Apoptotic positive cells in Krabbe brain and induction of apoptosis in rat C6 glial cells by psychosine.

摘要: Abstract Globoid cell leukodystrophy (GLD) is an autosomal recessive disorder of infants, caused by deficient activity cerebroside-β-galactosidase resulting in loss myelin accompanied oligodendrocytes. The oligodendrocyte population accumulation psychosine, which considered as the molecule responsible for observed pathophysiology GLD. We were able to detect apoptotic cells terminal dUTP nick-end labeling assay and nuclear localization p53 postmortem brain tissue Krabbe's disease patients, not detected control brain. To study role psychosine death, we investigated effect on C6 glial survival 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. Similar ceramide (43.8% loss) galactopsychosine glucopsychosine treatment killed up 46.3 48.75% cells, respectively. On other hand, sphingosine had no effect. DNA laddering confirmed these results. Moreover, psychosine-induced detection annexin-V positive supports a death via pathway. These results indicate that may play GLD