Fibrillin-1 Deficiency Perturbs Aortic Cholinergic Relaxation and Adrenergic Contraction in a Mouse Model of Early Onset Progressively Severe Marfan Syndrome.

Abstract excerpt

INTRODUCTION: The pathogenic role of nitric oxide (NO) signaling during development of thoracic aortic aneurysm (TAA) in Marfan syndrome (MFS) is currently unclear. We characterized vasomotor function and its relationship to the activity of the NO-generating enzymes in mice with early onset progressively severe MFS. METHODS: Wire myography, immunoblotting, measurements of aortic NO, and superoxide levels were...

Topics

• Fibrillin-1
• Disease Models, Animal
• Marfan Syndrome
• Nitric Oxide
• Aortic Aneurysm, Thoracic
• Aorta, Thoracic