| Project/Area Number |
25860623
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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 |
Cardiovascular medicine
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| Research Institution | Keio University |
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Principal Investigator |
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| Research Collaborator |
YUASA Shinsuke 慶應義塾大学, 医学部, 講師 (90398628)
EGASHIRA Toru 慶應義塾大学, 医学部, 助教 (10465023)
HASHIMOTO Hisayuki 慶應義塾大学, 医学部, 助教 (90528390)
KUSUMOTO Dai 慶應義塾大学, 医学部, 助教 (70571727)
KUNITOMI Akira 慶應義塾大学, 医学部, 助教 (30570882)
KASHIMURA Shin 慶應義塾大学, 医学部, 助教 (90571125)
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| Project Period (FY) |
2013-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2013: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Keywords | ミトコンドリア病 / iPS細胞 / 疾患解析 / 疾患モデル / iPS細胞を用いた疾患解析 |
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| Outline of Final Research Achievements |
We successfully established iPSCs from MELAS- fibroblasts. MELAS is one of the most common mitochondrial disease and there are no effective ways to cure the disease at the moment. MELAS-iPSC lines ranged from 3.6% to 99.4% of m.3243A>G heteroplasmy levels. The enzymatic activities of mitochondrial respiratory complexes indicated that MELAS-iPSC-derived fibroblasts with high heteroplasmy levels showed a deficiency of complex I activity but MELAS-iPSC-derived fibroblasts with low heteroplasmy levels showed normal complex I activity. Our data indicate that MELAS-iPSCs can be models for MELAS but we should carefully select MELAS-iPSCs with appropriate heteroplasmy levels and respiratory functions for mitochondrial disease modeling.
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