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Noguchi Satoru  野口 悟

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NOGUCHI Satoru  野口 悟

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Researcher Number 00370982
Other IDs
Affiliation (Current) 2025: 国立研究開発法人国立精神・神経医療研究センター, 神経研究所 疾病研究第一部, 室長
Affiliation (based on the past Project Information) *help 2015 – 2017: 国立研究開発法人国立精神・神経医療研究センター, 神経研究所 疾病研究第一部, 室長
2015: 国立研究開発法人国立精神・神経医療研究センター, 神経研究所疾病研究第一部, 室長
2012 – 2014: 独立行政法人国立精神・神経医療研究センター, 神経研究所 疾病研究第一部, 室長
2013: 国立精神・神経医療研究センター神経研究所, 疾病研究第一部, 室長
2010 – 2011: 独立行政法人国立精神・神経医療研究センター, 神経研究所・疾病研究第一部, 室長 … More
2010: 独立行政法人 国立精神・神経医療研究センター, 神経研究所疾病研究第一部, 室長
2009: 国立精神・神経センター, 神経研究所・疾病研究第一部, 室長
2008: 国立精神, 神経センター・神経研究所疾病研究第一部, 室長
2008: 国立精神・神経センター, 神経研究所疾病研究第一部, 室長
2008: 東邦大学, 国立精神・神経センター・神経研究所疾病研究第一部, 室長
2007: 国立精神・神経センター, 神経研究所・疾病研究第一部, 室長
2007: National Institute of Neuroscience, Depertment of Neuromuscular Research, Section chief
2006: National Institute of Neuroscience, Department of Neuromuscular Research, Section Chief, 神経研究所 疾病研究第一部, 室長
2006: 国立精神・神経センター, 神経研究所疾病研究第一部, 室長
2004 – 2005: 国立精神・神経センター, 疾病研究第一部, 室長 Less
Review Section/Research Field
Principal Investigator
Neurology
Except Principal Investigator
Neurology / Pediatrics
Keywords
Principal Investigator
骨格筋 / 糖鎖 / 遺伝病 / チャンネル / モデルマウス / 病態 / カルシウム / ミオパチー / 遺伝性疾患 / アミロイド / 糖 / 蛋白質 / 細胞・組織 … More
Except Principal Investigator
… More myotilin / ZASP / GNE / HIBM / DMRV / オートファジー / 筋疾患 / 脳神経疾患 / 細胞・組織 / limb-girdle muscular dystrophy / myofibrillar myopathy / cDNA microarray / calpain 3 / sarcomere / Neuromuscular disorders / ミオフィプリラーミオパチー / 分子病理学 / 拡張型心筋症 / マルチミニコア / ミオフィブリラーミオパチー / 臨床遺伝学 / 肢帯型筋ジストロフィー / ミオティリン / 筋原線維性ミオパチー / cDNAマイクロアレイ / カルパイン3 / サルコメア / 神経・筋疾患 / autophagy / rimmed vacuole / 縁取り空砲 / 縁取り空胞 / 間質前駆細胞 / コラーゲン / 治療 / 骨格筋 / 遺伝病 / 発現制御 / 発現抑制 / ゲノム / メチル化 / エピゲノム / 遺伝学 / 神経分子病態 / 神経分子病態学 / 筋病理 / 遺伝子発現 / 遺伝子欠失 / スプライシング / long PCR法 / D4Z4リピート / Rimmed vacuoles / SIL1 / マリネスコーシェーグレン症候群 / 筋病理学 / 小児神経学 / マイクロマレイ / マクロアレイ / シグナル伝達 / 病理 / 遺伝子 / 臨床 / 筋ジストロフィー / 核膜 Less
  • Research Projects

    (10 results)
  • Research Products

    (82 results)
  • Co-Researchers

    (21 People)
  •  Elucidation of pathogenesis of muscular dystrophies caused by constitutive extracellular calcium influxPrincipal Investigator

    • Principal Investigator
      Noguchi Satoru
    • Project Period (FY)
      2015 – 2017
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Elucidation of molecular pathomechanism and development of therapy for collagen VI deficiency

    • Principal Investigator
      NISHINO Ichizo
    • Project Period (FY)
      2014 – 2016
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Epigenomics and muscular dystrophy

    • Principal Investigator
      HAYASHI Yukiko
    • Project Period (FY)
      2012 – 2013
    • Research Category
      Grant-in-Aid for Challenging Exploratory Research
    • Research Field
      Neurology
    • Research Institution
      Tokyo Medical University
      独立行政法人国立精神・神経医療研究センター
  •  Elucidation of the mechanism for formation of protein aggregates in skeletal myofibers with distal myopathy with rimmed vacuolesPrincipal Investigator

    • Principal Investigator
      NOGUCHI Satoru
    • Project Period (FY)
      2011 – 2014
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Elucidation of pathomechanism of and development of therapy ofautophagy-related muscle disorders

    • Principal Investigator
      NISHINO Ichizo
    • Project Period (FY)
      2008 – 2010
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Pathomechanism of SIL1-mutated Marinesco-Sjogren syndrome

    • Principal Investigator
      OKADA Mari
    • Project Period (FY)
      2007 – 2008
    • Research Category
      Grant-in-Aid for Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      Toho University
      National Center of Neurology and Psychiatry
  •  顔面肩甲上腕型筋ジストロフィーにおけるエピジェネティック変化と病態機序の解明

    • Principal Investigator
      林 由起子
    • Project Period (FY)
      2007
    • Research Category
      Grant-in-Aid for Exploratory Research
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Clinical and molecular studies of Emery-Dreifuss muscular dystrophy

    • Principal Investigator
      HAYASHI Yukiko
    • Project Period (FY)
      2006 – 2008
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Clarification of pathomechanism of inherited muscular disorder due to abnormality of sarcomeric proteins

    • Principal Investigator
      MINAMI Narihiro
    • Project Period (FY)
      2006 – 2007
    • Research Category
      Grant-in-Aid for Scientific Research (C)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry
  •  Research on the elucidation of molecular pathomechanism of and the development of therapy of lysosomal muscle diseases.

    • Principal Investigator
      NISHINO Ichizo
    • Project Period (FY)
      2004 – 2006
    • Research Category
      Grant-in-Aid for Scientific Research (A)
    • Research Field
      Neurology
    • Research Institution
      National Center of Neurology and Psychiatry

All 2017 2016 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 2004 Other

All Journal Article Presentation Book

  • [Book] Molecular Pathogenesis and Therapeutic Strategy in GNE Myopathy. Translational Research in Muscular Dystrophy (Ed. Takeda S, Miyagoe-Suzuki Y, Mori-Yoshimura M)2016

    • Author(s)
      Nishimura H, Noguchi S
    • Total Pages
      13
    • Publisher
      Springer Japan
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Book] Muscle aging, inclusion-body myositis and myopathies2012

    • Author(s)
      Malicdan MC, Noguchi S, Nishino I
    • Total Pages
      250
    • Publisher
      Wiley-Blackwell
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Book] 疾患モデルマウス表現型解析指南2011

    • Author(s)
      野口悟, Malicdan MC, 西野一三
    • Total Pages
      477
    • Publisher
      中山書店
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy2017

    • Author(s)
      Noguchi S, Ogawa M, Malicdan MC, Nonaka I, Nishino I
    • Journal Title

      EBioMedicine

      Volume: 15(2017) Pages: 193-202

    • DOI

      10.1016/j.ebiom.2016.12.011

    • Peer Reviewed / Acknowledgement Compliant / Open Access / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846, KAKENHI-PROJECT-26293214
  • [Journal Article] Sialic acid deficiency is associated with oxidative stress leading to muscle atrophy and weakness in the GNE myopathy2017

    • Author(s)
      1.Cho A, Malicdan MCV, Miyakawa M, Nonaka I, Nishino I, Noguchi S
    • Journal Title

      Hum Mol Genet

      Volume: 26 Issue: 16 Pages: 3081-3093

    • DOI

      10.1093/hmg/ddx192

    • Peer Reviewed / Open Access / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Journal Article] Calcium Dyshomeostasis in Tubular Aggregate Myopathy2016

    • Author(s)
      Lee JM, Noguchi S
    • Journal Title

      Int J Mol Sci.

      Volume: 17(11) Issue: 11 Pages: 1952-1952

    • DOI

      10.3390/ijms17111952

    • Peer Reviewed / Acknowledgement Compliant / Open Access / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Journal Article] Milder forms of muscular dystrophy associated with POMGN2 mutations.2015

    • Author(s)
      Endo Y, Dong M, Noguchi S, Ogawa M, Hayashi YK, Kuru S, Sugiyama K, Nagai S, Ozasa S, Nonaka I, Nishino I
    • Journal Title

      Neurol Genet.

      Volume: 1(4) Issue: 4

    • DOI

      10.1212/nxg.0000000000000033

    • Peer Reviewed / Open Access
    • Data Source
      KAKENHI-PROJECT-26293214
  • [Journal Article] Plasma IP-10 level distinguishes inflammatory myopathy2015

    • Author(s)
      Uruha, A., S. Noguchi, W. Sato, H. Nishimura, S. Mitsuhashi, T. Yamamura, and I. Nishino
    • Journal Title

      Neurology

      Volume: 85 Issue: 3 Pages: 293-294

    • DOI

      10.1212/wnl.0000000000001767

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-24229006, KAKENHI-PROJECT-26860679, KAKENHI-PROJECT-26293214
  • [Journal Article] Allele-specific Gene Silencing of Mutant mRNA Restores Cellular Function in Ullrich Congenital Muscular Dystrophy Fibroblasts.2014

    • Author(s)
      Noguchi S, Ogawa M, Kawahara G, Malicdan MC, Nishino I
    • Journal Title

      Mol Ther Nucleic Acids

      Volume: 3 Pages: e171-e171

    • DOI

      10.1038/mtna.2014.22

    • Peer Reviewed / Acknowledgement Compliant / Open Access
    • Data Source
      KAKENHI-PROJECT-23390236, KAKENHI-PROJECT-26293214
  • [Journal Article] Mutation profile of the GNE gene in Japanese patients with distal myopathy with rimmed vacuoles (GNE myopathy).2014

    • Author(s)
      Cho A, et al.
    • Journal Title

      J Neurol Neurosurg Psychiatry.

      Volume: 85 Issue: 8 Pages: 914-917

    • DOI

      10.1136/jnnp-2013-305587

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-23390236, KAKENHI-PROJECT-25461323
  • [Journal Article] Sialyllactose ameliorates myopathic phenotypes in symptomatic GNE myopathy model mice.2014

    • Author(s)
      27.Yonekawa T, Malicdan MC, Cho A, Hayashi YK, Nonaka I, Mine T, Yamamoto T, Nishino I, Noguchi S
    • Journal Title

      Brain

      Volume: 137 Issue: 10 Pages: 2670-2679

    • DOI

      10.1093/brain/awu210

    • Acknowledgement Compliant
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] Congenital fiber type disproportion myopathy caused by LMNA mutations.2014

    • Author(s)
      Kajino S, Ishihara K, Goto K, Ishigaki K, Noguchi S, Nonaka I, Osawa M, Nishino I, Hayashi YK
    • Journal Title

      J Neurol Sci

      Volume: 340 Issue: 1-2 Pages: 94-98

    • DOI

      10.1016/j.jns.2014.02.036

    • Peer Reviewed / Acknowledgement Compliant
    • Data Source
      KAKENHI-PROJECT-24390227, KAKENHI-PROJECT-24659437
  • [Journal Article] Absence of beta-amyloid deposition in the central nervous system of a transgenic mouse model of distal myopathy with rimmed vacuoles.2014

    • Author(s)
      Anada RP, Wong KT, Malicdan MC, Goh KJ, Hayashi YK, Nishino I, Noguchi S
    • Journal Title

      Amyloid

      Volume: 21 Issue: 2 Pages: 138-139

    • DOI

      10.3109/13506129.2014.889675

    • Peer Reviewed / Acknowledgement Compliant
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] A nationwide survey on Marinesco-Sjogren syndrome in Japan.2014

    • Author(s)
      Masahide Goto, Mari Okada, Hirofumi Komaki, Kenji Sugai, Masayuki Sasaki, Satoru Noguchi, Ikuya Nonaka, Ichizo Nishino, and Yukiko K. Hayashi.
    • Journal Title

      Orphanet J Rare Diseases

      Volume: 9 Issue: 1 Pages: 58-58

    • DOI

      10.1186/1750-1172-9-58

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-24390227, KAKENHI-PROJECT-24659437
  • [Journal Article] Peracetylated N-acetylmannosamine, a synthetic sugar molecule, effectively rescues muscle phenotype and biochemical defects in a mouse model of sialic acid deficient myopathy2012

    • Author(s)
      Malicdan MC, Noguchi S, Tokutomi T, Goto YI, Nonaka I, Hayashi YK, Nishino I
    • Journal Title

      Journal of Biological Chemistry

      Volume: 287 Issue: 4 Pages: 2689-2705

    • DOI

      10.1074/jbc.m111.297051

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] シアル酸の低下により引き起こされる骨格筋疾患2011

    • Author(s)
      野口悟
    • Journal Title

      生化学

      Volume: 83 Pages: 316-320

    • NAID

      10029068226

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] 遠位型ミオパチーの治療法開発2011

    • Author(s)
      野口悟, 西野一三
    • Journal Title

      生体の科学

      Volume: 62 Pages: 142-145

    • Data Source
      KAKENHI-PROJECT-23390236
  • [Journal Article] Autophagic degradation of nuclear components in mammalian cells2009

    • Author(s)
      Park YE, Hayashi YK, Bonne G, Arimura T, Noguchi S, Nonaka I, Nishino I
    • Journal Title

      Autophagy 5

      Pages: 795-804

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] Chapter 19 Monitoring Autophagy in Muscle Diseases2009

    • Author(s)
      Malicdan MCV, Noguchi S, Nishino I
    • Journal Title

      Methods Enzymol 453C

      Pages: 379-396

    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] Congenital myotonic dystrophy can show congenital fiber type disproportion pathology2009

    • Author(s)
      Tominaga, Hayashi YK, Goto K, Minami N, Noguchi S, 他
    • Journal Title

      Acta Neuropathol. 119

      Pages: 481-486

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] Novel FHL1 mutations in fatal and benign reducing body myopathy2009

    • Author(s)
      Shalaby S, Hayashi YK, Nonaka I, Noguchi S, Nishino I
    • Journal Title

      Neurology 72巻

      Pages: 375-376

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Autophagic degradation of nuclear components in mammalian cells2009

    • Author(s)
      Park YE, Hayashi YK, Bonne, G, Arimura T, Noguchi S, Nonaka I, Nishino I.
    • Journal Title

      Autophagy 5巻(in press)

      Pages: 0-0

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Prophylactic treatment with sialic acid metabolites precludes the development of the myopathic phenotype in the DMRV-hIBM mouse model2009

    • Author(s)
      Malicdan MC, Noguchi S, Hayashi YK, Nonaka I, Nishino I.
    • Journal Title

      Nat med 15

      Pages: 690-695

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Nuclear changes in skeletal muscle extend to satellite cells in autosomal dominant Emery-Dreifuss muscular dystrophy/limb-girdle muscular dystrophy 1B2009

    • Author(s)
      Park YE, Hayashi YK, Goto K, Komaki H, Hayashi Y, Inuzuka T, Noguchi S, Nonaka I, Nishino I.
    • Journal Title

      Neuromuscul Disord 19巻

      Pages: 29-36

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Autophagic degradation of nuclear components in mammalian cells2009

    • Author(s)
      Park YE, Hayashi YK, Bonne G, Arimura T, Noguchi S, 他
    • Journal Title

      Autophagy. 5

      Pages: 795-804

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency2009

    • Author(s)
      Liang WC, Ohkuma A, Hayashi YK, Lopez LC, Hirano M, Nonaka I, Noguchi S, Chen LH, Jong YJ, Nishino I.
    • Journal Title

      Neuromuscul Disord 19巻

      Pages: 212-216

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Congenital myotonic dystrophy can show congenital fiber type disproportion pathology2009

    • Author(s)
      Tominaga, Hayashi YK, Goto K, Minami N, Noguchi S, Nonaka I, Miki T, Nishino I
    • Journal Title

      Acta Neuropathol 119

      Pages: 481-486

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] Clinical and genetic analysis of lipid storage myopathies2009

    • Author(s)
      Ohkuma A, Noguchi S, Sugie H, Malicdan MC, Fukuda T, Shimazu K, Lopez LC, Hirano M, Hayashi YK, Nonaka I, Nishino I.
    • Journal Title

      Muscle Nerve 39巻

      Pages: 333-342

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] A novel POMT2 mutation causes mild congenital muscular dystrophy with normal brain MRI2009

    • Author(s)
      Murakami T, Hayashi YK, Ogawa M, Noguchi S, Campbell KP, Togawa M, Inoue T, Oka A, Ohno K, Nonaka I, Nishino I
    • Journal Title

      Brain Dev 31巻

      Pages: 465-468

    • NAID

      10025580402

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Distal lipid storage myopathy due to PNPLA2 mutation2008

    • Author(s)
      Ohkuma A, Nonaka I, Malicdan MC, Noguchi S, Ohji S, Nomura K, Sugie H, Hayashi YK, Nishino I
    • Journal Title

      Neuromuscul Disord 18巻

      Pages: 671-674

    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Rigid spine syndrome caused by a novel mutation in four-and-a-half LIM domain 1 gene (FHL1)2008

    • Author(s)
      Shalaby S, Hayashi YK, Goto K, Ogawa M, Nonaka I, Noguchi S, Nishino I.
    • Journal Title

      Neuromuscul Disord 18巻

      Pages: 959-961

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Diminished binding of mutated collagen VI to the extracellular matrix surrounding myocytes2008

    • Author(s)
      Kawahara G, Ogawa M, Okada M, Malicdan MCV, Goto Y, Hayashi YK, Noguchi S, Nishino I
    • Journal Title

      Muscle Nerve 38

      Pages: 1192-1195

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Journal Article] Diminished binding of mutated collagen VI to the extracellular matrix surrounding myocytes2008

    • Author(s)
      Kawahara G, Ogawa M, Okada M, Malicdan MC, Goto Y, Hayashi YK, Noguchi S, Nishino I.
    • Journal Title

      Muscle Nerve 38巻

      Pages: 1192-1195

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Emerinopathy and laminopathy clinical, pathological and molecular features of muscular dystrophy with nuclear envelopathy in Japan2007

    • Author(s)
      Astejada, MN, Goto K, Nagano A, Ura S, Noguchi S, Nonaka I, Nishino I, Hayashi YK
    • Journal Title

      Acta Myol 26巻

      Pages: 159-164

    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Limb-girdle muscular dystrophy due to emerin gene mutations2007

    • Author(s)
      Ura S, Hayashi YK, Goto K, Astejada MN, Murakami T, Nagato M, Ohta S, Daimon Y, Takekawa H, Hirata K, Nonaka I, Noguchi S, Nishino I
    • Journal Title

      Archives of neurology 64巻

      Pages: 1038-1041

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Reduced cell anchorage may cause sarcolemma-specific collagen VI deficiency in Ullrich disease2007

    • Author(s)
      Kawahara G, Okada M, Morone N, Ibarra CA, Nonaka I, Noguchi S, Hayashi YK, Nishino I.
    • Journal Title

      Neurology 69巻

      Pages: 1043-1049

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Primary collagen VI deficiency is the second most common congenital muscular dystrophy in Japan, Neurology2007

    • Author(s)
      Okada M, Kawahara G, Noguchi S, Sugie K, Murayama K, Nonaka I, Hayashi YK, Nishino I.
    • Journal Title

      Neurology 69巻

      Pages: 1035-1042

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-19591232
  • [Journal Article] Emerin-lacking mice show minimal motor and cardiac dysfunctions with nuclear-associated vacuoles2006

    • Author(s)
      Ozawa R, Hayashi YK, Ogawa M, Kurokawa R, Matsumoto H, Noguchi S, Nonaka I, Nishino I
    • Journal Title

      Am J Pathol 168巻

      Pages: 907-917

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-18390265
  • [Journal Article] Reduction of UDP-G1cNAc 2-epimerase/ManNAc kinase activity andsialylation in distal myopathy with rimmed vacuoles.2004

    • Author(s)
      Noguchi S, et al.
    • Journal Title

      J Biol Chem 279

      Pages: 11402-11407

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16209029
  • [Journal Article] Reduction of UDP-G1cNAc 2-epimerase/ManNAc kinase activity and sialylation in distal myopathy with rimmed vacuoles.2004

    • Author(s)
      Noguchi S, et al.
    • Journal Title

      J Biol Chem 279

      Pages: 11402-11407

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16209029
  • [Journal Article] Reduction of UDP-GlcNAc 2-epimerase/ManNAc kinase activity and sialylation in distal myopathy with rimmed vacuoles.2004

    • Author(s)
      Noguchi S, et al.
    • Journal Title

      J Biol Chem 279

      Pages: 11402-11407

    • Data Source
      KAKENHI-PROJECT-16209029
  • [Presentation] Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy2017

    • Author(s)
      Satoru Noguchi
    • Organizer
      International Conference on Collagen VI Disorders
    • Place of Presentation
      Washington, U.S.A.
    • Year and Date
      2017-02-24
    • Invited / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-26293214
  • [Presentation] Functional Effect of ORAI1 Mutations in Muscle Diseases2017

    • Author(s)
      Noguchi S
    • Organizer
      Gordon Research Conferences 2017
    • Invited / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy.2017

    • Author(s)
      Satoru Noguchi
    • Organizer
      International Conference on Collagen VI Disorders
    • Place of Presentation
      Washington, U.S.A.
    • Year and Date
      2017-02-24
    • Invited / Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] Tubular aggregate myopathy with dystrophic features2017

    • Author(s)
      Lee J, Yoshimura M, Hirano R, Miyatake W, Koshimizu E, Matsumoto N, Mori H, Tachii N, Suzuki M, Ogata K, Nishino I, Noguchi S
    • Organizer
      22nd International Congress of the World Muscle Society
    • Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] S-Nitrosylation Is Responsible for Muscle Atrophy and Weakness in GNE Myopathy2017

    • Author(s)
      Miyakawa M, Cho A, Malicdan MC, Nishino I, Noguchi S
    • Organizer
      ASBMB ANNUAL MEETING/ Experimental Biology 2017
    • Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] A mouse with exon 9 deletion in Col6a1 as a model for dominant collagen VI-related disorders2017

    • Author(s)
      Noguchi S, Ogawa M, Nishino I
    • Organizer
      22nd International Congress of the World Muscle Society
    • Int'l Joint Research
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] ジストロフィン-結合糖タンパク質複合体研究の最近の展開2016

    • Author(s)
      野口 悟
    • Organizer
      第2回日本筋学会学術集会記念講演
    • Place of Presentation
      東京都、小平市(国立精神・神経医療研究センター)
    • Year and Date
      2016-08-05
    • Invited
    • Data Source
      KAKENHI-PROJECT-15H04846
  • [Presentation] ジストロフィン-結合糖タンパク質複合体研究の最近の展開2016

    • Author(s)
      野口 悟
    • Organizer
      第2回日本筋学会学術集会記念講演
    • Place of Presentation
      東京都小平市(国立精神・神経医療研究センター)
    • Year and Date
      2016-08-05
    • Invited
    • Data Source
      KAKENHI-PROJECT-26293214
  • [Presentation] Two events in the pathogenesis of congenital muscular dystrophy with collagen VI deficiency.2015

    • Author(s)
      Noguchi S
    • Organizer
      10th Japanese-French Workshop
    • Place of Presentation
      Paris, France(Institute of Myology)
    • Year and Date
      2015-07-02
    • Invited
    • Data Source
      KAKENHI-PROJECT-26293214
  • [Presentation] Sialyllactose trial on GNE myopathy mouse model.2014

    • Author(s)
      Noguchi S
    • Organizer
      GNE myopathy Consortium Workshop,
    • Place of Presentation
      Berlin, Germany (BEUTH HOCHSCHULE FUR TECHNIK BERLIN University of Applied Sciences)
    • Year and Date
      2014-10-12
    • Invited
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] ネマリンミオパチーの臨床遺伝学的解析2013

    • Author(s)
      林由起子,本村和嗣,後藤加奈子,野口 悟,宮武聡子,輿水江里子,松本直通,西野一三
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Data Source
      KAKENHI-PROJECT-24659437
  • [Presentation] GNE myopathy - Inclusion body myopathy (IBM2).2013

    • Author(s)
      Noguchi S
    • Organizer
      EUROPEAN NEURO MUSCULAR CENTRE (ENMC) WORKSHOP
    • Place of Presentation
      The Netherlands (NH Jan Tabak Hotel)
    • Invited
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Mutation screening of a large cohort of nemaline myopathy2013

    • Author(s)
      Hayashi YK, Goto K, Noguchi S, Matsumoto N, Laing N, North K, Clark N, Nonaka I, Nishino I
    • Organizer
      International Congress of the World Muscle Society
    • Place of Presentation
      USA
    • Data Source
      KAKENHI-PROJECT-24659437
  • [Presentation] GNE myopathy - Pathogenesis and therapeutic strategies.2012

    • Author(s)
      Noguchi S, et al.
    • Organizer
      9th Japanese- French Symposium for ‘muscular dystrophy’ Tokyo
    • Place of Presentation
      東京、JA共済ビル
    • Invited
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Metabolic changes in sialic acid synthesis pathway in GNE-myopathy model mice with long-term sialic acid treatment.2012

    • Author(s)
      Noguchi S, et al.
    • Organizer
      2012 New Directions in Biology and Disease of Skeletal Muscle Conference
    • Place of Presentation
      The Westin New Orleans Canal Place, New Orleans, USA
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Allele-specific knockdown to mutant mRNA retrieves cellular function in fibroblasts with point-mutated Ullrich CMD.2012

    • Author(s)
      Noguchi S, et al.
    • Organizer
      17th International Congress of the World Muscle Society
    • Place of Presentation
      Perth Convention Exhibition Centre, Perth, Australia
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] GNEミオパチーの発症機序と治療戦略2012

    • Author(s)
      野口 悟,他
    • Organizer
      第35回日本分子生物学会年会
    • Place of Presentation
      福岡、福岡国際会議場
    • Invited
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Metabolic changes in sialic acid synthesis pathway in DMRV/hIBM model mice with long-term sialic acid treatment2011

    • Author(s)
      Noguchi S, Malicdan MC, Funato F, Nishino I
    • Organizer
      16th International Congress of the World Muscle Society
    • Place of Presentation
      アルガルヴェ,ポルトガルRia Park Hotel
    • Year and Date
      2011-10-19
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Metabolic changes in sialic acid synthesis pathway in DMRV/hIBM model mice with long-term sialic acid treatment2011

    • Author(s)
      Noguchi S, Malicdan MC, Funato F, Nishino I
    • Organizer
      51th American Society of cell Biology
    • Place of Presentation
      アメリカ,デンバーColorado Convention Center
    • Year and Date
      2011-11-04
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Prominent therapeutic potential of peracetylated N-acetylmannosamine, a synthetic sugar molecule, in DMRV/hIBM mice2011

    • Author(s)
      Noguchi S, Malicdan MC, Nishino I
    • Organizer
      Experimental Biology 2011
    • Place of Presentation
      アメリカ,ワシントンWashington Convention Center
    • Year and Date
      2011-04-10
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Lamp-1 overexpression rescues cardiomyopathy in Lamp-2 deficient cells by correcting cellular lysosomal function.2010

    • Author(s)
      Honda S, Noguchi S, Malicdan MC, Hayashi YK, 他
    • Organizer
      15th International Congress of the World Muscle Society(WMS)
    • Place of Presentation
      熊本、崇城大学市民ホール
    • Year and Date
      2010-10-13
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Genetic, clinical, and pathological features of congenital fiber type disproportion in Japan2010

    • Author(s)
      Tominaga K, Hayashi YK, Goto K, Minami N, Noguchi S, Nonaka I, Miki T, Nishino I
    • Organizer
      15th International Congress of the World Muscle Society (WMS)
    • Place of Presentation
      Kumamoto, Japan
    • Year and Date
      2010-10-13
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Lamp-1 overexpression rescues cardiomyopathy in Lamp-2 deficient cells by correcting cellular lysosomal function2010

    • Author(s)
      Honda S, Noguchi S, Malicdan MCV, Hayashi YK, Saftig P, Nishino I
    • Organizer
      15th International Congress of the World Muscle Society (WMS)
    • Place of Presentation
      Kumamoto, Japan
    • Year and Date
      2010-10-13
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] 縁取り空胞を伴う遠位型ミオパチーの治療法開発2009

    • Author(s)
      西野一三, Malicdan MCV, 野口悟
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(神奈川県横浜市)
    • Year and Date
      2009-12-10
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Sialic acid metabolites preclude the development of myopathic phenotype in the DMRV/hIBM mouse model2009

    • Author(s)
      Malicdan MCV, Noguchi S, Hayashi YK, Nonaka I, 他
    • Organizer
      第14回世界筋学会国際会議(WMS)
    • Place of Presentation
      UNI-MAIL(スイス ジュネーブ)
    • Year and Date
      2009-09-09
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Lamp1 overexpression may rescue cardiomyopathy in Lamp2 deficient mice2009

    • Author(s)
      Honda S, Noguchi S, Malicdan MCV, Hayashi YK, Saftig P, Nishino I
    • Organizer
      14th International Congress of the World Muscle Society (WMS)
    • Place of Presentation
      Geneva, Switzerland
    • Year and Date
      2009-09-11
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] 過剰発見によるダノン病治療への試み2009

    • Author(s)
      本田真也, 野口悟, 他
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(神奈川県横浜市)
    • Year and Date
      2009-12-12
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Congenital myotonic dystrophy in patients diagnosed as congenital fiber type disproportion2009

    • Author(s)
      Tominaga K, Hayashi YK, Goto K, Minami N, Noguchi S, Nonaka I, Nishino I
    • Organizer
      14th International Congress of the World Muscle Society (WMS)
    • Place of Presentation
      Geneva, Switzerland
    • Year and Date
      2009-09-11
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] 縁取り空胞を伴う遠位型ミオパチーの治療法開発2009

    • Author(s)
      西野一三,Malicdan MCV,野口悟
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      横浜
    • Year and Date
      2009-12-10
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Sialic acid metabolites preclude the development of myopathic phenotype in the DMRV/hIBM mouse model2009

    • Author(s)
      Malicdan MCV, Noguchi S, Hayashi YK, Nonaka I, Nishino I
    • Organizer
      Congress of the World Muscle Society (WMS)
    • Place of Presentation
      Geneva, Switzerland
    • Year and Date
      2009-09-09
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Lamp1 overexpression may rescue cardiomyopathy in Lamp2 deficient mice2009

    • Author(s)
      Honda S, Noguchi S, Malicdan MCV, 他
    • Organizer
      第14回世界筋学会国際会議(WMS)
    • Place of Presentation
      UNI-MAIL(スイス ジュネーブ)
    • Year and Date
      2009-09-11
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] LAMP1過剰発見によるダノン病治療への試み2009

    • Author(s)
      本田真也,野口悟,Malicdan MCV,林由起子,Saftig P,西野一三
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      横浜
    • Year and Date
      2009-12-12
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Cytoplasmic body with acid phosphatase activity-Hallmark of adult-onset Pompe disease on muscle pathology2008

    • Author(s)
      Nishino I, Oya Y, Monma K, Noguchi S, Hayashi YK, Nonaka I
    • Organizer
      Congress of the World Muscle Society (WMS), Newcastle Upon Tyne
    • Place of Presentation
      United Kingdom
    • Year and Date
      2008-10-01
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Becker型筋ジストロフィーにおける縁取り空胞の出現に関する臨床病理学的検討2008

    • Author(s)
      門間一成,野口悟,林由起子,南成祐,元吉和夫,鎌倉恵子,埜中征哉,西野一三
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Novel ETFDH mutations and normal CoQ10 level in Taiwanese patients with multiple acyl-CoA dehydrogenase deficiency2008

    • Author(s)
      Liang WC, Ohkuma A, Hayashi YK, Lopez LC, Hirano M, Nonaka I, Noguchi S, Jong YJ, Nishino I
    • Organizer
      Congress of the World Muscle Society (WMS), Newcastle Upon Tyne
    • Place of Presentation
      United Kingdom
    • Year and Date
      2008-09-30
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Congenital myotonic dystrophy and myotubular myopathy may be differentiated by type 2C fibers and peripheral halos2008

    • Author(s)
      Fujimura C, Noguchi S, Minami N, Nonaka I, Hayashi YK, Nishino I
    • Organizer
      Congress of the World Muscle Society (WMS), Newcastle Upon Tyne
    • Place of Presentation
      United Kingdom
    • Year and Date
      2008-10-01
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] Clinico-pathological characteristics of the Becker muscle dystrophy with rimmed vacuole2008

    • Author(s)
      Monma K, Noguchi S, Hayashi YK
    • Organizer
      Congress of the World Muscle Society (WMS), Newcastle Upon Tyne
    • Place of Presentation
      United Kingdom
    • Year and Date
      2008-10-01
    • Data Source
      KAKENHI-PROJECT-20390250
  • [Presentation] GNE myopathy: Assessment of an adeno associated virus AAV2/8 based GNE gene delivery system.

    • Author(s)
      Mitrani-Rosenbaum S, Yakovlev L, Becker Cohen M, Rivni O, Harazi A, Noguchi S, Nishino I, Fellig Y, Argov Z
    • Organizer
      19th International Congress of the World Muscle Society,
    • Place of Presentation
      Berlin, Germany (Langenbeck-Virchow-Haus)
    • Year and Date
      2014-10-07 – 2014-10-10
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Metabolic changes in sialic acid synthesis pathway in GNE-myopathy model mice with long-term sialic acid treatment.

    • Author(s)
      Noguchi S, et al.
    • Organizer
      Experimental Biology 2012
    • Place of Presentation
      San Diego Convention Center, San Diego, USA
    • Data Source
      KAKENHI-PROJECT-23390236
  • [Presentation] Activation of mesenchymal progenitor cells in skeletal muscles of Collagen VI deficient mice.

    • Author(s)
      Noguchi S, Ogawa M, Nishino I:
    • Organizer
      19th International Congress of the World Muscle Society
    • Place of Presentation
      Berlin, Germany (福岡国際会議場)
    • Year and Date
      2014-10-07 – 2014-10-11
    • Data Source
      KAKENHI-PROJECT-26293214
  • [Presentation] 6’-シアリル乳糖は高齢発症GNEミオパチーマウスの筋症状を回復する

    • Author(s)
      112.米川貴博,野口 悟,Malicdan MC,林由起子,埜中征哉,峯 利喜,山本 岳,西野一三
    • Organizer
      第55回日本神経学会学術大会
    • Place of Presentation
      福岡 (福岡国際会議場)
    • Year and Date
      2014-05-21 – 2014-05-24
    • Data Source
      KAKENHI-PROJECT-23390236
  • 1.  NISHINO Ichizo (00332388)
    # of Collaborated Projects: 8 results
    # of Collaborated Products: 35 results
  • 2.  HAYASHI Yukiko (50238135)
    # of Collaborated Projects: 6 results
    # of Collaborated Products: 37 results
  • 3.  NONAKA Ikuya (80040210)
    # of Collaborated Projects: 3 results
    # of Collaborated Products: 18 results
  • 4.  OKADA Mari (80328046)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 3 results
  • 5.  NISHIMURA Hiroaki (10399909)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 6.  URUHA Akinori (70635078)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 7.  KUBOTA Takeo (70293511)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 8.  MINAMI Narihiro (20392417)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 9.  金子 加奈子 (00392415)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 10.  西川 敦子 (70737262)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 11.  原 雄二 (60362456)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 12.  HONDA Shinya
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 6 results
  • 13.  TOMINAGA Kayo
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 4 results
  • 14.  MONMA Kazunari
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 3 results
  • 15.  KIYONO Chieko
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 16.  SATO Hidenori
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 17.  SATO Takatoshi
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 18.  ISHIYAMA Teruhiko
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 19.  AMANAKA Kohei
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 20.  GOTO Kanako
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 2 results
  • 21.  山村 隆
    # of Collaborated Projects: 0 results
    # of Collaborated Products: 1 results

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