The cause of neuropathochemistry of inherited Neurodegeneration

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The cause of neurological findings in inherited neurodegernerative disorders is unknown. In this studies, we attempt to elucidate the possible cause of neurological disorder, particularly in Gaucher disease.Gaucher disease is characterized by the accumulation of glucocerebroside in reticuloendotherial cells caused by a deficiency of lysosomal glucocerebrosidase. In central nervous tissues with Gaucher disease, there is few accumulation of glucocerebroside. The difference of the degree of accumulation of glucocerebroside seems to be cell type specific phenomenon. Therefore, we tested these finding using different tumor cells such as human oligodendroglioma cells, human neuroblastoma cells, Conduritol-B-epoxide (CBE) is a potent inhibitor for lysosomal betaglucosidase. Administration of CBE,less than 100ug/ml per bottle into cultured human oligodendroglioma cells produced significant accumulation of glucocerebroside. Simultaneously, enzyme activity was completely inhibited by administration of CBE at the same concentration. Morphological pictures shows abnormal membranous cytoplasmic body in HOG cells, whereas in human neuroblastoma cells. These data suggest the morphological picture in Gaucher disease is different from cells to cells.