Non-Invasive Imaging of Neurological Glycogen Storage Disease

Scope of Work-University of Kentucky The Sun and Gentry lab established a high throughput metabolomics and MALDI pipeline that utilizes high- resolution mass spectrometry and bioinformatics software developed internally for rapid comprehensive profile of polar metabolites and glycogen/carbohydrate molecular imaging. The goal of this R01 subcontract project is to facilitate Dr. Nirbhay Yadav to further decipher the metabolic perturbations mouse model of neurodegeneration, with a strong focus on terminal glycogen levels and associated metabolites. Dr. Yadav will use the Sun/Gentry lab’s metabolomics and MALDI imaging expertise to 1) define the metabolism and disease pathology of mouse models of neurodegeneration and aging; 2) define the glycogen structure in association with NMR defined glycogen distribution in live mouse brains; and 3) to determine neuropathology in mouse models of neurodegeneration. Dr. Sun, Dr. Relich, Dr. Gentry, and Dr. Bruntz will assist in sample preparation, data curation/batch analysis, and system level interpretations. There will be machine time (GCMS and MALDI) available for Dr. Yadav’s planned experiments and current estimated sample size. Dr. Sun, Dr. Relich, Dr. Bruntz, and Dr. Gentry will have regular meetings over zoom with Dr. Yadav to evaluation project progression and offer help on manuscripts preparation.