| Project/Area Number |
24591269
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Kumamoto University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
ERA Takumi 熊本大学, 発生医学研究所, 教授 (00273706)
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| Project Period (FY) |
2012-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2012: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Keywords | 筋萎縮性側索硬化症 / iPS細胞 / 遠位型ミオパチー / 小胞体ストレス / 運動ニューロン |
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| Outline of Final Research Achievements |
We have established iPS cell lines derived from sporadic and familial ALS (SOD1, FUS, and MATR3 mutations) patients. We induced these iPS cells into motor neurons, and examined the expression of endoplasmic reticulum (ER) stress-related molecules. In this experiment, some of ER stress-related molecules including ER chaperones, transcription factors, and pro-apoptotic factors were upregulated in the mutant FUS-expressing cells. However, a few molecules were affected uniformly in the different types of the FUS mutations. A further examination would be required to confirm the results. We also needs to consider limitations under the experiments using iPS cells, such as variability among iPS clones and developmental condition into motor neurons.
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