title: (1) H magnetic resonance spectroscopy of neurodegeneration in a mouse model of niemann-pick type C1 disease.
abstract: OBJECTIVE
  To evaluate brain metabolite levels as in vivo indicators of disease progression in a widely studied mouse model of Niemann-Pick type C1 (NPC1) disease with quantitative (1) H magnetic resonance spectroscopy (MRS).
  
  
  METHODS
  Single voxel MRS experiments were carried out in vivo in a mouse model of NPC1 disease and in control mice in two brain regions (central and posterior) at two timepoints (presymptomatic and endstage) to examine changes in metabolite levels in NPC1 disease. Concentrations of nine metabolites were quantified by fitting a simulated basis set of metabolite signals to the acquired spectra.
  
  
  RESULTS
  The only differences found in brain metabolite levels between NPC1 disease model and control mice were increased myo-inositol and decreased taurine in the posterior region of the brain at the endstage of the disease. Metabolite changes reported in past clinical MRS studies of NPC disease were not found in the current study of the mouse model.
  
  
  CONCLUSIONS
  The (1) H spectra obtained from NPC1 mice and control mice were very similar, even at endstages of the disease. Although differences in two metabolites associated with neurodegenerative diseases were found and could inform future studies of the disease model, it appears that MRS in this mouse model of NPC1 disease does not have the sensitivity desired for a biomarker.
authors.affiliation: Biomedical Engineering, University of Arizona, Tucson, AZ 85721, USA.
authors.forename: John W
authors.initials: JW
authors.lastname: Totenhagen
authors.forename: Eriko S
authors.initials: ES
authors.lastname: Yoshimaru
authors.forename: Robert P
authors.initials: RP
authors.lastname: Erickson
authors.forename: Theodore P
authors.initials: TP
authors.lastname: Trouard
mesh_terms: D000818:Animals; D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D001921:Brain / Q000378:metabolism*; D002794:Choline / Q000032:analysis*; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D051379:Mice; D008807:Mice, Inbred BALB C; D008822:Mice, Transgenic; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000378:metabolism*; D052556:Niemann-Pick Disease, Type C / Q000175:diagnosis / Q000378:metabolism*; D011522:Protons; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D014018:Tissue Distribution
chemical_list: D015415:Biomarkers; D011522:Protons; D001224:Aspartic Acid; C000179:N-acetylaspartate; D002794:Choline