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Recently, a de novo mutation in the\n                    <jats:italic>ELOVL1<\/jats:italic>\n                    gene, which encodes fatty acid elongase, was identified in patients with neurocutaneous disorders involving skin ichthyosis and multiple neurological abnormalities, including hypomyelination, spastic paraplegia, and high\u2010frequency deafness. However, the consequences of\n                    <jats:italic>ELOVL1<\/jats:italic>\n                    deficiency for lipid composition and detailed pathological changes in the brain remain unclear. Here, we analyzed\n                    <jats:italic>Elovl1<\/jats:italic>\n                    mutant mice as a model of human\n                    <jats:italic>ELOVL1<\/jats:italic>\n                    deficiency. The mice exhibited a decreased postnatal survival rate, and some died of startle epilepsy. The acyl chain length of sphingolipids such as galactosylceramides, sulfatides, sphingomyelins, and ceramides in the brains of these mice was markedly shortened. Moreover, the mice exhibited reduced levels of galactosylceramides, which are important for myelin formation and stability. Electron microscope analysis of the corpus callosum in\n                    <jats:italic>Elovl1<\/jats:italic>\n                    mutant mice revealed modest hypomyelination, especially in large\u2010diameter axons. Furthermore, behavioral testing of the mice revealed deficits such as poorer motor coordination and reduced acoustic startle response to high\u2010intensity stimulus. These findings provide clues to the molecular mechanism of the neurological symptoms of patients with the\n                    <jats:italic>ELOVL1<\/jats:italic>\n                    mutation.\n                  <\/jats:p>","DOI":"10.1096\/fba.2019-00067","type":"journal-article","created":{"date-parts":[[2019,11,1]],"date-time":"2019-11-01T15:09:32Z","timestamp":1572620972000},"page":"747-759","source":"Crossref","is-referenced-by-count":27,"title":["Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase\n                    <i>Elovl1<\/i>"],"prefix":"10.1096","volume":"1","author":[{"given":"Masashi","family":"Isokawa","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":"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":"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":[[2019,11,22]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2011.06.004"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2016.04.002"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4757-1830-0_5"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2014.12.016"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.23.13280"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80093-8"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/jb\/mvs105"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.4062\/biomolther.2014.017"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(65)90036-6"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)39911-8"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.plipres.2006.01.004"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1005572107"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.201700947R"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00192-13"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.plipres.2016.04.001"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2018.09.010"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2017-105172"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2018-105711"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.201800291R"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms14610"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.90.21.10265"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.031971"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20050291"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M406649200"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0458-08.2008"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21205"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00418-007-0344-0"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(01)80046-5"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.151.1.143"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M022715"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M116.714030"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2006.08.006"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0903279106"}],"container-title":["FASEB 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