Electronic Thesis and Dissertation Repository

Degree

Master of Science

Program

Medical Biophysics

Supervisor

Dr. Lisa Hoffman

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disease that is the result of a loss of functional dystrophin, which causes cardiomyocyte fibrosis and death, leading to cardiomyopathy. In this thesis, I have utilized dynamic contrast-enhanced computed tomography (DCE-CT), positron emission tomography-fluorodeoxyglucose (PET-FDG), echocardiography, and traditional histology to longitudinally assess disease progression and degree of cardiomyopathy in a murine model of DMD (mdx:utrn-/-). No significant changes were observed in the blood flow, blood volume, or cardiac volume measured via DCE-CT, nor in standard uptake value (SUV) of glucose as measured by PET-FDG in the left myocardium between and within the two study groups (of mdx:utrn -/- mice and healthy wild-type mice) over time. Our pilot echocardiography study and histological results show possible morphological/architectural and functional changes in affected myocardia of mdx:utrn-/- mice. These findings may provide us with an avenue to longitudinally characterize the progression of cardiomyopathy in the murine model of DMD, mdx:utrn -/- , in addition to providing a potential baseline for a comparison with future therapeutics.

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