{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T22:20:47Z","timestamp":1777414847129,"version":"3.51.4"},"reference-count":28,"publisher":"Institute of Physiology of the Czech Academy of Sciences","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Physiol Res"],"published-print":{"date-parts":[[2016,2,28]]},"abstract":"<jats:p>Huntington\u2019s 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 1H 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.<\/jats:p>","DOI":"10.33549\/physiolres.932967","type":"journal-article","created":{"date-parts":[[2020,2,12]],"date-time":"2020-02-12T12:50:08Z","timestamp":1581511808000},"page":"155-163","source":"Crossref","is-referenced-by-count":8,"title":["Minipig Model of Huntington\u00b4s Disease: 1H Magnetic Resonance Spectroscopy of the Brain"],"prefix":"10.33549","author":[{"given":"M.","family":"JOZEFOVICOVA","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"HERYNEK","sequence":"additional","affiliation":[{"name":"Magnetic Resonance Unit, Department of Diagnostic and Interventional Radiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"JIRU","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"DEZORTOVA","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"JUHASOVA","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"JUHAS","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"MOTLIK","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"HAJEK","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"19169","published-online":{"date-parts":[[2016,2,14]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","unstructured":"BANO D, ZANETTI F, MENDE Y, NICOTERA P: Neurodegenerative processes in Huntington\u2019s disease. Cell Death Dis 2: e228, 2011.","DOI":"10.1038\/cddis.2011.112"},{"key":"ref1","doi-asserted-by":"publisher","unstructured":"BAXA M, HRUSKA-PLOCHAN M, JUHAS S, VODICKA P, PAVLOK A: A transgenic minipig model of Huntington\u2019s disease. J Huntingtons Dis 2: 47-68, 2013.","DOI":"10.3233\/jhd-130001"},{"key":"ref2","doi-asserted-by":"publisher","unstructured":"BENOVA I, KUPCOVA SKALNIKOVA H, KLIMA J, JUHAS S, MOTLIK J: C03 Activation of cytokine production in F1 and F2 generation of miniature pigs transgenic for N-terminal part of mutated human huntingtin. J Neurol Neurosurg Psychiatry 83 (Suppl 1): A16-A16, 2012.","DOI":"10.1136\/jnnp-2012-303524.50"},{"key":"ref3","doi-asserted-by":"publisher","unstructured":"BOHANNA I, GEORGIOU-KARISTIANIS N, HANNAN AJ, EGAN GF: Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington\u2019s disease. Brain Res Rev 58: 209-225, 2008.","DOI":"10.1016\/j.brainresrev.2008.04.001"},{"key":"ref4","unstructured":"CASTILLO M, SMITH JK, KWOCK L: Correlation of myo-inositol levels and grading of cerebral astrocytomas. AJNR Am J Neuroradiol 21: 1645-1649, 2000."},{"key":"ref5","doi-asserted-by":"publisher","unstructured":"G\u00d3MEZ-ANS\u00d3N B, ALEGRET M, MU\u00d1OZ E, SAINZ A, MONTE GC, TOLOSA E: Decreased frontal choline and neuropsychological performance in preclinical Huntington disease. Neurology 68: 906-910, 2007.","DOI":"10.1212\/01.wnl.0000257090.01107.2f"},{"key":"ref6","doi-asserted-by":"publisher","unstructured":"GOVINDARAJU V, YOUNG K, MAUDSLEY AA: Proton NMR chemical shifts and coupling constants for brain metabolites. NMR Biomed 13: 129-153, 2000.","DOI":"10.1002\/1099-1492(200005)13:3<129::aid-nbm619>3.0.co;2-v"},{"key":"ref7","doi-asserted-by":"publisher","unstructured":"HEIKKINEN T, LEHTIM\u00c4KI K, VARTIAINEN N, PUOLIV\u00c4LI J, HENDRICKS SJ, GLASER JR, BRADAIA A, WADEL K, TOULLER C, KONTKANEN O, YRJ\u00c4NHEIKKI JM, BUISSON B, HOWLAND D, BEAUMONT V, MUNOZ-SANJUAN I, PARK LC: Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington\u2019s disease. PLoS One 7: e50717, 2012.","DOI":"10.1371\/journal.pone.0050717"},{"key":"ref8","doi-asserted-by":"publisher","unstructured":"HENRY P-G, MOCHEL F: The search for sensitive biomarkers in presymptomatic Huntington disease. J Cereb Blood Flow Metab 32: 769-770, 2012.","DOI":"10.1038\/jcbfm.2012.17"},{"key":"ref9","doi-asserted-by":"publisher","unstructured":"JENKINS BG, ANDREASSEN OA, DEDEOGLU A, LEAVITT B, HAYDEN M, BORCHELT D, ROSS CA, FERRANTE RJ, BEAL MF: Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington\u2019s disease. J Neurochem 95: 553-562, 2005.","DOI":"10.1111\/j.1471-4159.2005.03411.x"},{"key":"ref10","doi-asserted-by":"publisher","unstructured":"JIRU F, SKOCH A, WAGNEROVA D, DEZORTOVA M, HAJEK M: jSIPRO - analysis tool for magnetic resonance spectroscopic imaging. Comput Methods Programs Biomed 112: 173-188, 2013.","DOI":"10.1016\/j.cmpb.2013.06.018"},{"key":"ref11","doi-asserted-by":"publisher","unstructured":"MACAKOVA M, HANSIKOVA H, ANTONIN P, HAJKOVA Z, SADKOVA J, JUHA S, JUHASOVA J, BAXA M, ZEMAN J, MOTLIK J: C04 Reproductive parameters and mitochondrial function in spermatozoa of F1 and F2 minipig boars transgenic for n-terminal part of the human mutated huntingtin. J Neurol Neurosurg Psychiatry 83 (Suppl 1): A16-A16, 2012.","DOI":"10.1136\/jnnp-2012-303524.51"},{"key":"ref12","doi-asserted-by":"crossref","unstructured":"MORTON AJ, HOWLAND DS: Large genetic animal models of Huntington\u2019s disease. J Huntingtons Dis 2: 3-19, 2013.","DOI":"10.3233\/JHD-130050"},{"key":"ref13","doi-asserted-by":"crossref","unstructured":"PEREZ-DE LA CRUZ V, SANTAMARIA A: Integrative hypothesis for Huntington\u2019s disease: a brief review of experimental evidence. Physiol Res 56: 513-526, 2007.","DOI":"10.33549\/physiolres.931049"},{"key":"ref14","doi-asserted-by":"publisher","unstructured":"POUWELS PJ, KRUSE B, KORENKE GC, MAO X, HANEFELD FA, FRAHM J: Quantitative proton magnetic resonance spectroscopy of childhood adrenoleukodystrophy. Neuropediatrics 29: 254-264, 1998.","DOI":"10.1055\/s-2007-973571"},{"key":"ref15","doi-asserted-by":"publisher","unstructured":"PROVENCHER SW: Estimation of metabolite concentrations from localized in vivo proton NMR spectra. Magn Reson Med 30: 672-679, 1993.","DOI":"10.1002\/mrm.1910300604"},{"key":"ref16","doi-asserted-by":"publisher","unstructured":"REYNOLDS NC, PROST RW, MARK LP: Heterogeneity in 1H-MRS profiles of presymptomatic and early manifest Huntington\u2019s disease. Brain Res 1031: 82-89, 2005.","DOI":"10.1016\/j.brainres.2004.10.030"},{"key":"ref17","doi-asserted-by":"publisher","unstructured":"ROSAS HD, TUCH DS, HEVELONE ND, ZALETA AK, VANGEL M, HERSCH SM, SALAT DH: Diffusion tensor imaging in presymptomatic and early Huntington\u2019s disease: selective white matter pathology and its relationship to clinical measures. Mov Disord 21: 1317-1325, 2006.","DOI":"10.1002\/mds.20979"},{"key":"ref18","doi-asserted-by":"publisher","unstructured":"ROTONDO E, BRUSCHETTA G, SACC\u00c0 A, BRAMANTI P, DI PASQUALE MR: Straightforward relative quantitation and age-related human standards of N-acetylaspartate at the centrum semiovale level by CSI 1H-MRS. Magn Reson Imaging 21: 1055-1060, 2003.","DOI":"10.1016\/s0730-725x(03)00211-x"},{"key":"ref19","doi-asserted-by":"publisher","unstructured":"STURROCK A, LAULE C, DECOLONGON J, DAR SANTOS R, COLEMAN AJ, CREIGHTON S, BECHTEL N, REILMANN R, HAYDEN MR, TABRIZI SJ, MACKAY AL, LEAVITT BR: Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease. Neurology 75: 1702-1710, 2010.","DOI":"10.1212\/wnl.0b013e3181fc27e4"},{"key":"ref20","doi-asserted-by":"publisher","unstructured":"SUNDGREN PC, JENNINGS J, ATTWOOD JT, NAN B, GEBARSKI S, MCCUNE WJ, PANG Y, MALY P: MRI and 2D-CSI MR spectroscopy of the brain in the evaluation of patients with acute onset of neuropsychiatric systemic lupus erythematosus. Neuroradiology 47: 576-585, 2005.","DOI":"10.1007\/s00234-005-1371-y"},{"key":"ref21","doi-asserted-by":"publisher","unstructured":"TKAC I, HENRY P-G, ZACHAROFF L, WEDEL M, GONG W, DEELCHAND DK, LI T, DUBINSKY JM: Homeostatic adaptations in brain energy metabolism in mouse models of Huntington disease. J Cereb Blood Flow Metab 32: 1977-1988, 2012.","DOI":"10.1038\/jcbfm.2012.104"},{"key":"ref22","doi-asserted-by":"publisher","unstructured":"UNSCHULD PG, EDDEN RA, CARASS A, LIU X, SHANAHAN M, WANG X, OISHI K, BRANDT J, BASSETT SS, REDGRAVE GW, MARGOLIS RL, VAN ZIJL PC, BARKER PB, ROSS CA: Brain metabolite alterations and cognitive dysfunction in early Huntington\u2019s disease. Mov Disord 27: 895-902, 2012.","DOI":"10.1002\/mds.25010"},{"key":"ref23","doi-asserted-by":"publisher","unstructured":"VAN DEN BOGAARD SJA, DUMAS EM, TEEUWISSE WM, KAN HE, WEBB A, ROOS RAC, VAN DER GROND J: Exploratory 7-Tesla magnetic resonance spectroscopy in Huntington\u2019s disease provides in vivo evidence for impaired energy metabolism. J Neurol 258: 2230-2239, 2011.","DOI":"10.1007\/s00415-011-6099-5"},{"key":"ref24","doi-asserted-by":"publisher","unstructured":"VAN OOSTROM JC, SIJENS PE, ROOS RAC, LEENDERS KL: 1H magnetic resonance spectroscopy in preclinical Huntington disease. Brain Res 1168: 67-71, 2007.","DOI":"10.1016\/j.brainres.2007.05.082"},{"key":"ref25","doi-asserted-by":"publisher","unstructured":"WEAVER KE, RICHARDS TL, LIANG O, LAURINO MY, SAMII A, AYLWARD EH: Longitudinal diffusion tensor imaging in Huntington\u2019s Disease. Exp Neurol 216: 525-529, 2009.","DOI":"10.1016\/j.expneurol.2008.12.026"},{"key":"ref26","doi-asserted-by":"publisher","unstructured":"ZACHAROFF L, TKAC I, SONG Q, TANG C, BOLAN PJ, MANGIA S, HENRY P-G, LI T, DUBINSKY JM: Cortical metabolites as biomarkers in the R6\/2 model of Huntington\u2019s disease. J Cereb Blood Flow Metab 32: 502-514, 2012.","DOI":"10.1038\/jcbfm.2011.157"},{"key":"ref27","doi-asserted-by":"publisher","unstructured":"ZUCCATO C, VALENZA M, CATTANEO E: Molecular mechanisms and potential therapeutical targets in Huntington\u2019s disease. Physiol Rev 90: 905-981, 2010.","DOI":"10.1152\/physrev.00041.2009"}],"container-title":["Physiological Research"],"original-title":[],"language":"en","deposited":{"date-parts":[[2022,10,15]],"date-time":"2022-10-15T10:13:34Z","timestamp":1665828814000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.biomed.cas.cz\/physiolres\/pdf\/65\/65_155.pdf"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,2,14]]},"references-count":28,"URL":"https:\/\/doi.org\/10.33549\/physiolres.932967","relation":{},"ISSN":["1802-9973","0862-8408"],"issn-type":[{"value":"1802-9973","type":"electronic"},{"value":"0862-8408","type":"print"}],"subject":[],"published":{"date-parts":[[2016,2,14]]}}}