study to build a detailed molecular portrait of why the right side of the heart fails in pulmonary hypertension and how a common heart medicine might help.
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.