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Sj\u00f6gren\u2010Larsson syndrome (SLS) is one such disorder, which is caused by mutations in the fatty aldehyde dehydrogenase\n                    <jats:italic>ALDH3A2<\/jats:italic>\n                    . To date, the molecular mechanism underlying SLS pathology has remained unknown. In this study, we found that\n                    <jats:italic>Aldh3a2<\/jats:italic>\n                    is expressed in oligodendrocytes and neurons in the mouse brain, and neurons of\n                    <jats:italic>Aldh3a2<\/jats:italic>\n                    knockout (KO) mice exhibited impaired metabolism of the long\u2010chain base, a component of sphingolipids.\n                    <jats:italic>Aldh3a2<\/jats:italic>\n                    KO mice showed several abnormalities corresponding to SLS symptoms in behavioral tests, including increased paw slips on a balance beam and light\u2010induced anxiety. In their brain tissue, 2\u2010hydroxygalactosylceramide, an important lipid for myelin function and maintenance, was reduced by the inactivation of fatty acid 2\u2010hydroxylase. Our findings provide important new insights into the molecular mechanisms responsible for neural pathogenesis caused by lipid metabolism abnormalities.\u2014Kanetake, T., Sassa, T., Nojiri, K., Sawai, M., Hattori, S., Miyakawa, T., Kitamura, T., Kihara, A. Neural symptoms in a gene knockout mouse model of Sj\u00f6gren\u2010Larsson syndrome are associated with a decrease in 2\u2010hydroxygalactosylceramide. FASEB J. 33, 928\u2013941 (2019).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.201800291r","type":"journal-article","created":{"date-parts":[[2018,8,7]],"date-time":"2018-08-07T15:29:32Z","timestamp":1533655772000},"page":"928-941","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Neural symptoms in a gene knockout mouse model of Sj\u00f6gren\u2010Larsson syndrome are associated with a decrease in 2\u2010hydroxygalactosylceramide"],"prefix":"10.1096","volume":"33","author":[{"given":"Tsukasa","family":"Kanetake","sequence":"first","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Sassa","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koki","family":"Nojiri","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megumi","family":"Sawai","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoko","family":"Hattori","sequence":"additional","affiliation":[{"name":"Division of Systems Medical Science Institute for Comprehensive Medical Science Fujita Health University  Toyoake Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuyoshi","family":"Miyakawa","sequence":"additional","affiliation":[{"name":"Division of Systems Medical Science Institute for Comprehensive Medical Science Fujita Health University  Toyoake Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuya","family":"Kitamura","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akio","family":"Kihara","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry Faculty of Pharmaceutical Sciences Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4757-1830-0_5"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2014.12.016"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80093-8"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0458-08.2008"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.21172"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0196-52"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20181"},{"key":"e_1_2_8_9_1","first-page":"1","article-title":"Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study","volume":"113","author":"Sj\u00f6gren T.","year":"1957","journal-title":"Acta Psychiatr. 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