| Budget Amount *help |
¥17,160,000 (Direct Cost: ¥13,200,000、Indirect Cost: ¥3,960,000)
Fiscal Year 2020: ¥5,590,000 (Direct Cost: ¥4,300,000、Indirect Cost: ¥1,290,000)
Fiscal Year 2019: ¥5,590,000 (Direct Cost: ¥4,300,000、Indirect Cost: ¥1,290,000)
Fiscal Year 2018: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000)
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| Outline of Final Research Achievements |
Spinocerebellar ataxias (SCAs) are genetically heterogeneous group of autosomal dominantly inherited progressive disorders. Most SCA cause prominent damage to cerebellar Purkinje cells with consecutive cerebellar atrophy, whereas Purkinje cells are only mildly affected in some SCAs. To investigate differences of cell vulnerability in SCAs, we differentiated Purkinje cells and non-Purkinje cells, such as cortical neurons or motor neurons, from SCA patient-derived iPS cells. We examined cell type specific vulnerabilities utilizing histological analysis and transcriptome analysis combination with perturbation of culture conditions. We observed a certain type of SCA derived Purkinje cells revealed robustness whereas other SCA-derived Purkinje cells were fragile in morphological changes.
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