Nuclear rupture and repair in LMNA-related cardiomyopathy
Official title: Ikegami - R01 - Nuclear Rupture and Repair in Lamin-Associated Cardiomyopathy
Funded at Cincinnati Childrens Hosp Med Ctr · NIH-11427136 · Data updated 2026-11-12
A study to understand how defects in the nuclear envelope cause a dangerous heart disease and to test new ways to repair it.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Cincinnati Childrens Hosp Med Ctr NIH-funded |
| Lab location | 1 site (Cincinnati, United States) |
| Project start | September 5, 2026 |
| Project end | May 31, 2030 |
| Funding year | 2026 |
| Award (this year) | $608,215 |
| NIH project number | 1R01HL187557-01 |
| Project ID | NIH-11427136 on NIH RePORTER |
Who could benefit from this research
✓Could help
Patients with LMNA-related dilated cardiomyopathy or heart tissue affected by LMNA mutations.
✕Less likely to help
Patients without LMNA mutations or with non-disease-related heart conditions may not benefit.
What this research studies
LMNA-related dilated cardiomyopathy is a deadly genetic heart condition with few treatment options beyond transplantation. The research uses mouse models and human heart tissue to study how ruptures in the nuclear envelope may drive disease and how repairing those ruptures could protect the heart. The team will develop fluorescent tools to identify heart cells with nuclear rupture, examine how rupture affects gene expression and heart cell contraction, and test gene therapy strategies to enhance repair or prevent re-rupture. Findings from patient samples will validate the mechanism and guide a potential therapy.
Why it matters
Potential benefit: If successful, this work could lead to a therapy that prevents heart damage in LMNA-DCM and reduces the need for heart transplantation.
How similar studies have gone: Prior studies have linked nuclear rupture to LMNA-DCM and shown that repairing ruptures could be protective, but therapeutic approaches are still in development.
Where this research is happening
-
Cincinnati Childrens Hosp Med Ctr
Cincinnati, United States Active
Researchers
- Principal investigator: Ikegami, Kohta — Cincinnati Childrens Hosp Med Ctr
- Study coordinator: Ikegami, Kohta
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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.