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Elucidating cell type-specific pathology of ALS using isogenic iPS cells

Research Project

Project/Area Number 15H05667
Research Category

Grant-in-Aid for Young Scientists (A)

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionTohoku University

Principal Investigator

Suzuki Naoki  東北大学, 大学病院, 助教 (70451599)

Project Period (FY) 2015-04-01 – 2018-03-31
Project Status Completed (Fiscal Year 2017)
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)
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
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.

Report

(4 results)
  • 2017 Annual Research Report   Final Research Report ( PDF )
  • 2016 Annual Research Report
  • 2015 Annual Research Report
  • Research Products

    (19 results)

All 2017 2016 2015

All Journal Article (16 results) (of which Int'l Joint Research: 4 results,  Peer Reviewed: 14 results,  Open Access: 10 results,  Acknowledgement Compliant: 6 results) Presentation (3 results) (of which Invited: 3 results)

  • [Journal Article] Aberrant astrocytic expression of chondroitin sulfate proteoglycan receptors in a rat model of amyotrophic lateral sclerosis2017

    • Author(s)
      Shijo Tomomi、Warita Hitoshi、Suzuki Naoki、Kitajima Yasuo、Ikeda Kensuke、Akiyama Tetsuya、Ono Hiroya、Mitsuzawa Shio、Nishiyama Ayumi、Izumi Rumiko、Aoki Masashi
    • Journal Title

      Journal of Neuroscience Research

      Volume: 96 Issue: 2 Pages: 222-233

    • DOI

      10.1002/jnr.24127

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Comprehensive targeted next-generation sequencing in Japanese familial amyotrophic lateral sclerosis.2017

    • Author(s)
      Nisiyama A, Niihori T, Warita H, Izumi R, Akiyama T, Kato M, Suzuki N, Aoki Y, Aoki M.
    • Journal Title

      Neurobiol Aging

      Volume: 53 Pages: 194.e1-194.e8

    • DOI

      10.1016/j.neurobiolaging.2017.01.004

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [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.
    • Journal Title

      Ann Neurol.

      Volume: 81 Issue: 4 Pages: 512-525

    • DOI

      10.1002/ana.24919

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Comprehensive targeted next-generation sequencing in Japanese familial amyotrophic lateral sclerosis.2017

    • Author(s)
      Nishiyama, A... Suzuki, N et al.
    • Journal Title

      Neurobiol Aging

      Volume: 53

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).2016

    • Author(s)
      Klionsky, D. J. ... Suzuki N,...et al.
    • Journal Title

      Autophagy

      Volume: 12 Pages: 1-222

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Genotype-phenotype relationships in familial ALS with FUS/TLS mutations in Japan2016

    • Author(s)
      Akiyama T, Warita H, Kato M, Nishiyama A, Izumi R, Ikeda C, Kamada M, Suzuki N, Aoki M
    • Journal Title

      Muscle Nerve

      Volume: Jan 28 Issue: 3 Pages: 398-404

    • DOI

      10.1002/mus.25061

    • Related Report
      2016 Annual Research Report 2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [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
    • Journal Title

      Stem Cell Reports

      Volume: 6 Issue: 4 Pages: 496-510

    • DOI

      10.1016/j.stemcr.2016.02.011

    • Related Report
      2016 Annual Research Report 2015 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] SLC52A3, A Brown-Vialetto-van Laere syndrome candidate gene is essential for mouse development, but dispensable for motor neuron differentiation.2016

    • Author(s)
      Intoh, A., Suzuki, N., Koszka, K. & Eggan, K.
    • Journal Title

      Hum Mol Genet

      Volume: 25 Issue: 9 Pages: 1814-1823

    • DOI

      10.1093/hmg/ddw053

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease.2016

    • Author(s)
      Burberry, A... Suzuki, N et al.
    • Journal Title

      Sci Transl Med

      Volume: 8 Issue: 347

    • DOI

      10.1126/scitranslmed.aaf6038

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [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.
    • Journal Title

      Neurobiology of Aging

      Volume: 39 Pages: 219-219

    • DOI

      10.1016/j.neurobiolaging.2015.11.030

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Deficient RNA-editing enzyme ADAR2 in an amyotrophic lateral sclerosis patient with a FUSP525L mutation2016

    • Author(s)
      Aizawa H, Hideyama T, Yamashita T, Kimura T, Suzuki N, Aoki M, Kwak S
    • Journal Title

      J Clin Neurosci

      Volume: 32 Pages: 128-129

    • DOI

      10.1016/j.jocn.2015.12.039

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging.2016

    • Author(s)
      Tian, F... Suzuki, N et al.
    • Journal Title

      Nat Commun

      Volume: 7 Issue: 1 Pages: 13283-13283

    • DOI

      10.1038/ncomms13283

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Guidelines for the Use and Interpretation of Assays for Monitoring Autophagy (3rd edition)2016

    • Author(s)
      Klionsky DJ …Suzuki N…Zughaier SM.
    • Journal Title

      Autophagy

      Volume: 12 Pages: 1-222

    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Journal Article] SLC52A3, A Brown-Vialetto-van Laere Syndrome Candidate Gene is Essential for Mouse Development but Dispensable for Motor Neuron Differentiation.2016

    • Author(s)
      Intoh A, Suzuki N, Kathryn K, Eggan K.
    • Journal Title

      Hum Mol Genet

      Volume: .

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [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.
    • Journal Title

      Neurology Genetics

      Volume: .

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] [Review/Advances in Neurological Therapeutics (2014)]2015

    • Author(s)
      Suzuki N, Kato M, Warita H, Aoki M.
    • Journal Title

      Neurological Therapeutics

      Volume: 32 Pages: 475-478

    • Related Report
      2015 Annual Research Report
  • [Presentation] マイクロ流体デバイスを用いた運動ニューロンの軸索病態の解析.2017

    • Author(s)
      鈴木直輝,秋山徹也,川田治良,舟山亮,岡野栄之, 中山啓子,藤井輝夫,青木正志
    • Organizer
      第69回日本細胞生物学会大会
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] Regenerative therapies for ALS using hepatocyte growth factor2016

    • Author(s)
      Naoki Suzuki
    • Organizer
      3rd World Centenarian Intiative International Symposium on Amyotrophic Lateral Sclerosis
    • Place of Presentation
      Senri Life Science center, Osaka, Japan
    • Related Report
      2016 Annual Research Report
    • Invited
  • [Presentation] Deciphering the expression and function of C9ORF72 using mouse model2016

    • Author(s)
      Naoki Suzuki
    • Organizer
      57th Annual Meeting of the Japanese Society of Neurology
    • Place of Presentation
      Convention center, Kobe, Japan
    • Related Report
      2016 Annual Research Report
    • Invited

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Published: 2015-04-16   Modified: 2019-03-29  

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