"Characterization of Cardiomyopathy in a Mouse Model of Duchenne Muscul" by Seyed Hamed Moazami
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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