| Budget Amount *help |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Outline of Final Research Achievements |
In this study, I have investigated the effect of TFEB on cellular pathology of lysosomal storage diseases. In the model cells of GM1-gangliosidosis and Gaucher disease, TFEB was localized in the nucleus and was up-regulated its transcriptional activities. When cells were overexpressed with TFEB, the activities of mutant lysosomal enzymes were enhanced. Moreover, TFEB with chaperone compounds showed synergetic effects on the enhancement of mutant enzyme activities in GM1-gangliosidosis cells. These results indicates the possibility that up-regulation of TFEB activity could induce a beneficial effect on cells with lysosomal storage disease.
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