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Abstract #3148

Imaging the effect of high-protein diet on the brain Glutaryl-CoA dehydrogenase deficient mice using GluCEST MRI

Puneet Bagga1, Damodara Reddy1, Delia Talos2, Kimberly Sansalone2, Leah Jacobs2, Gaurav Verma1, Hari Hariharan1, and Ravinder Reddy1

1Department of Radiology, University of Pennsylvania, Philadelphia, PA, United States, 2Department of Neurology, University of Pennsylvania, Philadelphia, PA, United States

Autosomal recessive inheritance of Glutaryl-CoA dehydrogenase (GCDH) deficiency or Glutaric Acidemia-1 is one of the common inherited metabolic disorders affecting many children worldwide. We applied GluCEST MRI to image the metabolic changes in the brain of GCDH-/- mice following the exposure to high-protein diet. GluCEST contrast was found to be significantly reduced in the striatum and cortex of the GCDH-/- mice following high-protein diet exposure. 1H MRS data corroborated with GluCEST results showing a significant reduction in the striatal glutamate level. GluCEST MRI may be used as an imaging biomarker for assessing the neurovascular damage caused by high-protein diet in GCDH-/- subjects.

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