Budget Amount *help |
¥45,630,000 (Direct Cost: ¥35,100,000、Indirect Cost: ¥10,530,000)
Fiscal Year 2016: ¥8,970,000 (Direct Cost: ¥6,900,000、Indirect Cost: ¥2,070,000)
Fiscal Year 2015: ¥8,970,000 (Direct Cost: ¥6,900,000、Indirect Cost: ¥2,070,000)
Fiscal Year 2014: ¥8,970,000 (Direct Cost: ¥6,900,000、Indirect Cost: ¥2,070,000)
Fiscal Year 2013: ¥8,840,000 (Direct Cost: ¥6,800,000、Indirect Cost: ¥2,040,000)
Fiscal Year 2012: ¥9,880,000 (Direct Cost: ¥7,600,000、Indirect Cost: ¥2,280,000)
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Outline of Final Research Achievements |
Peroxisomal disorders caused by defects in peroxisome biogenesis, peroxisomal β-oxidation, or biosynthesis of plasmalogens manifest severe neural disorders of the central nervous system (CNS). Their molecular pathogenesis remains to be elucidated. In this research, we showed that peroxisomal metabolism was essential for not only the morphogenesis but also the integrity of CNS. The impairment of a secretory factor essential for the morphogenesis of CNS was found in the mouse defective in peroxisome biogenesis. We also proposed the relationship between pathological phenotype and defect of peroxisomal metabolisms using fibroblasts from the patients with peroxisomal biogenesis disorders and peroxisomal single enzyme deficiency diseases. Furthermore, we also reported that the plasmalogen-dependent regulation of cholesterol biosynthesis and release of catalase, a peroxisomal matrix enzyme, from peroxisomes to the cytosol upon the oxidative stress.
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