Assessment of metabolic abnormalities from the cerebellar region of the brain of a canine model of mucopolysaccharidosis type I (MPS I) using in vivo 1H MRS
Manoj Kumar 1 , Jessica Bagel 2 , Patricia ODonnell 3 , Katherine P Ponder 4 , James M Wilson 5 , Mark Haskins 3 , Charles H Vite 2 , and Harish Poptani 1
Radiology, University of Pennsylvania,
Philadelphia, PA, United States,
Studies, University of Pennsylvania, Philadelphia, PA,
Pathobiology, University of
Pennsylvania, Philadelphia, PA, United States,
Medicine, Washington University School of Medicine, St.
Louis MO, United States,
and Laboratory Medicine, University of Pennsylvania,
Philadelphia, PA, United States
In vivo 1H MRS was performed on MPS I (n=8) and normal
dogs (n=4) from the cerebellar regions of the brain to
assess metabolic changes. LC-model was used to measure
concentration of the major brain metabolites [N-acetylaspartate
(NAA), creatine (Cr), choline (Cho), and myo-inositol (mI)].
NAA, Cho, and mI peaks values were normalized to Cr peak
values. We observed significantly reduced NAA/Cr and
significantly increased Cho/Cr in MPS I along with
slightly elevated mI/Cr reflecting increased
demyelination, gliosis, and axonal injury. Our studies
indicate that 1H MRS might be a sensitive technique for
detecting and quantifying the neuroaxonal injury status
in this model.
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