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

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.