Understanding how fibroblasts influence muscular dystrophy progression
Official title: In vivo role of the fibroblast in muscular dystrophy-Renewal
Funded at Cincinnati Childrens Hosp Med Ctr · NIH-11401970 · Data updated 2026-11-12
This project studies how muscle-support cells interact with damaged muscle to drive disease in muscular dystrophy, using a mouse model to uncover new therapeutic targets for patients.
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 | April 1, 2018 |
| Project end | June 30, 2030 |
| Funding year | 2026 |
| Award (this year) | $324,009 |
| NIH project number | 5R01AR071301-07 |
| Project ID | NIH-11401970 on NIH RePORTER |
Who could benefit from this research
✓Could help
Ideal candidates are individuals with Ullrich or related collagen VI–related myopathies and their families, who may contribute samples or participate in future translational studies.
✕Less likely to help
Patients without CMD or those with different neuromuscular conditions may not directly benefit from these findings.
What this research studies
Muscular dystrophy, especially congenital forms like Ullrich CMD, is driven by defects in the muscle’s supporting structure. The researchers will use a Col6a2-deficient mouse model to explore how detachment of muscle fibers from the basement membrane activates integrin signals and TGFβ, promoting a cycle of degeneration. They aim to map the molecular steps that lead from basement-membrane injury to persistent muscle dysfunction, with 3 specific aims guiding the work. By clarifying these pathways, the study seeks to identify points where therapies could interrupt disease progression and improve muscle health. This effort could lay the groundwork for future treatments that target the cellular environment around muscle fibers.
Why it matters
Potential benefit: If successful, the findings could guide development of therapies that preserve muscle attachment and slow progression of CMD in patients.
How similar studies have gone: Previous work has shown that targeting the extracellular matrix and fiber attachment can influence disease trajectories in muscular dystrophies, supporting potential translational relevance here.
Where this research is happening
-
Cincinnati Childrens Hosp Med Ctr
Cincinnati, United States Active
Researchers
- Principal investigator: Vanhoutte, Davy — Cincinnati Childrens Hosp Med Ctr
- Study coordinator: Vanhoutte, Davy
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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.