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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