半乳糖基鞘氨醇 (d18:1) (Galactosylsphingosine) 是半乳糖脑苷脂酶 (GALC) 酶的底物，是 Krabbe 病的潜在生物标志物。
| Cas No. | 2238-90-6 |
| 别名 | 神经鞘氨醇半乳糖苷,Galactosylsphingosine |
| Canonical SMILES | O[C@@H]1[C@@H](O)[C@@H](O)[C@@H](CO)O[C@H]1OC[C@@H]([C@H](O)/C=C/CCCCCCCCCCCCC)N |
| 分子式 | C24H47NO7 |
| 分子量 | 461.6 |
| 溶解度 | Ethanol: >1 mg/ml; PBS (pH 7.2): >1 mg/ml |
| 储存条件 | Store at -20°C, protect from light |
| General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
| Shipping Condition | Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request |
| 产品描述 | 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. |
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