How changes in microfibrils affect blood vessel development and related diseases
Impact of microfibril turnover on vascular development and disease
This study is looking at how a protein called ADAMTS6 affects the tiny fibers that help keep blood vessels strong, especially in people with Marfan syndrome, to find new ways to help prevent serious heart problems like aortic aneurysms.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Case Western Reserve University NIH-funded |
| Lab location | 1 site (Cleveland, United States) |
| Project ID | NIH-10986095 on NIH RePORTER |
What this research studies
This research investigates the role of a specific protein, ADAMTS6, in the turnover of microfibrils, which are crucial for the structure of blood vessels. It focuses on understanding how mutations in the FBN1 gene, which lead to Marfan syndrome, can cause serious vascular issues like aortic aneurysms. By studying the effects of ADAMTS6 on vascular smooth muscle cells, the research aims to identify new therapeutic strategies that could modify the disease process and improve patient outcomes. The approach includes genetic analysis and mouse models to explore the relationship between microfibril turnover and vascular health.
Who could benefit from this research
Good fit: Ideal candidates for this research are individuals diagnosed with Marfan syndrome or those at risk due to genetic factors related to FBN1 mutations.
Not a fit: Patients without genetic predispositions to Marfan syndrome or those with unrelated vascular conditions may not benefit from this research.
Why it matters
Potential benefit: If successful, this research could lead to new treatments that prevent or reduce the severity of vascular complications in patients with Marfan syndrome.
How similar studies have performed: Previous research has shown promising results in targeting similar pathways in connective tissue disorders, suggesting potential for success in this novel approach.
Where this research is happening
Cleveland, United States
- Case Western Reserve University — Cleveland, United States (Active)
Researchers
- Principal investigator: Mead, Timothy Joseph — Case Western Reserve University
- Study coordinator: Mead, Timothy Joseph
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.