跳转至内容
Merck
CN
  • Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice.

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

Lipids (2020-06-17)
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, 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
摘要

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, 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.

材料
产品编号
品牌
产品描述

Sigma-Aldrich
3,3′-二氨基联苯胺 四盐酸盐, tablet, 10 mg substrate per tablet
Sigma-Aldrich
抗-髓磷脂碱性蛋白抗体,a.a.82-87, culture supernatant, clone 12, Chemicon®
Sigma-Aldrich
4-硝基苯基磷酸盐 二钠盐 六水合物, tablet
Sigma-Aldrich
硝基苯磷酸盐液体底物系统, liquid
Sigma-Aldrich
DL-甘油醛-3-磷酸 溶液, 45-55 mg/mL in H2O
Sigma-Aldrich
MBP(髓磷脂碱性蛋白),牛,纯化
Sigma-Aldrich
p-Xylene-bis(N-pyridinium bromide), ≥95% (TLC)
Sigma-Aldrich
Anti-GAPDH antibody produced in chicken, affinity isolated antibody, buffered aqueous solution