myelin-basic-protein and Mucopolysaccharidosis-I

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) mH

Topics: Animals; Case-Control Studies; Cholesterol; Corpus Callosum; Female; Gene Expression; Lipids; Male; Mice, Inbred C57BL; Mice, Knockout; Mucopolysaccharidosis I; Myelin Basic Protein; Myelin Sheath