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Non\u2010invasive imaging markers to detect early disease in <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>1 will facilitate testing and implementation of potential therapies. We have previously demonstrated the sensitivity of neurochemical levels measured by <jats:sup>1<\/jats:sup>H magnetic resonance spectroscopy (<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content>) to progressive neurodegeneration using a transgenic mouse model of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>1. In order to investigate very early neurochemical changes related to neurodegeneration, here we utilized a knock\u2010in mouse model, the <jats:italic>Sca1<\/jats:italic><jats:sup><jats:italic>154Q\/2Q<\/jats:italic><\/jats:sup> line, which displays milder cerebellar pathology than the transgenic model. We measured cerebellar neurochemical profiles of <jats:italic>Sca1<\/jats:italic><jats:sup><jats:italic>154Q\/2Q<\/jats:italic><\/jats:sup> mice and wild\u2010type littermates using 9.4T <jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> at ages 6, 12, 24, and 39\u00a0weeks and assessed the cerebellar pathology of a subset of the mice at each time point. The <jats:italic>Sca1<\/jats:italic><jats:sup><jats:italic>154Q\/2Q<\/jats:italic><\/jats:sup> mice displayed very mild cerebellar pathology even at 39\u00a0weeks, however, were distinguished from wild types by <jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> starting at 6\u00a0weeks. Taurine and total choline levels were significantly lower at all ages and glutamine and total creatine levels were higher starting at 12\u00a0weeks in <jats:italic>Sca1<\/jats:italic><jats:sup><jats:italic>154Q\/2Q<\/jats:italic><\/jats:sup> mice than controls, demonstrating the sensitivity of neurochemical levels to neurodegeneration related changes in the absence of overt pathology.\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc12435-fig-0007-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><jats:p>We measured cerebellar neurochemical alterations in a knock\u2010in mouse model of spinocerebellar ataxia type 1, a hereditary movement disorder, using ultra\u2010high field magnetic resonance spectroscopy (MRS). Very early neurochemical alterations were detectable prior to overt pathology in the volume\u2010of\u2010interest for MRS. Alterations were indicative of osmolytic changes and of disturbances in membrane phospholipid and energy metabolism.<\/jats:p>","DOI":"10.1111\/jnc.12435","type":"journal-article","created":{"date-parts":[[2013,8,27]],"date-time":"2013-08-27T13:48:56Z","timestamp":1377611336000},"page":"660-668","source":"Crossref","is-referenced-by-count":27,"title":["Non\u2010invasive detection of neurochemical changes prior to overt pathology in a mouse model of spinocerebellar ataxia type 1"],"prefix":"10.1111","volume":"127","author":[{"given":"Uzay E.","family":"Emir","sequence":"first","affiliation":[{"name":"Department of Radiology Medical School Center for Magnetic Resonance Research University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Howard","family":"Brent Clark","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Medical School University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manda L.","family":"Vollmers","sequence":"additional","affiliation":[{"name":"Department of Radiology Medical School Center for Magnetic Resonance Research University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn E.","family":"Eberly","sequence":"additional","affiliation":[{"name":"Division of Biostatistics School of Public Health University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fclin","family":"\u00d6z","sequence":"additional","affiliation":[{"name":"Department of Radiology Medical School Center for Magnetic Resonance Research University of Minnesota Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,9,17]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90273-2"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.17-19-07385.1997"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0069782"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870130510"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmre.2001.2340"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1522-2594(200002)43:2<319::AID-MRM22>3.0.CO;2-1"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.1910030602"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e32834875ba"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23559"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.10135"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1095-6433(99)00160-9"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1021\/cr900250y"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00250262"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-94-007-1333-8_29"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23067"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.5612-09.2010"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2011.09.021"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0730-725X(95)02033-P"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.1910300604"},{"key":"e_1_2_9_21_1","volume-title":"LCModel & LCMgui User's Manual","author":"Provencher S. 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