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2007 Fiscal Year Final Research Report Summary

The Effect of Cell Fusion with the Host Brain Cells on the Neuronal Differentiation of Transplanted Mesenchymal Stem Cells in the Brain

Research Project

Project/Area Number 18591163
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionOsaka City University

Principal Investigator

TANAKA Akemi  Osaka City University, Graduate School of Medicine, Associate Professor (30145776)

Co-Investigator(Kenkyū-buntansha) MAEDA Mitsuyo  Osaka City University, Graduate School of Medicine, Fellow (40122080)
SETO Toshiyuki  Osaka City University, Graduate School of Medicine, Fellow (60423878)
Project Period (FY) 2006 – 2007
KeywordsInborn errors of metabolism / hereditary neurodegenerative disorders / regeneration therapy / transplantation / neurogenesis / differentiation / GM1-gangliosidosis / β-galactosidase
Research Abstract

Lysosomal storage disease (LSD) is a group of genetic diseases caused by deficiency of an enzyme for the catabolism in lysosome. Most of them show systemic accumulation of undigested substrates in lysosomes, which cause progressive diseases including the brain. Bone marrow transplantation and enzyme replacement therapy are clinically available as effective therapy for some LSDs. But the brain involvement is an exception. We transplanted fetal brain cells, cultured neuronal cells, or bone marrow derived mesenchymal stem cells into the ventricle of neonatal mouse brain to deliver the deficient enzyme and protect the brain from the disease, and studied the migrating area of the grafted cells and their viable period. β-Galactosidase knock-out mouse (GM1-gangliosidosis model mouse) and transgenic mouse expressing human β-galactosidase were used as the recipient and the donor, respectively. The cells of 0.5-1.0×10^5 were injected into the right-side ventricle on the 2nd day after birth. The treated mice were examined histologically by B-galactosidase activity staining (X-Gal) to see the grafted cell distribution, and ganglioside-GM1 immunostaining to examine the therapeutic effect. The injected cells were grafted on the surface of the right-side of ventricle one week after the injection. They migrated into the deep area of both hemisphere, and the number of X-Gal positive cells became 10-20 fold at two weeks after the injection. Intracerebral transplantation of some special cells would have a possibility of permanent cure of the brain in LSDs.

  • Research Products

    (22 results)

All 2008 2007 2006

All Journal Article (9 results) (of which Peer Reviewed: 5 results) Presentation (11 results) Book (2 results)

  • [Journal Article] Fluorescence in situ hybridization analysis is useful for the diagnosis of the carrier state of X-linked ichthyosis.2008

    • Author(s)
      Hosomi, N.Fukai, K.Tanaka, A.Fujita, H.Ishii, M.
    • Journal Title

      International Journal of Dermatology 47

      Pages: 529-530

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] Fluorescence in situ hybridization analysis is useful for the diagnosis of the carrier state of X-linked ichthyosis2008

    • Author(s)
      Hosomi, N, Fukai, K, Tanaka, A, Fujita, H, Ishii, M
    • Journal Title

      International Journal of Dermatology 47(in press)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] ムコ多糖症治療法の進歩2007

    • Author(s)
      田中 あけみ
    • Journal Title

      日本小児科学会雑誌 11

      Pages: 1255-1262

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] ガングリオシドーシス2007

    • Author(s)
      田中 あけみ
    • Journal Title

      小児内科増刊号「小児中枢神経疾患の画像診断」 36

      Pages: 488-492

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Targeted gene therapy toward astrocytoma using a Cre/loxP-based adenovirus system2006

    • Author(s)
      Maeda M, Namikawa K, Kobayashi I, Ohba N, Takehara Y, Kadono C, Tanaka A, Kiyama H
    • Journal Title

      Brain Research 1081

      Pages: 34-43

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] Sanfilippo症候群B型とWilson病を合併した1男児例2006

    • Author(s)
      高浦 奈津子、田中 あけみ、吉田 敏子、竹下 由紀子、清水 教一、青木 継稔、玉井 浩、山野 恒一
    • Journal Title

      脳と発達 38

      Pages: 49-53

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] Hurler病の1症例における酵素補充療法の効果2006

    • Author(s)
      田中 あけみ、澤田 智、藤岡 弘季、山野 恒一
    • Journal Title

      日本小児科学会雑誌 110

      Pages: 945-950

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] ジメチルメチレンブルー呈色反応による尿のムコ多糖症スクリーニング法:見逃し症例に基づく反省と方法の再検討2006

    • Author(s)
      坂口 知子(梶田 知子)、田中 あけみ、山野 恒一
    • Journal Title

      日本マススクリーニング学会誌 16

      Pages: 87-91

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] Targeted gene therapy toward astrocytoma using a Cre/loxP-based adenovirus system2006

    • Author(s)
      Maeda, M, Namikawa, K, Kobayashi, I, Ohba, N, Takehara, Y, Kadono, C, Tanaka, A, Kiyama, H
    • Journal Title

      Brain Res. 1081

      Pages: 34-43

    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] Efficacy of bone marrow/hematopoietic stem cell transplantation in mucopoly-saccharidosis type II:A historical cohort study and clinical experience.2007

    • Author(s)
      Tanaka, A
    • Organizer
      International Symposium of Lysosomal Storage Diseases
    • Place of Presentation
      千葉
    • Year and Date
      20071129-1201
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] Efficacy of bone marrow/hematopoietic stem cell transplantation in mucopolysaccharidosis type II: A historical cohort study and clinical experience2007

    • Author(s)
      Tanaka, A
    • Organizer
      International Symposium of Lysosomal Storage Diseases
    • Place of Presentation
      Chiba
    • Year and Date
      20071129-1201
    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] A limit of the efficacy of enzyme replacement therapy: A study of an autopsy case with mucopolysaccharidosis type I2007

    • Author(s)
      Tanaka, A, Sawada, T, Aso, K, Wanibuchi, H, Tsuruhara, A, Yamano, T
    • Organizer
      International Symposium of Lysosomal Storage Diseases
    • Place of Presentation
      Chiba
    • Year and Date
      20071129-1201
    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] 細胞移植によるライソゾーム病脳病変の長期治療の可能性についての検討2007

    • Author(s)
      澤田 智、田申 あけみ、瀬戸 俊之、前田 光代、高村 歩美、檜垣 克美、難波 栄二、松田 潤一郎、山口 悦子、山野 恒一
    • Organizer
      第49回日本先天代謝異常学会
    • Place of Presentation
      山形
    • Year and Date
      20071115-17
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] ムコ多糖症II型とIII型における知能障害の発生機序と造血幹細胞移植の知能障害への高価に関する研究2007

    • Author(s)
      田中 あけみ、澤田 智、鈴木 康之、奥山 虎之、山野 恒一
    • Organizer
      第49回日本先天代謝異常学会
    • Place of Presentation
      山形
    • Year and Date
      20071115-17
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] ライソゾーム病の脳病変に対する細胞治療2007

    • Author(s)
      澤田 智、田中 あけみ、松田 潤一郎、難波 栄二、山野 恒一
    • Organizer
      第49回日本小児神経学会
    • Place of Presentation
      大阪
    • Year and Date
      20070705-07
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] Blood or urine?: as a material for screening of mucopolysaccharidoses2006

    • Author(s)
      Tanaka, A, Sakaguchi, T, Morita, A, Yamano, T
    • Organizer
      The 6th Meeting of the International Society for Neonatal Screening
    • Place of Presentation
      Yumebutai, Japan
    • Year and Date
      20060916-19
    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] Cell therapy for the brain involvement in lysosomal storage disease.2006

    • Author(s)
      Sawada T, Tanaka A, Seto T, Maeda M, Jikihara I, Yamaguchi E, Matsuda J, Nanba E, Yamano T
    • Organizer
      The 10th International Congress of Inborn Errors of Metabolism
    • Place of Presentation
      千葉
    • Year and Date
      20060912-16
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] Cell therapy for the brain involvement in lysosomal storage disease2006

    • Author(s)
      Sawada, T, Tanaka, A, Seto, T, Maeda, M, Jikihara, I, Yamaguchi, E, Matsuda, J, Nanba, E, Yamano, T
    • Organizer
      The 10th International Congress of Inborn Errors of Metabolism
    • Place of Presentation
      Makuhari, Japan
    • Year and Date
      20060912-16
    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] Efficacy estimation of bone marrow transplantation on the brain in mucopolysaccharidosis type II: a comparative study with natural histories2006

    • Author(s)
      Tanaka, A, Sawada, T, Seto, T, Yamano, T
    • Organizer
      The 10th International Congress of Inborn Errors of Metabolism
    • Place of Presentation
      Makuhari, Japan
    • Year and Date
      20060912-16
    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] Efficacies of enzyme replacement therapy in a Japanese patient with Hurler disease2006

    • Author(s)
      Sawada, T, Tanaka, A, Fujioka, H, Asada, M, Seto, T, Yamano, T
    • Organizer
      The 9th International Symposium on MPS and Related Diseases
    • Place of Presentation
      Venice, Italy
    • Year and Date
      20060629-0702
    • Description
      「研究成果報告書概要(欧文)」より
  • [Book] EBM小児疾患の治療2006

    • Author(s)
      田中 あけみ(五十 嵐隆, 他編集)
    • Total Pages
      708
    • Publisher
      中外医学社
    • Description
      「研究成果報告書概要(和文)」より
  • [Book] 新領域別症候群シリーズ No.12006

    • Author(s)
      田中 あけみ, 他
    • Total Pages
      759
    • Publisher
      日本臨床社
    • Description
      「研究成果報告書概要(和文)」より

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Published: 2010-02-04  

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