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. Lipids 2020 Read More


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. © 2020 AOCS

Full Text


Posted on June 22, 2020
Posted in: Axon Injury & Repair, Lysosome, NeuroRestorative Therapy, Publications Authors: ,