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2011 Fiscal Year Final Research Report

温度感受性に基づく代謝機能回復因子の網羅的探索と新規治療法の開発

Research Project

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Project/Area Number 21591318
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionGifu University

Principal Investigator

SHIMOZAWA Nobuyuki  岐阜大学, 生命科学総合研究支援センター, 教授 (00240797)

Project Period (FY) 2009 – 2011
Keywords脂質代謝異常 / ペルオキシソーム
Research Abstract

To clarify the molecular mechanism of a temperature-sensitive(TS) phenotype in peroxisomal biogenesis disorders(PBD), we analyzed the change of gene expression in the fibroblasts from PBD patients at 37℃and 30℃, using micro array method. Several hypoxia inducible genes may be possible to associate with TS phenomenon, therefore, we continue further study at a condition of hypoxia. Furthermore, we demonstrated mice fed on high-fat diet(HFD) induced hepatic gene expression of peroxisomal metabolism, which may provide useful fundamental information to understand the role of the peroxisomal function in obesity and lifestyle-related diseases.

  • Research Products

    (29 results)

All 2012 2011 2010 2009 Other

All Journal Article (17 results) (of which Peer Reviewed: 9 results) Presentation (2 results) Book (9 results) Remarks (1 results)

  • [Journal Article] Defective lipid remodeling of GPI anchors in peroxisomal disorders, Zellweger syndrome and rhizomelic chondrodysplasia punctata2012

    • Author(s)
      Noriyuki Kanzawa, Nobuyuki Shimozawa, Ronald J. A. Wanders, Kazutaka Ikeda, Yoshiko Murakami, Hans R. Waterham, Satoru Mukai, Morihisa Fujita, Yusuke Maeda, Ryo Taguchi, Yukio Fujiki, and Taroh Kinoshita
    • Journal Title

      J Lipid Res

      Volume: 53 Pages: 653-63

    • DOI

      doi:10.1194/jlr.M021204

    • Peer Reviewed
  • [Journal Article] Induction of peroxisomal lipid metabolism in mice fed a high-fat diet2011

    • Author(s)
      Kozawa S, Honda A, Kajiwara N, Takemoto Y, Nagase T, Nikami H, Okano Y, Nakashima S, Shimozawa N
    • Journal Title

      Mol Med Report

      Volume: 4 Pages: 1157-1162

    • DOI

      doi:10.3892/mmr.2011.560

    • Peer Reviewed
  • [Journal Article] Clinical biochemical and molecular characterization of peroxisomal diseases in Arabs2011

    • Author(s)
      Shaheen R, Al-Dirbashi OY, Al-Hassnan ZN, Al-Owain M, Makhsheed N, Basheeri F, Seidahmed MZ, Salih MAM, Faqih E, Zaidan H, Al-Sayed M, Rahbeeni Z, Al-Sheddi T, Hashem M, Kurdi W, Shimozawa N, Alkuraya FS
    • Journal Title

      Clin Genet

      Volume: 79 Pages: 60-70

    • DOI

      doi:10.1111/j.1399-0004.2010.01498.x.

    • Peer Reviewed
  • [Journal Article] Identification of novel SNPs of ABCD1, ABCD2, ABCD3, and ABCD4 genes in patients with X-linked adrenoleukodystrophy(ALD) based on comprehensive resequencing and association studies with ALD phenotypes2011

    • Author(s)
      Matsukawa T, Asheuer M, Takahashi Y, Goto J, Suzuki Y, Shimozawa N, Takano H, Onodera O, Nishizawa M, Aubourg P, Tsuji S
    • Journal Title

      Neurogenetics

      Volume: 12 Pages: 41-50

    • DOI

      doi:10.1007/s10048-010-0253-6

    • Peer Reviewed
  • [Journal Article] ABC subfamily D proteins and very long chain fatty acid metabolism as novel targets in adrenoleukodystrophy2011

    • Author(s)
      Morita M, Shimozawa N, Kashiwayama Y, Suzuki Y, Imanaka T
    • Journal Title

      Current Drug Targets

      Volume: 12 Pages: 694-706

    • Peer Reviewed
  • [Journal Article] X-linked adrenoleukodystrophy : Diagnostic and follow-up system in Japan2011

    • Author(s)
      Shimozawa N, Honda A, Kajiwara N, Kozawa S, Nagase T, Takemoto Y, Suzuki Y
    • Journal Title

      J Hum Genet

      Volume: 56 Pages: 106-109

    • DOI

      doi:10.1038/jhg.2010.139

    • Peer Reviewed
  • [Journal Article] Molecular and clinical findings and diagnostic flowchart of peroxisomal disease2011

    • Author(s)
      Shimozawa N
    • Journal Title

      Brain Dev

      Volume: 33 Pages: 770-776

    • DOI

      doi:10.1016/j.braindev.2011.03.004.

    • Peer Reviewed
  • [Journal Article] Clinical aspects and adrenal functions in eleven Japanese children with X-linked adrenoleukodystrophy2010

    • Author(s)
      Miyoshi Y, Sakai N, Hamada Y, Tachibana M, Hasegawa Y, Kiyohara Y, Yamada H, Murakami M, Kondou H, Kimura-Ohba S, Mine J, Sato T, Kamio N, Ueda H, Suzuki Y, Shiomi M, Ohta H, Shimozawa N, Ozono K
    • Journal Title

      Endocr J

      Volume: 57 Pages: 965-972

    • DOI

      doi:10.1507/endocrj.K10E-204

    • Peer Reviewed
  • [Journal Article] ペルオキシソーム形成異常症-Zellweger症候群-,別冊日本臨床新領域別症候群シリーズNo132010

    • Author(s)
      下澤伸行
    • Journal Title

      肝・胆道系症候群(第2版)

      Pages: 515-518

  • [Journal Article] 日本先天代謝異常学会学会賞受賞論文「ペルオキシソーム病との30年:二人三脚の旅」2010

    • Author(s)
      鈴木康之, 下澤伸行
    • Journal Title

      日本先天代謝異常学会雑誌

      Volume: 26巻 Pages: 2-12

  • [Journal Article] ペルオキシソーム病を見逃していませんか?2010

    • Author(s)
      下澤伸行
    • Journal Title

      小児内科

      Volume: 42巻 Pages: 1167-1173

  • [Journal Article] ペルオキシソーム病(副腎白質ジストロフィー, Zellweger症候群)2010

    • Author(s)
      下澤伸行
    • Journal Title

      小児科診療

      Pages: 515-517

  • [Journal Article] Zellweger syndrome caused by PEX13 deficiency : Report of two novel mutations2009

    • Author(s)
      Al-Dirbashi OY, Shaheen R, Al-Sayed M, Al-Dosari M, Makhseed N, Safieh LA, Santa T, Meyer BF, Shimozawa N, Alkuraya FS
    • Journal Title

      Am J Med Genet

      Volume: 149A Pages: 1219-1223

    • DOI

      DOI:10.1002/ajmg.a.32874

    • Peer Reviewed
  • [Journal Article] ペルオキシソーム病2009

    • Author(s)
      下澤伸行
    • Journal Title

      小児内科小児疾患診療のための病態生理2

      Volume: 41巻 Pages: 479-486

  • [Journal Article] 日本人が発見に関わった疾患遺伝子ペルオキシソーム病2009

    • Author(s)
      下澤伸行
    • Journal Title

      小児科特集「小児疾患における臨床遺伝学の進歩」

      Volume: 50巻 Pages: 907-913

  • [Journal Article] 同一遺伝子異常を持ちながら臨床型が異なるALD兄弟例2009

    • Author(s)
      福原忍, 水江伸夫, 坂井拓郎, 稲澤奈津子, 池本亘, 足立憲昭, 下澤伸行
    • Journal Title

      小児科臨床

      Volume: 62巻 Pages: 457-461

  • [Journal Article] ペルオキシソーム代謝異常症

    • Author(s)
      下澤伸行
    • Journal Title

      内分泌・糖尿病・代謝内科

      Volume: 34(3) Pages: 198-203

  • [Presentation] International Symposium on Epilepsy in Neurometabolic Diseases. 2010. 3 Taipei2010

    • Author(s)
      Shimozawa N
    • Organizer
      Clinical findings and diagnostic flowchart of peroxisomal disease
    • Place of Presentation
      Plenary Lecture
    • Year and Date
      20100300
  • [Presentation] 第51回日本先天代謝異常学会2009

    • Author(s)
      鈴木康之、下澤伸行
    • Organizer
      第8回アジア先天代謝異常症シンポジウム
    • Place of Presentation
      東京
    • Year and Date
      20091100
  • [Book] RCDP type1.先天代謝異常症Diagnosis at a Glance日本先天代謝異常学会編2011

    • Author(s)
      下澤伸行
    • Total Pages
      152-153
    • Publisher
      診断と治療社
  • [Book] 副腎白質ジストロフィー.先天代謝異常症Diagnosis at a Glance日本先天代謝異常学会編2011

    • Author(s)
      鈴木康之、小関道夫、下澤伸行
    • Total Pages
      149-151
    • Publisher
      診断と治療社
  • [Book] Zellweger症候群.先天代謝異常症Diagnosis at a Glance日本先天代謝異常学会編2011

    • Author(s)
      下澤伸行
    • Total Pages
      146-148
    • Publisher
      診断と治療社
  • [Book] 脳肝腎症候群(ツェルウェーガー症候群):症候群ハンドブック2011

    • Author(s)
      下澤伸行
    • Total Pages
      126-127
    • Publisher
      中山書店
  • [Book] ペルオキシソーム病:小児科臨床ピクシス23見逃せない先天代謝異常2010

    • Author(s)
      下澤伸行
    • Total Pages
      214-217
    • Publisher
      中山書店
  • [Book] 極長鎖脂肪酸測定:小児科臨床ピクシス23見逃せない先天代謝異常2010

    • Author(s)
      下澤伸行
    • Total Pages
      112-115
    • Publisher
      中山書店
  • [Book] 出生時からの著明な筋緊張低下,哺乳不良に特異な顔貌を認めた生後2ヶ月男児:日本先天代謝異常学会編.症例から学ぶ先天代謝異常症2010

    • Author(s)
      下澤伸行,鈴木康之,折居忠夫
    • Total Pages
      112-115
    • Publisher
      診断と治療社
  • [Book] 成績低下,行動異常から心療内科を受診していた11歳男児:日本先天代謝異常学会編.症例から学ぶ先天代謝異常症2009

    • Author(s)
      菊地正広,下澤伸行
    • Total Pages
      216-219
    • Publisher
      診断と治療社
  • [Book] ペルオキシソーム病:日本先天代謝異常学会編. Meet the Expert症例から学ぶ先天代謝異常症2009

    • Author(s)
      下澤伸行
    • Total Pages
      212-215
    • Publisher
      診断と治療社
  • [Remarks] ペルオキシソーム病ホームページ

    • URL

      http://www1.gifu-u.ac.jp/~lsrc/dgr/shimozawa-hp/

URL: 

Published: 2013-07-31  

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