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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