Keywords: Epilepsy, Deuterium, metabolism, glycolysis, TCA cycle, glucose, beta-hydroxybutyrate
Motivation: The ketogenic diet is a first-line clinical treatment for glucose-transporter-1 deficiency syndrome (GLUT1-DS).
Goal(s): To examine brain energy metabolism in a mouse model of GLUT1-DS by applying deuterium metabolic imaging (DMI) to mice fed either a normal diet (ND) or ketogenic diet (KD).
Approach: Following administration of [6,6-2H2]glucose (Glc) or [3,4,4,4-2H4]β-hydroxybutyrate (βHB), employ DMI to evaluate GLUT1-DS mice and wild-type (WT) littermates on ND or KD.
Results: The KD results in significant changes in levels of substrates Glc and βHB, and downstream metabolites, compared to the ND.
Impact: DMI is directly clinically translatable. These results suggest it holds promise for quantification of the metabolic changes in GLUT1-DS patients, thereby relating these changes to the underlying pathophysiology, clinical symptoms and treatment efficacy in individual patients.
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