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Abstract #2986

Demyelination of the corpus callosum in a mouse model of mucopolysaccharidosis type I

Ivan Tkac1, Igor Nestrasil2, Steven Q Le3, Jakub Tolar4, and Patricia I Dickson3

1Center for Magnetic Resonance Research, University of Minnesota, Minneapolis, MN, United States, 2Departmant of Pediatrics, University of Minnesota, Minneapolis, MN, United States, 3Department of Pediatrics, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, CA, United States, 4Pediatric Bone and Marrow Transplantation, University of Minnesota, Minneapolis, MN, United States

Mucopolysaccharidosis type I (MPS I) is an autosomal lysosomal storage disease caused by deficiency of α-L-iduronidase enzyme, which results in glycosaminoglycans accumulation within the lysosomes. MPS I leads to progressive loss of cognitive function and substantial physical disease in children. Abnormal myelin composition and reduced expression of myelin-related genes has been recently reported in a canine model of MPS I. The purpose of this study was to demonstrate whether demyelination of the corpus callosum can be detected also in a mouse model of MPS I using in vivo 1H MRS at 9.4 T.

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