Abstract #3201

Pre- and post-symptomatic longitudinal metabolic assessment of the Twitcher mouse model of Krabbe Disease at 9.4 Tesla

Poonam Choudhary^1,2, Nadav I. Weinstock³, Marilena Preda⁴, Lawrence Wrabetz^2,3, Robert Zivadinov^2,4, and Ferdinand Schweser^2,4

¹Department of Medical Physics, University at Buffalo, The State University of New York, Buffalo, NY, United States, ²Buffalo Neuroimaging Analysis Center, Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, Buffalo, NY, United States, ³Department of Biochemistry, Hunter James Kelly Research Institute, Buffalo, NY, United States, ⁴Center for Biomedical Imaging at the Clinical and Translational Science Institute, University at Buffalo, The State Univeristy of New York, Buffalo, NY, United States

Krabbe Disease (KD) is a rare progressive globoid cell leukodystrophy caused by a deficiency of galactocerebrosidase (GALC), necessary for the metabolism of galactosylceramide and psychosine. Accumulation of these neurotoxic sphingolipids results in demyelination, neuroinflammation and ultimately death in infancy. This study aimed to investigate if localized proton magnetic resonance spectroscopy (¹H-MRS) may serve as useful markers to detect pre-symptomatic metabolic alterations related to KD.

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