{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T06:12:23Z","timestamp":1789020743337,"version":"build-2803163510"},"reference-count":32,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2005,10,3]],"date-time":"2005-10-03T00:00:00Z","timestamp":1128297600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["NMR in Biomedicine"],"published-print":{"date-parts":[[2005,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Sandhoff disease, one of several related lysosomal storage disorders, results from the build up of <jats:italic>N<\/jats:italic>\u2010acetyl\u2010containing glycosphingolipids in the brain and is caused by mutations in the genes encoding the hexosaminidase \u03b2\u2010subunit. Affected individuals undergo progressive neurodegeneration in response to the glycosphingolipid storage. <jats:sup>1<\/jats:sup>H magnetic resonance spectra of perchloric acid extracts of Sandhoff mouse brain exhibited several resonances ca 2.07\u2009ppm that were not present in the corresponding spectra from extracts of wild\u2010type mouse brain. High\u2010performance liquid chromatography and mass spectrometry of the Sandhoff extracts post\u2010MRS identified the presence of <jats:italic>N<\/jats:italic>\u2010acetylhexosamine\u2010containing oligosaccharides, which are the likely cause of the additional MRS resonances. MRS of intact brain tissue with magic angle spinning also showed additional resonances at ca 2.07\u2009ppm in the Sandhoff case. These resonances appeared to increase with disease progression and probably arise, for the most part, from the stored glycosphingolipids, which are absent in the aqueous extracts. Hence <jats:italic>in vivo<\/jats:italic> MRS may be a useful tool for detecting early\u2010stage Sandhoff disease and response to treatment. Copyright \u00a9 2005 John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/nbm.984","type":"journal-article","created":{"date-parts":[[2005,10,3]],"date-time":"2005-10-03T11:16:37Z","timestamp":1128338197000},"page":"517-526","source":"Crossref","is-referenced-by-count":16,"title":["MRS reveals additional hexose <i>N<\/i>\u2010acetyl resonances in the brain of a mouse model for Sandhoff disease"],"prefix":"10.1002","volume":"18","author":[{"given":"J. P.","family":"Lowe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. J.","family":"Stuckey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. 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