title: Minipig model of Huntington's disease: ¹H magnetic resonance spectroscopy of the brain.
abstract: Huntington's disease (HD) is an inherited autosomal neurodegenerative disorder affecting predominantly the brain, characterized by motor dysfunctions, behavioral and cognitive disturbances. The aim of this study was to determine changes in the brain of transgenic minipigs before HD onset using (1)H magnetic resonance (MR) spectroscopy. Measurements were performed on a 3 T MR scanner using a single voxel spectroscopy sequence for spectra acquisition in the white matter and chemical shift imaging sequence for measurement in the striatum, hippocampus and thalamus. A decrease of (phospho)creatine (tCr) concentration was found only in the thalamus (p=0.002) of transgenic minipigs, nevertheless we found significant changes in metabolite ratios. Increase of the ratio choline compounds (tCho)/tCr was found in all examined areas: striatum (p=0.010), thalamus (p=0.011) as well as hippocampus (p=0.027). The ratio N-acetylaspartate+N-acetylaspartylglutamate (tNAA)/tCr (p=0.043) and glutamate+glutamine (Glx)/tCr (p=0.039) was elevated in the thalamus, the ratio myo-inositol (Ins)/tCr (p=0.048) was significantly increased in the hippocampus. No significant differences were observed in the metabolite concentrations in the white matter, however we found significant increase of ratios tNAA/tCr (p=0.018) and tCho/tCr (p=0.003) ratios in transgenic boars. We suppose that the majority of the observed changes are predominantly related to changes in energy metabolism caused by decrease of tCr.
authors.affiliation: Magnetic Resonance Unit, Department of Diagnostic and Interventional Radiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic, Department of NMR Spectroscopy and Mass Spectroscopy, Faculty of Chemical and Food Technology, Slovak University of Technology, Bratislava, Slovak Republic. vit.herynek@medicon.cz.
authors.forename: M
authors.initials: M
authors.lastname: Jozefovicova
authors.forename: V
authors.initials: V
authors.lastname: Herynek
authors.forename: F
authors.initials: F
authors.lastname: Jiru
authors.forename: M
authors.initials: M
authors.lastname: Dezortova
authors.forename: J
authors.initials: J
authors.lastname: Juhasova
authors.forename: S
authors.initials: S
authors.lastname: Juhas
authors.forename: J
authors.initials: J
authors.lastname: Motlik
authors.forename: M
authors.initials: M
authors.lastname: Hajek
mesh_terms: D000818:Animals; D030801:Animals, Genetically Modified; D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism*; D004195:Disease Models, Animal; D006816:Huntington Disease / Q000000981:diagnostic imaging* / Q000378:metabolism*; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D011522:Protons; D013552:Swine; D013556:Swine, Miniature
chemical_list: D011522:Protons