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Abstract #2558

Preliminary study of a Lafora Disease mouse model using glycoNOE MRI

Chongxue Bie1,2,3, Yang Zhou4, Peter C. M. van Zijl1,2, Jiadi Xu1,2, Ramon C. Sun5, Matthew S. Gentry66, and Nirbhay N. Yadav1,2
1The Russell H. Morgan Department of Radiology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States, 2F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, United States, 3Department of Information Science and Technology, Northwest University, Xi'an, China, 4Institute of Biomedical and Health Engineering, Shenzhen Institutes of Advanced Technology, Chinese Academy of Sciences, Shenzhen, China, 5Department of Neuroscience, University of Kentucky, Lexington, KY, United States, 6Department of Molecular and Cellular Biochemistry, University of Kentucky, Lexington, KY, United States

Lafora disease (LD) is a glycogen storage disease marked by an intracellular accumulation of starch-like polyglucosan “Lafora bodies” (LBs) in brain and other tissues. There are no non-invasive approaches to quickly image brain glycogen and biopsies are difficult. We evaluate the feasibility of glycoNOE MRI to detect the accumulation of LBs in a laforin-deficient (Epm2a-/-) LD mouse model for this disease. Results suggest that the distribution of LBs in the brain can be mapped using glycoNOE MRI showing potential for studying this disease and its treatment non-invasively in vivo.

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