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

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) mH₂ O than controls. Principal components analyses of polar, ceramide, a

SUBMITTER: Le SQ 

PROVIDER: S-EPMC7992919 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature