CAS NO: | 2238-90-6 |
规格: | 98% |
分子量: | 461.6 |
包装 | 价格(元) |
1mg | 电议 |
5mg | 电议 |
10mg | 电议 |
Background:
Galactosylsphingosine is a galactoside of sphingosine and a constituent of cerebrosides. The lysosomal enzyme β-galactosylceramidase is responsible for cleaving the glycosydic linkage of galactosylceramide and galactosylsphingosine. Accumulation of galactosylsphingosine during β-galactosylceramidase deficiency, as seen with Krabbe disease, is cytotoxic and preferentially affects oligodendrocytes, leading to progressive demyelination and infiltration of activated macrophages into the brain.