Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice

Steven Q. Le, Igor Nestrasil, Shih hsin Kan, Martin Egeland, Jonathan D. Cooper, David Elashoff, Rong Guo, Jakub Tolar, Jennifer K. Yee, Patricia I. Dickson

Research output: Contribution to journalArticle

Abstract

Mucopolysaccharidosis type I (MPS I) is a lysosomal disease with progressive central nervous system involvement. 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 week-old, male MPS I mice and littermate, heterozygote controls (n = 12 per group). Male MPS I mice showed lower phosphatidylcholine and ether-linked phosphatidylcholine quantities than controls (p < 0.05). Twenty-two 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) mH2O 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 versus heterozygous controls. There was a trend toward lower proteolipid protein-1 levels in MPS I mice (p = 0.06). MPS I mice show subtle changes in white matter composition, with an unknown impact on pathogenesis in this model.

Original languageEnglish
JournalLipids
DOIs
StateAccepted/In press - Jan 1 2020

Keywords

  • Glycosaminoglycans
  • Hurler syndrome
  • Inborn errors of metabolism
  • Lipidomics
  • Lysosomal storage disease

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    Le, S. Q., Nestrasil, I., Kan, S. H., Egeland, M., Cooper, J. D., Elashoff, D., Guo, R., Tolar, J., Yee, J. K., & Dickson, P. I. (Accepted/In press). Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice. Lipids. https://doi.org/10.1002/lipd.12261