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In vivo phenotypic vascular dysfunction extends beyond the aorta in a mouse model for fibrillin-1 (Fbn1) mutation
T Curry1,2, M E Barrameda3, T Currier Thomas4,5,6,7,8
1College of Medicine-Phoenix, University of Arizona, Phoenix, AZ, USA.
Scientific Reports
|March 9, 2024
Summary
Marfan syndrome (MFS) with fibrillin-1 gene (FBN1) mutations accelerates vascular aging, particularly in males. This study reveals sex-dependent vascular dysfunction in MFS mice, mimicking premature aging phenotypes.
Area of Science:
- Cardiovascular Biology
- Genetics
- Aging Research
Background:
- Marfan syndrome (MFS), caused by fibrillin-1 gene (FBN1) mutations, leads to vascular wall weakening and aortic aneurysms.
- MFS mouse models exhibit accelerated aging in elastic organs, but sex and age impacts on in vivo vascular function are underexplored.
Purpose of the Study:
- To investigate sex-dependent alterations in central and cerebral vascular function in MFS mice compared to aging controls.
- To assess the impact of FBN1 mutations on vascular structure and function across different ages and sexes.
Main Methods:
- In vivo ultrasound imaging of central and cerebral vasculature in 6-month-old male and female MFS and control mice.
- Comparison with sex-matched 12-month-old healthy control mice to evaluate aging effects.
Main Results:
- MFS mice showed aortic enlargement and stiffness, more pronounced in males.
- Decreased posterior cerebral artery blood flow velocity in 6-month-old MFS males compared to controls.
- Reduced mitral valve early-filling velocities and left ventricular hypertrophy observed in male MFS mice.
Conclusions:
- Biological sex significantly influences vascular function and structure in MFS mice.
- MFS induces a premature vascular aging phenotype comparable to older healthy controls.
- Findings support re-evaluating MFS diagnostics and therapeutic strategies considering sex differences.
