2015 Fiscal Year Final Research Report
Elucidating the RNA pathomechanism in FUS-FALS iPS induced motor neurons
Project/Area Number |
25293199
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Partial Multi-year Fund |
Section | 一般 |
Research Field |
Neurology
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Research Institution | Tohoku University |
Principal Investigator |
Aoki Masashi 東北大学, 医学(系)研究科(研究院), 教授 (70302148)
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Co-Investigator(Kenkyū-buntansha) |
Kato MASAAKI 東北大学, 病院, 助教 (50622479)
Naoki SUZUKI 東北大学, 病院, 助教 (70451599)
Hitoshi WARITA 東北大学, 大学院医学系研究科, 助教 (30400245)
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Co-Investigator(Renkei-kenkyūsha) |
OKANO Hideyuki 慶應義塾大学, 医学部, 教授 (60160694)
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Project Period (FY) |
2013-04-01 – 2016-03-31
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Keywords | 筋萎縮性側索硬化症 / iPS細胞 |
Outline of Final Research Achievements |
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease resulting in the selective death of motor neurons. ALS symptoms are associated with muscle weakness and paralysis and approximately 80% of ALS patients die within 5 years after the onset of these symptoms. We generated induced pluripotent stem cells (iPSC) from familial ALS (FALS) patients with a missense mutation in the fused-in sarcoma (FUS) gene carrying the heterozygous FUS H517D mutation These cell-derived motor neurons mimicked several neurodegenerative phenotypes including mis-localization of FUS into cytosolic and stress granules under stress conditions, and cellular vulnerability (Ichiyanagi et al. Stem Cell Reports 2016).
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Free Research Field |
神経内科学
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