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Abstract #0743

In Vivo OXPHOS Measurement by Magnetic Resonance Imaging in Metabolic Myopathy

Catherine DeBrosse 1 , Ravi Prakash Reddy Nanga 1 , Neil Wilson 1 , Kevin D'Aquilla 1 , Mark Elliott 1 , Hari Hariharan 1 , Felicia Yan 2 , Leat Perez 2 , Sara Nguyen 2 , Elizabeth McCormick 3 , Marni Falk 3,4 , Shana McCormack 2,4 , and Ravinder Reddy 1

1 Center for Magnetic Resonance and Optical Imaging, Department of Radiology, University of Pennsylvania, Philadelphia, Pennsylvania, United States, 2 Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, PA, United States, 3 Division of Human Genetics, The Children's Hospital of Philadelphia, Philadelphia, PA, United States, 4 Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States

Patients with metabolic myopathies experience exercise intolerance due to oxidative phosphorylation (OXPHOS) defects. OXPHOS capacity is proportional to the recovery of phosphocreatine (PCr) after exercise, measured with 31 P magnetic resonance spectroscopy (MRS). To maintain ATP during exercise, creatine (Cr) levels increase in proportion to PCr depletion. Therefore, OXPHOS can also be measured using the rate of Cr recovery. We have implemented an imaging technique with high spatial resolution and improved sensitivity over 31 P MRS to measure Cr recovery: creatine chemical exchange saturation transfer (CrCEST). Using CrCEST, we observed differences in resting Cr levels and post-exercise recovery rates in patients vs. healthy subjects.

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