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Cerebral metabolic derangements as translatable biomarkers in pantothenate kinase-associated neurodegeneration mouse model via 1H MRS

Puneet Bagga¹, Jeffrey Steinberg², Walter Akers², Zoltan Patay¹, Beth McCarville¹, Chitra Subramanian³, Charles O Rock³, and Suzanne Jackowski³
¹Department of Diagnostic Imaging, St Jude Children's Research Hospital, Memphis, TN, United States, ²Center for In Vivo Imaging and Therapeutics (CIVIT), St Jude Children's Research Hospital, Memphis, TN, United States, ³Department of Infectious Diseases, St Jude Children's Research Hospital, Memphis, TN, United States

1H MRS detectable metabolic derangements in a preclinical model of pantothenate kinase-associated neurodegeneration (PKAN) as biomarkers of drug response.

Figure 2. Representative ¹H MR spectra from three groups Left. Wild Type, Mid. Pank1,2 neuronal KO, and Right. Pank1,2 neuronal KO mouse treated with BBP-671

Figure 3. Metabolite to total creatine ratios of glutamate+glutamine (Glx), γ-amino butyric acid, N-acetyl aspartate, and lactate