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.

中文翻译：

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粘多糖贮积症 I 型小鼠胼胝体的髓鞘和脂质组成。

I 型粘多糖贮积症 (MPS I) 是一种溶酶体疾病，累及进行性中枢神经系统。本研究检测了 MPS I 小鼠胼胝体白质的脂质、胆固醇和髓鞘碱性蛋白组成。我们研究了 50 周大的雄性 MPS I 小鼠和同窝的杂合子对照（每组n = 12）。雄性 MPS I 小鼠的磷脂酰胆碱和醚连接的磷脂酰胆碱含量低于对照组 ( p  < 0.05)。22 种磷脂或神经酰胺在总量百分比上表现出显着差异。关于特定的脂质种类，MPS I 小鼠表现出较低量的鞘磷脂 18:1、磷脂酰丝氨酸 38:3 和己糖​​神经酰胺 d18:1(22:1) mH ₂O 比控制。分别对极性、神经酰胺和己糖神经酰胺脂质的主成分分析显示 MPS I 和对照小鼠有一些分离。我们发现 MPS I 小鼠与杂合对照小鼠的髓磷脂基因表达、髓磷脂碱性蛋白或总胆固醇没有显着差异。MPS I 小鼠的蛋白脂质蛋白-1 水平有降低的趋势（p = 0.06）。MPS I 小鼠在白质组成方面表现出细微的变化，在该模型中对发病机制的影响未知。