Neurochemical profiling and volumetric MRI in the murine model of Hurler syndrome (MPS IH)
Igor Nestrasil 1 , Chester B Whitley 1 , Renee Cooksley 2 , Brenda Koniar 3 , Roland Gunther 3 , and Ivan Tkac 4
Department of Pediatrics, University of
Minnesota, Minneapolis, MN, United States,
Therapy Center, University of Minnesota, Minneapolis,
MN, United States,
University of Minnesota, Minneapolis, MN, United States,
for Magnetic Resonance Research, University of
Minnesota, Minneapolis, MN, United States
Hurler syndrome, the severe form of
mucopolysaccharidosis type I (MPS IH) is a lysosomal
storage diseases caused by the deficiency in
alpha-L-iduronidase (IDUA) enzyme which results in
lysosomal accumulation of glycosaminoglycans. Knockout
mice deficient for IDUA were used as a model of MPS IH.
MRS and high-resolution MRI were used to investigate
neurochemical and volumetric changes in MPS mice. The
whole brain volumes and the relative sizes of ventricles
were enlarges in MPS mice relative to controls.
Increased levels of ascorbate (19%) in the hippocampus
of MPS mice indicate a protective response against the
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