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Neurochemistry of mouse model investigated with 7T MR spectroscopy

Research Project

Project/Area Number 24591790
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Radiation science
Research InstitutionTokyo Women's Medical University (2014-2015)
Toho University (2012-2013)

Principal Investigator

Takanashi Jun-ichi  東京女子医科大学, 医学部, 教授 (00302555)

Co-Investigator(Kenkyū-buntansha) Terada Hitoshi  東邦大学, 医学部, 教授 (90227520)
Co-Investigator(Renkei-kenkyūsha) Aoki Ichio  国立研究開発法人放射線医学総合研究所, 分子イメージング研究センター, チームリーダー (10319519)
Project Period (FY) 2012-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2015: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2014: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2013: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2012: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
KeywordsMRスペクトルスコピー / 核磁気共鳴画像(MRI) / 先天性大脳白質形成不全症 / Pelizaeus-Merzbacher 病 / N-acetylaspartate (NAA) / choline (Cho) / モデルマウス / Pelizaeus-Merzbacher病 / Choline / マウスモデル / 副腎白質ジストロフィー / 18q-症候群 / 大脳白質形成不全症 / 磁気共鳴画像 / MRスペクトルスコピー / 脳代謝 / 髄鞘形成不全
Outline of Final Research Achievements

Proton MR spectroscopy (MRS) of a hypomyelinating mouse model, a myelin synthesis-deficient mouse, a model of connatal Pelizaeus-Merzbacher disease (PMD) with mutation of the Plp1 gene, revealed increased N-acetylaspartate (NAA) and creatine (Cr) and decreased choline (Cho). That of a shiverer mouse with an autosomal recessive mutation of the Mbp gene showed decreased Cho with normal NAA and Cr. Accordingly, the reduction of Cho on MRS might be a common marker for hypomyelinating disorders. NAA concentrations range from normal to increased, probably depending upon the underlying pathology of oligodendrocytes. NAA may be increased in hypomyelination with a reduced number of mature oligodendrocytes, such as PMD.
The MRS pattern with reduced Cho and normal to increased NAA may lead to a diagnosis of hypomyelinating disorders, which is difficult to differentiate from demyelinating disorders on MR imaging.

Report

(5 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (52 results)

All 2016 2015 2014 2013 2012 Other

All Journal Article (23 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 19 results,  Open Access: 2 results,  Acknowledgement Compliant: 3 results) Presentation (20 results) (of which Int'l Joint Research: 2 results,  Invited: 18 results) Book (5 results) Remarks (4 results)

  • [Journal Article] The magnetic resonance imaging spectrum of Pelizaeus-Merzbacher disease: A multicenter study of 19 patients.2016

    • Author(s)
      Sumida K, Inoue K, Takanashi JI, Sasaki M, Watanabe K, Suzuki M, Kurahashi H, Omata T, Tanaka M, Yokochi K, Iio J, Iyoda K, Kurokawa T, Matsuo M, Sato T, Iwaki A, Osaka H, Kurosawa K, Yamamoto T, Matsumoto N, Maikusa N, Mastuda H, Sato N
    • Journal Title

      Brain Dev

      Volume: in press Issue: 6 Pages: 571-80

    • DOI

      10.1016/j.braindev.2015.12.007

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Drugs indicated for mitochondrial dysfunction as treatments for acute encephalopathy with onset of febrile convulsive status epileptics2016

    • Author(s)
      Omata T, Fujii K, Takanashi J, Murayama K, Takayanagi M, Muta K, Kodama K, Iida Y, Watanabe Y, Shimojo N.
    • Journal Title

      J Neurol Sci

      Volume: 360 Pages: 57-60

    • DOI

      10.1016/j.jns.2015.11.043

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Neurochemistry of Hypomyelination Investigated with MR Spectroscopy2015

    • Author(s)
      Takanashi J.
    • Journal Title

      Magnetic Resonance in Medical Sciences

      Volume: 14 Issue: 2 Pages: 85-91

    • DOI

      10.2463/mrms.2014-0064

    • NAID

      130005069922

    • ISSN
      1347-3182, 1880-2206
    • Related Report
      2015 Annual Research Report 2014 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Disrupted glutamate-glutamine cycle in acute encephalopathy with biphasic seizures and late reduced diffusion.2015

    • Author(s)
      Takanashi JI, Mizuguchi M, Terai M, Barkovich AJ.
    • Journal Title

      Neuroradiology

      Volume: 57 Issue: 11 Pages: 1163-1168

    • DOI

      10.1007/s00234-015-1573-x

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Predictive score for early diagnosis of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD).2015

    • Author(s)
      Tada H, Takanashi J, Okuno H, Kubota M, Yamagata T, Kawano G, Shiihara T, Hamano S, Hirose S, Hayashi T, Osaka H, Mizuguchi M.
    • Journal Title

      NEUROL SCI.

      Volume: 358(1-2) Issue: 1-2 Pages: 62-5

    • DOI

      10.1016/j.jns.2015.08.016

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Diffusion-weighted MRI for early diagnosis of neonatal herpes simplex encephalitis.2015

    • Author(s)
      Okanashi T, Yamamoto H, Hosokawa T, Ando N, Nagayama Y, Hashimoto Y, Maihara T, Goto T, Kutota M, Kawaguchi C, Yoshida H, Sugiura K, Itomi S, Ohno K, Takanashi J, Hayakawa M, Otsubo H, Okumura A.
    • Journal Title

      Brain Dev

      Volume: 37 Issue: 4 Pages: 423-431

    • DOI

      10.1016/j.braindev.2014.07.006

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Journal Article] Clinical and radiologic features of encephalopathy during 2011 E coli O111 outbreak in Japan.2014

    • Author(s)
      Takanashi JI, Taneichi H, Misaki T, Yahata Y, Okumura A, Ishida YI, Miyawaki T, Okabe N, Sata T, Mizuguchi M.
    • Journal Title

      Neurology

      Volume: 82 Issue: 7 Pages: 564-572

    • DOI

      10.1212/wnl.0000000000000120

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Journal Article] Expanding the phenotypic spectrum of TUBB4A-associated2014

    • Author(s)
      Miyatake S, Osaka H, Shiina M, Sasaki M, Takanashi J, Haginoya K, Wada T,
    • Journal Title

      Neurology

      Volume: 82(24) Issue: 24 Pages: 2230-7

    • DOI

      10.1212/wnl.0000000000000535

    • Related Report
      2014 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] けいれん・意識障害 画像検査の選択と解釈2014

    • Author(s)
      髙梨潤一
    • Journal Title

      小児内科

      Volume: 46 Pages: 1152-1156

    • Related Report
      2014 Research-status Report
  • [Journal Article] 神経画像検査 (1) MRI, CT.2014

    • Author(s)
      髙梨潤一
    • Journal Title

      日本臨床

      Volume: 72 Pages: 819-826

    • Related Report
      2014 Research-status Report
  • [Journal Article] Neurochemistry in Shiverer mouse depicted on MR spectroscopy.2014

    • Author(s)
      Takanashi J, Nitta N, Iwasaki N, saito S, Tanaka R, Aoki I.
    • Journal Title

      J Magn Reson Imaging

      Volume: 39 Issue: 6 Pages: 1550-1557

    • DOI

      10.1002/jmri.24306

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Epidemiological, clinical, and genetic landscapes of hypomyelinating leukodystrophies.2014

    • Author(s)
      Numata Y, Gotoh L, Iwaki A, Kurosawa K, Takanashi J, Deguchi K, Yamamoto T, Osaka H, Inoue K.
    • Journal Title

      J Neurol.

      Volume: 261(4) Issue: 4 Pages: 752-758

    • DOI

      10.1007/s00415-014-7263-5

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] MR spectroscopy in 18q- syndrome suggesting other than hypomyelination.2014

    • Author(s)
      Tada H, Takanashi J.
    • Journal Title

      Brain Dev

      Volume: 36 Issue: 1 Pages: 57-60

    • DOI

      10.1016/j.braindev.2012.12.003

    • Related Report
      2013 Research-status Report 2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Intragenic mutations in SMN1may contribute more significantly to clinical severity than SMN2 copy numbers in some spinal muscular atrophy (SMA) patients2014

    • Author(s)
      Yamamoto T, Sato H, Lai PS, Nurputra DK, Harahap NIF, Morikawa S, Nishimura N, Kurashige T, Tomohiko O, Nakajima H, Yamada H, Nishida Y, Toda S, Takanashi J, Takeuchi A, Tohyama Y, Kubo Y, Saito K, Takeshima Y, Matsuo M, Nishio H
    • Journal Title

      Brain Dev

      Volume: 未 Issue: 10 Pages: 914-920

    • DOI

      10.1016/j.braindev.2013.11.009

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] C5orf42 is the major gene responsible for OFD syndrome type VI.2014

    • Author(s)
      Lopez E, Thauvin-Robinet C, Reversade B, Khartoufi NE, Devisme L, Holder M, Ansart-Franquet H, Avila M, Lacombe D, Kleinfinger P, Kaori I, Takanashi JI, Le Merrer M, Martinovic J, Noël C, Shboul M, Ho L, Güven Y, Razavi F, Burglen L, Faivre L, Attié-Bitach T
    • Journal Title

      Hum Genet

      Volume: 未 Issue: 3 Pages: 367-377

    • DOI

      10.1007/s00439-013-1385-1

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Different patterns of cerebellar abnormality and hypomyelination between POLR3A and POLR3B mutations.2013

    • Author(s)
      98. Takanashi JI, Osaka H, Saitsu H, Sasaki M, Mori H, Shibayama H, Tanaka M,
    • Journal Title

      Brain Dev.

      Volume: May 3 Issue: 3 Pages: 259-263

    • DOI

      10.1016/j.braindev.2013.03.006

    • Related Report
      2013 Research-status Report 2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly2013

    • Author(s)
      Yoneda Y, Haginoya K,…..*Saitsu H
    • Journal Title

      Ann Neurol

      Volume: 73(1):48-57 Issue: 1 Pages: 48-57

    • DOI

      10.1002/ana.23736

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 先天性大脳白質形成不全症―ゲノム解析から診断、治療への取り組み2013

    • Author(s)
      井上健、岩城明子、黒澤健司、髙梨潤一、出口貴美子、山本俊至、小坂仁
    • Journal Title

      脳と発達

      Volume: 45 Pages: 122-126

    • Related Report
      2013 Research-status Report
  • [Journal Article] Clinical value of routine use of thin-section 3D MRI using 3D FSE sequences with a variable flip angle technique for internal derangements of the knee joint at 3T.2013

    • Author(s)
      Kudo H, Inaoka T, Kitamura N, Nakatsuka T, Kasuya S, Kasai R, Tozawa M, Nakagawa K, Terada H.
    • Journal Title

      Magn Reson Imaging

      Volume: 31 Issue: 8 Pages: 1309-1317

    • DOI

      10.1016/j.mri.2013.02.003

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] MRI, MR spectroscopyによる先天性大脳白質形成不全症の診断、病態解明2013

    • Author(s)
      高梨潤一
    • Journal Title

      脳と発達

      Volume: 45 Pages: 132-136

    • NAID

      10031156275

    • Related Report
      2012 Research-status Report
  • [Journal Article] Metabolic changes in early childhood using LCModel with corrected water scaling method.2012

    • Author(s)
      Takanashi J, Somazawa F, Maruyama K,Terada H, Xu D, A. Barkovich AJ.
    • Journal Title

      J Magn Reson Imaging

      Volume: 35 Issue: 1 Pages: 174-180

    • DOI

      10.1002/jmri.22802

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Brain N-acetylaspartate is increased in mice with hypomyelination2012

    • Author(s)
      Takanashi J, Saito S, Aoki I, Barkovich J, Itoh Y, Inoue K.
    • Journal Title

      J Magn Reson Imaging

      Volume: 35 Issue: 2 Pages: 418-425

    • DOI

      10.1002/jmri.22817

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Hypoyelination in I-cell disease; MRI, MR spectroscopy and neuropathological correlation.2012

    • Author(s)
      Takanashi J, Hayashi M, Yuasa S, Satoh H, Terada H.
    • Journal Title

      Brain Dev

      Volume: 34 Issue: 9 Pages: 780-783

    • DOI

      10.1016/j.braindev.2011.12.013

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Presentation] Neuroimaging in acute encephalopathy with biphasic seizures and late reduced diffusion (AESD)2015

    • Author(s)
      高梨潤一
    • Organizer
      10th Asian Oceanian Congress of Neuroradiology (AOCNR)
    • Place of Presentation
      Hilton Fukuoka Sea Hawk(福岡県福岡市)
    • Year and Date
      2015-11-05
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] MR spectroscopyで診る脳病態2015

    • Author(s)
      高梨潤一
    • Organizer
      第8回Neuro-imaging Refresher Club
    • Place of Presentation
      東京コンファレンスセンター・品川(東京都品川区)
    • Year and Date
      2015-11-01
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] MRで診る脳代謝:MRスペクトルスコピーのあれこれ2015

    • Author(s)
      高梨潤一
    • Organizer
      第56回神奈川小児神経懇話会
    • Place of Presentation
      ホテル横浜キャメロットジャパン(神奈川県横浜市)
    • Year and Date
      2015-07-25
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 臨床から迫る白質変性症2015

    • Author(s)
      高梨潤一
    • Organizer
      第57回日本小児神経学会学術集会
    • Place of Presentation
      帝国ホテル大阪(大阪府大阪市)
    • Year and Date
      2015-05-27
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] Neurochenistry in hypomyelination on MR spectroscopy2015

    • Author(s)
      高梨潤一
    • Organizer
      第56回日本神経学会学術大会
    • Place of Presentation
      新潟コンベンションセンター(新潟県新潟市)
    • Year and Date
      2015-05-20
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] Neuroimaging findings in children with acute encephalopathy2015

    • Author(s)
      高梨潤一
    • Organizer
      13th Asian Oceanian Congress of Child Neurology (AOCCN)
    • Place of Presentation
      台北(台湾)
    • Year and Date
      2015-05-13
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] MR spectroscopyを用いた神経疾患モデルマウス脳代謝解析2015

    • Author(s)
      高梨潤一
    • Organizer
      第3回 日本生物物理学会九州支部・熊本大学イメージングセミナー
    • Place of Presentation
      熊本大学(熊本県熊本市)
    • Year and Date
      2015-05-07
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 小児の白質病変2014

    • Author(s)
      髙梨潤一
    • Organizer
      第7回 Neuro-Imaging Refresher Club
    • Place of Presentation
      東京コンファレンスセンター・品川(東京都)
    • Year and Date
      2014-11-23
    • Related Report
      2014 Research-status Report
    • Invited
  • [Presentation] 小児神経疾患における proton MR spectroscopy の臨床応用2014

    • Author(s)
      髙梨潤一
    • Organizer
      第42回日本磁気共鳴医学会総会
    • Place of Presentation
      ホテルグランビア京都(京都市)
    • Year and Date
      2014-09-18 – 2014-09-20
    • Related Report
      2014 Research-status Report
    • Invited
  • [Presentation] Neurochemistry in hypomyelination depicted on MR spectroscopy.2014

    • Author(s)
      Takanashi J, Nitta N, Terada H, Barkovich AJ, Tada H, Aoki I.
    • Organizer
      20th Symposium Neuroradiologicum
    • Place of Presentation
      イスタンブール(トルコ)
    • Year and Date
      2014-09-07 – 2014-09-12
    • Related Report
      2014 Research-status Report
  • [Presentation] Proton MR spectroscopy in neurological disorders.2014

    • Author(s)
      髙梨潤一
    • Organizer
      第50回日本小児放射線学会学術集会
    • Place of Presentation
      神戸国際会議場(神戸市)
    • Year and Date
      2014-06-27 – 2014-06-28
    • Related Report
      2014 Research-status Report
    • Invited
  • [Presentation] How to read/interplete neuroimagings?2014

    • Author(s)
      髙梨潤一
    • Organizer
      第56回日本小児神経学会学術集会
    • Place of Presentation
      アクトシティー浜松(浜松市)
    • Year and Date
      2014-05-28 – 2014-05-31
    • Related Report
      2014 Research-status Report
    • Invited
  • [Presentation] 小児神経放射線 Topics

    • Author(s)
      高梨潤一
    • Organizer
      第55回日本小児神経学会学術集会
    • Place of Presentation
      大分オアシスタワーホテル(大分市)
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] 白質病変の新しい病態解析とMRマーカー:hypomyelinationのMRを用いたneurochemistry.

    • Author(s)
      高梨潤一
    • Organizer
      第41回日本磁気共鳴医学会大会
    • Place of Presentation
      アスティーとくしま(徳島市)
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] Clinical keys for the diagnosis of pediatrics leukodystrophy

    • Author(s)
      高梨潤一
    • Organizer
      第73回日本医学放射線学会
    • Place of Presentation
      パシフィコ横浜(横浜市)
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] 小児神経疾患の画像診断 up to date

    • Author(s)
      高梨潤一
    • Organizer
      第46回新潟小児神経研究会
    • Place of Presentation
      コープシシティー花岡ガレッソホール(新潟市)
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] MRI, MR spectroscopyによる先天性大脳白質形成不全症の診断、病態解明

    • Author(s)
      高梨潤一
    • Organizer
      第54回日本小児神経学会
    • Place of Presentation
      ホテルロイトン札幌(北海道)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] H-MRS in neurodegeneration

    • Author(s)
      Takanashi J
    • Organizer
      12th International Child Neurology Congress
    • Place of Presentation
      Brisbane Convention & Exhibition Center(ブリスベン・オーストラリア))
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] シバラーマウスにおけるMR spectroscopyによる脳代謝解析

    • Author(s)
      高梨潤一、岩崎信明、齋藤茂芳、新田展大、青木伊知男、多田弘子、寺田一志
    • Organizer
      第40回日本磁気共鳴医学会
    • Place of Presentation
      国立京都国際会館(京都府)
    • Related Report
      2012 Research-status Report
  • [Presentation] 髄鞘低形成のMR spectroscopy を用いた neurochemistry

    • Author(s)
      高梨潤一
    • Organizer
      第41回日本磁気共鳴医学会
    • Place of Presentation
      アスティ徳島(徳島県)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Book] 続・イメージからせまる小児神経疾患2015

    • Author(s)
      高梨潤一
    • Total Pages
      119
    • Publisher
      診断と治療社
    • Related Report
      2015 Annual Research Report
  • [Book] 疾患から見る画像診断の進め方・読み方2015

    • Author(s)
      高梨潤一
    • Total Pages
      348
    • Publisher
      診断と治療社
    • Related Report
      2015 Annual Research Report
  • [Book] Proton MRS の臨床有用性コンセンサスガイド 2013年度版2013

    • Author(s)
      高梨潤一(日本磁気共鳴医学会 Proton MRS の臨床有用性検討会編集)
    • Total Pages
      81
    • Publisher
      日本磁気共鳴医学会
    • Related Report
      2013 Research-status Report
  • [Book] これでわかる拡散MRI 第3版2013

    • Author(s)
      高梨潤一(青木茂樹、阿部修、増谷佳孝、高原太郎 編集)
    • Total Pages
      487
    • Publisher
      秀潤社
    • Related Report
      2013 Research-status Report
  • [Book] 3-2-6 小児脳、発達(磁気共鳴スペクトルの医学応用)2013

    • Author(s)
      髙梨潤一(成瀬昭二、梅田雅宏、原田雅史、田中忠蔵編)
    • Publisher
      インナービジョン
    • Related Report
      2012 Research-status Report
  • [Remarks] 先天性大脳白質形成不全症:PMDと類縁疾患に関するネットワーク

    • URL

      http://plaza.umin.ac.jp/~pmd/

    • Related Report
      2015 Annual Research Report
  • [Remarks] 遺伝性白質疾患の診断・治療・研究システムの構築班

    • URL

      http://plaza.umin.ac.jp/~pmd/iden_introduction.html

    • Related Report
      2015 Annual Research Report
  • [Remarks] 先天性大脳白質形成不全症:PMDと類縁疾患に関するネットワーク

    • URL

      http://kcmc.jp/pmd/about.html

    • Related Report
      2014 Research-status Report
  • [Remarks] 先天性大脳白質形成不全症:PMDと類縁疾患に関するネットワーク

    • URL

      http://kcmc.jp/pmd/about.html

    • Related Report
      2013 Research-status Report

URL: 

Published: 2013-05-31   Modified: 2019-07-29  

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