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.
Keywords