Galactosylsphingosine (d18:1) (Synonyms: Galactosylsphingosine, Psychosine) |
| Catalog No.GC43723 |
La galactosilesfingosina (d18:1) (galactosilesfingosina), un sustrato de la enzima galactocerebrosidasa (GALC), es un biomarcador potencial de la enfermedad de Krabbe.
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Cas No.: 2238-90-6
Sample solution is provided at 25 µL, 10mM.
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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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