| Project/Area Number |
25830040
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Multi-year Fund |
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| Research Field |
Nerve anatomy/Neuropathology
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| Research Institution | Tokai University |
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Principal Investigator |
OTOMO Asako 東海大学, 医学部, 助教 (50535226)
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| Project Period (FY) |
2013-04-01 – 2016-03-31
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| Project Status |
Completed (Fiscal Year 2015)
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| Budget Amount *help |
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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| Keywords | ALS / ALS2 / iPS cell / 筋萎縮性側索硬化症2型 / iPS / 筋萎縮性側索硬化症 / 筋萎縮性側索硬化症２型 / iPS細胞 / 運動ニューロン / 末梢血 / 家族性筋萎縮性側索硬化症2型 |
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| Outline of Final Research Achievements |
ALS2 is a juvenile recessive form of ALS. We and others reported that loss of function of the ALS2 protein (ALS2) accounts for motor neuron degeneration (MND) in the patients. However, the underling mechanism of MND in the patient is not fully understood. Recently, Japanese ALS2 cases were reported. In this study, to further analyze the pathogenesis of ALS2, we established iPS cell lines of Japanese ALS2 patients and analyzed the character of a motor neuron model of ALS2 differentiated from the iPS cells. We succeeded to generate iPS cell lines from two independent ALS2 patients. Further, by the treatment of several inhibitors, we differentiated the iPS cells to HB9/Ilet-1-positive motor neurons, which allowed us to explore the cellular phenotype. Currently, a comparison of RNA-seq data from the ALS2 model cells with those from non-patient derived motor neurons is in progress. Further analysis may uncover the signal pathway specifically disturbed in ALS2.
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