| Budget Amount *help |
¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
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| Outline of Final Research Achievements |
Glycogen storege disease type Ia (GSD Ia) develop accumulation of glycogen in liver and hypoglycemia by impaired glyconeogenesis because of defect of Glucose-6-Phosphatase (G6Pase) in endoplasmic reticulum. In this study, we developed each iPS cell line with mutation of G6Pase gene from two patients with GSD Ia. Then, we induced iPS cells derived form normal subject and patients with GSD Ia into differentiated hepatocyte. The autophagy in hepatocyte from patients with GSD Ia more enhanced compared to that of hepatocyte from normal subject. Moreover, the autophagy in hepatocyte from patients with GSD Ia increasingly upregulated under low-glucose culture.
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