Targeting DNA Damage Pathways in LMNA-Related Heart Disease
Official title: Mechanisms and Therapeutic Targeting of DNA Damage in Dilated Cardiomyopathy Caused by LMNA Mutations
Funded at University of Texas Hlth Sci Ctr Houston · NIH-11394336 · Data updated 2026-11-12
A study to understand how damaged DNA and inflammatory signals contribute to dilated cardiomyopathy caused by LMNA mutations and explore therapies that can protect the heart.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Texas Hlth Sci Ctr Houston NIH-funded |
| Lab location | 1 site (Houston, United States) |
| Project start | March 15, 2016 |
| Project end | May 31, 2028 |
| Funding year | 2026 |
| Award (this year) | $568,920 |
| NIH project number | 5R01HL132401-11 |
| Project ID | NIH-11394336 on NIH RePORTER |
Who could benefit from this research
✓Could help
Individuals with LMNA-related dilated cardiomyopathy or related cardiac muscle cell disorders who may contribute samples or participate in future translational studies.
✕Less likely to help
Patients without LMNA-related cardiomyopathy may not directly benefit from this specific research.
What this research studies
This project looks at how broken DNA in heart cells triggers inflammation and fibrosis in LMNA-related cardiomyopathy. Researchers use advanced chromatin and DNA-damage techniques to map where breaks occur and how the CGAS-STING1 pathway influences heart disease in mouse models and human samples. The goal is to identify therapeutic targets that can improve heart function and reduce scarring. The work builds on prior findings showing that blocking CGAS can extend survival and lessen damage in affected hearts.
Why it matters
Potential benefit: If successful, this work could lead to new treatments that reduce damage and improve heart function for people with LMNA-related cardiomyopathy.
How similar studies have gone: Previous studies in LMNA cardiomyopathy with CGAS pathway modulation showed improved heart function and survival in models, suggesting a promising approach.
Where this research is happening
-
University of Texas Hlth Sci Ctr Houston
Houston, United States Active
Researchers
- Principal investigator: Marian, Ali J — University of Texas Hlth Sci Ctr Houston
- Study coordinator: Marian, Ali J
Related research
Other NIH-funded projects on the same topics.
-
Discovery of rare genetic variants linked to dilated cardiomyopathy using big biobank data
A large-scale genetic study will look for rare gene variants that affect dilated cardiomyopathy to improve how we diagnose and manage the condition.
-
Understanding how muscle ribosome mutations cause severe neonatal dilated cardiomyopathy and ways to treat it
This grant focuses on a rare genetic heart condition in newborns caused by mutations in a muscle-specific ribosomal protein, with hopes to find new ways to diagnose and treat it.
-
Unlocking how rare genetic changes cause dilated cardiomyopathy
A large study will look at genetic data from many people to understand which rare variants affect dilated cardiomyopathy and how this changes risk and treatment.
-
Role of Lmod2 in heart muscle and dilated cardiomyopathy
This NIH grant explores how a heart-specific protein (Lmod2) helps heart cells contract properly and how its problems can cause dilated cardiomyopathy in infants and children.
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Using miRNA to find new treatment paths for dilated cardiomyopathy
Researchers are studying heart-muscle disease (dilated cardiomyopathy) to discover miRNA-based clues that could lead to targeted therapies for patients.
-
Skeletal muscle actin mutations and heart disease: understanding how a specific mutation affects heart function
A physician-scientist at Johns Hopkins is studying how a skeletal muscle protein mutation (ACTA1 R256H) affects heart muscle function to help people with dilated cardiomyopathy.
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.