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This study examined the lipid, cholesterol, and myelin basic protein composition of white matter in the corpus callosum of MPS I mice. We studied 50\u2009week\u2010old, male MPS I mice and littermate, heterozygote controls (<jats:italic>n<\/jats:italic> = 12 per group). Male MPS I mice showed lower phosphatidylcholine and ether\u2010linked phosphatidylcholine quantities than controls (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Twenty\u2010two phospholipid or ceramide species showed significant differences in percent of total. Regarding specific lipid species, MPS I mice exhibited lower quantities of sphingomyelin 18:1, phosphatidylserine 38:3, and hexosylceramide d18:1(22:1) mH<jats:sub>2<\/jats:sub>O than controls. Principal components analyses of polar, ceramide, and hexosylceramide lipids, respectively, showed some separation of MPS I and control mice. We found no significant differences in myelin gene expression, myelin basic protein, or total cholesterol in the MPS I mice <jats:italic>versus<\/jats:italic> heterozygous controls. There was a trend toward lower proteolipid protein\u20101 levels in MPS I mice (<jats:italic>p<\/jats:italic> = 0.06). MPS I mice show subtle changes in white matter composition, with an unknown impact on pathogenesis in this model.<\/jats:p>","DOI":"10.1002\/lipd.12261","type":"journal-article","created":{"date-parts":[[2020,6,15]],"date-time":"2020-06-15T01:15:45Z","timestamp":1592183745000},"page":"627-637","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice"],"prefix":"10.1002","volume":"55","author":[{"given":"Steven Q.","family":"Le","sequence":"first","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"},{"name":"Department of Pediatrics Washington University School of Medicine  660 S. Euclid Saint Louis MO 63110 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Igor","family":"Nestrasil","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Minnesota  2450 Riverside Avenue Minneapolis MN 55454 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shih\u2010hsin","family":"Kan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"},{"name":"CHOC Children's Research Institute  2450 Riverside Avenue Orange CA 55454 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Egeland","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"},{"name":"Department of Pediatrics Washington University School of Medicine  660 S. Euclid Saint Louis MO 63110 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Elashoff","sequence":"additional","affiliation":[{"name":"Department of Medicine Statistics Core University of California  100 Medical Plaza Driveway Los Angeles CA 90095 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rong","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Medicine Statistics Core University of California  100 Medical Plaza Driveway Los Angeles CA 90095 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakub","family":"Tolar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Minnesota  2450 Riverside Avenue Minneapolis MN 55454 USA"},{"name":"Stem Cell Institute University of Minnesota  2001 6th Street SE Minneapolis MN 55455 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer K.","family":"Yee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia I.","family":"Dickson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The Lundquist Institute for Biomedical Innovation at Harbor\u2010UCLA Medical Center  1124 W. Carson Street Torrance CA 90502 USA"},{"name":"Department of Pediatrics Washington University School of Medicine  660 S. 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