Elucidating cell type-specific pathology of ALS using isogenic iPS cells
| Project/Area Number |
15H05667
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| Research Category |
Grant-in-Aid for Young Scientists (A)
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | Tohoku University |
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Principal Investigator |
Suzuki Naoki 東北大学, 大学病院, 助教 (70451599)
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥23,660,000 (Direct Cost: ¥18,200,000、Indirect Cost: ¥5,460,000)
Fiscal Year 2017: ¥7,150,000 (Direct Cost: ¥5,500,000、Indirect Cost: ¥1,650,000)
Fiscal Year 2016: ¥8,190,000 (Direct Cost: ¥6,300,000、Indirect Cost: ¥1,890,000)
Fiscal Year 2015: ¥8,320,000 (Direct Cost: ¥6,400,000、Indirect Cost: ¥1,920,000)
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| Keywords | 筋萎縮性側索硬化症 / 疾患特異的iPS細胞 / iPS / ゲノム編集 / iPS細胞 / FUS |
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| Outline of Final Research Achievements |
FUS gene mutation in 13 families was identified by accumulation of familial ALS families. Among them, skin biopsy of FUS-related ALS (FUS-ALS) in two families was carried out with consent in accordance with the procedure approved by the Ethics Committee of the University. From this, primary culture fibroblasts were established and iPS cells were established at a collaborating researcher's facility. We analyzed RNA sequence sequence after motor neuron differentiation and reported to Stem Cell Reports in 2016. We also produced isogenic lines using genome editing technology. In order to reproduce the characteristic long axon protrusions of motor neurons in the culture environment using the iPS cells produced, we also examined the condition in a novel microfluidic device. By RNAseq analysis, gene profiles expressed in cell bodies and axons were identified.
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Report
(4 results)
Research Products
(19 results)
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[Journal Article] Pathomechanisms of anti-cytosolic 5'-nucleotidase 1A autoantibodies in sporadic inclusion body myositis.2017
Author(s)
Tawara N, Yamashita S, Zhang X, Korogi M, Zhang Z, Doki T, Matsuo Y, Nakane S, Maeda Y, Sugie K, Suzuki N, Aoki M, Ando Y.
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Journal Title
Ann Neurol.
Volume: 81
Issue: 4
Pages: 512-525
DOI
Related Report
Peer Reviewed / Acknowledgement Compliant
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[Journal Article] Establishment of in vitro FUS-associated familial amyotrophic lateral sclerosis model using human induced pluripotent stem cells.2016
Author(s)
Naoki Ichiyanagi, Koki Fujimori, Masato Yano, Chikako Ishihara-Fujisaki, Takefumi Sone, Tetsuya Akiyama, Yohei Okada, Wado Akamatsu, Takuya Matsumoto, Mitsuru Ishikawa, Yoshinori Nishimoto, Yasuharu Ishihara, Tetsushi Sakuma, Takashi Yamamoto, Hitomi Tsuiji, Naoki Suzuki, Hitoshi Warita, Masashi Aoki, Hideyuki Okano
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Journal Title
Stem Cell Reports
Volume: 6
Issue: 4
Pages: 496-510
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Journal Article] Next-generation sequencing of 28 ALS-related genes in a Japanese ALS cohort2016
Author(s)
Ryoichi Nakamura, Jun Sone, Naoki Atsuta, Genki Tohnai, Hazuki Watanabe, Daichi Yokoi, Masahiro Nakatochi, Hirohisa Watanabe, Mizuki Ito, Jo Senda, Masahisa Katsuno, Fumiaki Tanaka, Yuanzhe Li, Yuishin Izumi, Mitsuya Morita, Akira Taniguchi, Osamu Kano, Masaya Oda, Satoshi Kuwabara, Koji Abe.
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Journal Title
Neurobiology of Aging
Volume: 39
Pages: 219-219
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
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[Journal Article] A mutation in hnRNPA1 causes isolated inclusion body myopathy in two families with multisystem proteinopathy2015
Author(s)
Izumi R, Warita H, Niihori T, Takahashi T, Tateyama M, Suzuki N, Nishiyama A, Shirota M, Funayama R, Nakayama K, Mitsuhashi S, Nishino I, Aoki Y, Aoki M.
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Journal Title
Neurology Genetics
Volume: .
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
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