Cellular engineering for treatment of animal model for inherited brain disorder

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1) Pathogenesis and treatment using animal model of Krabbe's disease : Animal inodel of Krabbe disease, named twitcher's brain was cultured and chased morphological and biochemical changes in oligodendroglial cell cultures. Pyschosine was accumulated 100 times more than control in twitcher tissues. the accumulation of psychosine alters membrane signal transduction system. 2) Niemann-Pick mouse model : Niemann-Pick mouse in mouse is equivalent to human Niemann-Pick type C, since free cholesterol and various glycolipids were accumulated in this mutant mice. The accumulation of these lipids are closely related to Golgi-lysosomal membrane complexes. Therefore, we speculate that abnormality of this mutant mice may be caused by lysosomal membrane transport system. 3) Introduction of nerve growth factor gene into neuroblastma cells. We succeeded transfection of NGF receptor gene intoneuroblastma cells and found benign process of neural cells.