Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells.

摘要: Cytotoxic capacity of psychosine (galactosylsphingosine) was evaluated in comparison with C6-ceramide cultured fibroblasts and the glia-derived MOCH-1 cells that have characteristics myelinating (1). Psychosine caused cytotoxic cell death DNA fragmentation at concentrations similar to were substantially more sensitive their effects than fibroblasts. In this system, pretreatment GM1-ganglioside failed protect from deleterious these compounds. These findings are consistent hypothesis is metabolite causes apoptotic oligodendrocyte globoid leukodystrophy (Krabbe disease). They further suggest protective unlikely be explanation for paradoxical improvement phenotype mouse simultaneously deficient two lysosomal β-galactosidases, galactosylceramidase β-galactosidase.