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Abstract #1039

Morphologic Abnormalities of Mucopolysaccharidosis Type VII Characterized by High Resolution MRI in a Mouse Model

Ilya Michael Nasrallah1, Sungheon Kim2, Ranjit Ittyerah1, Stephen Pickup1, John H. Wolfe3,4, Harish Poptani1

1Department of Radiology, University of Pennsylvania, Philadelphia, PA, United States; 2New York University; 3Departments of Pathobiology and Pediatrics, University of Pennsylvania; 4Children's Hospital of Philadelphia

Mucopolysaccharidosis Type VII (MPS VII) is one of the degenerative lysosomal storage diseases characterized by intracellular vacuolization. Using high-resolution MRI in a mouse model of MPS VII with manual segmentation, we identify decreases in corpus callosum and anterior commisure volume and slight increase in hippocampus volume in mutant mice. A decrease in corpus callosum volume thickness is confirmed at histology. These parameters could be used for monitoring experimental response to gene therapy treatments.