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Amyotrophic lateral sclerosis : on the decreased transcription activity of ribosomal RNA gene and cell degeneration

Research Project

Project/Area Number 14580735
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionTokyo Metropolitan Organization for Medical Research

Principal Investigator

OYANAGI Kiyomitsu  Tokyo Met.Org.Med.Res., Tokyo Met.Inst.Neurosci., Director, 東京都神経科学総合研究所, 参事研究員 (00134958)

Co-Investigator(Kenkyū-buntansha) KANDA Naotoshi  Tokyo Univ.of Agricul. & Tech., Professor, 農学部, 教授 (40075429)
WATABE Kazuhiko  Tokyo Met.Org.Med.Res., Tokyo Met.Inst.Neurosci., Scientist, 副参事研究員 (30240477)
MISAWA Hidemi  Tokyo Met.Org.Med.Res., Tokyo Met.Inst.Neurosci., Scientist, 主任研究員 (80219617)
Project Period (FY) 2002 – 2004
Project Status Completed (Fiscal Year 2004)
Budget Amount *help
¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 2004: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 2003: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 2002: ¥1,800,000 (Direct Cost: ¥1,800,000)
KeywordsAmyotrophic lateral sclerosis / ALS / Spinal cord / Anterior horn cell / Ribosome / Rough endoplasmic reticulum / Protein synthesis / Protein infolding / RNA / 転写活性
Research Abstract

Morphological changes of the protein synthesizing system including rough ER, molecular chaperon and Golgi apparatus, and transcription activity of ribosomal (r) RNA gene in the anterior horn cells of the patients with amyotrophic lateral sclerosis (ALS) were examined. The present research elucidated that the activity of the transcription of rRNA gene reduced very quickly after insults on the motor neurons, which induced decrease of cytoplasmic rRNA. Localization of molecular chaperon, KDEL, changed earlier than the fragmentation of the Golgi apparatus and decrease of Nissl substance in the anterior horn cells of the patients with ALS. The cisterns of the rER of the anterior horn cells of ALS were dilated irregularly, and the number of the attached ribosomes on the cistern decreased. Thus the protein synthesizing system in the anterior horn cells in ALS patients degenerated from upper stream to downstream.

Report

(4 results)
  • 2004 Annual Research Report   Final Research Report Summary
  • 2003 Annual Research Report
  • 2002 Annual Research Report
  • Research Products

    (37 results)

All 2005 2004 2003 Other

All Journal Article (26 results) Book (1 results) Publications (10 results)

  • [Journal Article] Oxidized Galectin-1 advances the functional recovery after peripheral nerve injury2005

    • Author(s)
      Kadoya T, et al.
    • Journal Title

      Neurosci. Lett. 380

      Pages: 284-288

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Oxidized Galectin-1 advances the functional recovery after peripheral nerve injury.2005

    • Author(s)
      Kadoya T, Oyanaai K, Kawakami E, Hasegawa M, Inagaki Y, Sohma Y, Horie H.
    • Journal Title

      Neurosci.Lett. 380

      Pages: 284-288

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Amyotrophic lateral sclerosis : on the origin of the degenerated fibers in the white matter of the spinal cord2005

    • Author(s)
      Oyanagi K, et al.
    • Journal Title

      Amyotrophic Lateral Sclerosis : New Research (印刷中)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] The nature of the parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam and magnesium deficiency2005

    • Author(s)
      Oyanagi K, et al.
    • Journal Title

      Parkinsonism and Related Disorders (印刷中)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Klotho insufficiency causes decrease of ribosomal RNA gene transcription activity, cytoplasmic RNA and rough ER in the spinal anterior horn cells2005

    • Author(s)
      Anamizu Y, et al.
    • Journal Title

      Acta Neuropathologica (印刷中)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Oxidized galectin-1 advances the functional recovery after peripheral nerve injury2005

    • Author(s)
      Kadoya T, et al.
    • Journal Title

      Neuroscience Letters (印刷中)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Synthesis, localization and externalization of galectin-1 in mature dorsal root ganglion neurons and Schwann cells2004

    • Author(s)
      Sango K, et al.
    • Journal Title

      Eur J Neurosci 19

      Pages: 55-64

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Ultrastructural localization of high-affinity choline transporter in the rat neuromuscular junction : Enrichment on synaptic vesicles2004

    • Author(s)
      Nakata K, et al.
    • Journal Title

      Synapse 53

      Pages: 53-56

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Calcium-permeable AMPA receptors promote misfolding of mutant S0D1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model2004

    • Author(s)
      Tateno M, et al.
    • Journal Title

      Hum. Mol. Genet. 13

      Pages: 2183-2196

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Distinctive expression of midkine in the repair period of rat brain during neurogenesis : Immunohistochemical and immunoelectron microscopic observations2004

    • Author(s)
      Kikuchi-Horie, K., Kawakami, E., Kamata, M., Wada, M., Hu, JG., Nakagawa, H., Ohara, K., Watabe, K., Oyanagi, K.
    • Journal Title

      J Neurosci Res 75

      Pages: 678-687

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Ultrastructural localization of high-affinity choline transporter in the rat neuromuscular junction : Enrichment on synaptic vesicles.2004

    • Author(s)
      Nakata, K., Okuda, T., Misawa, H.
    • Journal Title

      Synapse 53

      Pages: 53-56

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Distinctive expression of midkine in the repair period of rat brain during neurogenesis : Immunohistochemical and immunoelectron microscopic observations2004

    • Author(s)
      Kikuchi-Horie K, et al.
    • Journal Title

      Journal of Neuroscience Research 75

      Pages: 678-687

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Calcium-permeable AMPA receptors promote misfolding of mutant S0D1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model.2004

    • Author(s)
      Tateno M, et al.
    • Journal Title

      Human Molecular Genetics 13

      Pages: 2183-2196

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Neuropathology with clinical correlations of sporadic amyotrophic lateral sclerosis : 102 autopsy cases examined between 1962 and 20002003

    • Author(s)
      Piao, Y.S., Wakabayashi, K., Kakita, A., Yamada, M., Hayashi, S., Morita, T., Ikuta, F., Oyanagi, K., Takahashi, H.
    • Journal Title

      Brain Pathol 12

      Pages: 10-22

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia2003

    • Author(s)
      Toyoshima, Y., Piao, Y.-S., Tan, C.-H., Morita, M., Tanaka, M., Oyanagi, K., Okamoto, K., Takahashi, H.
    • Journal Title

      Acta Neuropathologica 106

      Pages: 50-56

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Topical instillation of ciliary neurotrophic factor inhibits retinal degeneration in streptozotocin-induced diabetic rats2003

    • Author(s)
      Aizu, Y., Katayama, H., Takahama, S., Hu, J.-G., Nakagawa, H., Oyanagi, K.
    • Journal Title

      NeuroReport 14

      Pages: 2067-2071

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Adenoviral GDNF gene transfer prevents motoneuron loss in the nucleus ambiguus2003

    • Author(s)
      Saito, K., Shiotani, A., Watabe, K., Moro, K., Fukuda, H., Ogawa, K.
    • Journal Title

      Brain Res 962

      Pages: 61-67

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Adenoviral gene transfer of GDNF, BDNF and TGFB2, but not CNTF, cardiotrophin-1 or IGFI, protects injured adult motoneurons after facial nerve avulsion.2003

    • Author(s)
      Sakamoto, T., Kawazoe, Y., Shen, JS., Takeda, Y., Arakawa, Y., Ogawa, J., Oyanagi, K., Ohashi, T., Watanabe, K., Inoue, K., Eto, Y., Watabe, K
    • Journal Title

      J Neurosci Res 72

      Pages: 54-64

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Oral administration of a neuroprotective compound T-588 prevents motoneuron degeneration after facial nerve avulsion in adult rats2003

    • Author(s)
      Ikeda, K., Sakamoto, T., Kawazoe, Y., Marubuchi, S., Nakagawa, M., Ono, S., Terashima, N., Kinoshita, M., Iwasaki, Y., Watabe, K.
    • Journal Title

      Amyotroph Lateral Scler Other Motor Neuron Disord 4

      Pages: 74-80

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Growth inhibitory factor prevents degeneration of injured adult rat motoneurons2003

    • Author(s)
      Sakamoto, T., Kawazoe, Y., Uchida, Y., Hozumi, I., Inuzuka, T., Watabe, K.
    • Journal Title

      Neuroreport 14

      Pages: 2147-2151

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] VAChT-Cre.Fast and VAChT-Cre.Slow : Postnatal Expression of Cre Recombinase in Somatomotor Neurons With Different Onset2003

    • Author(s)
      Misawa, H., Nakata, K., Toda, K., Matsuura, J., Oda, Y., Inoue, H., Tateno, M., Takahashi, R.
    • Journal Title

      genesis 37

      Pages: 44-50

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] The crucial role of caspase-9 in the disease progression of a transgenic ALS mouse model2003

    • Author(s)
      Inoue, H., Tsukita, K., Iwasato, T., Suzuki, Y., Tomioka, M., Tateno, M., Nagao, M., Kawata, A., Saido, T.C., Miura, M., Misawa, H., Itohara, S., Takahashi, R.
    • Journal Title

      EMBO J 22

      Pages: 6665-6674

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Klotho insufficiency causes decrease of ribosomal RNA gene transcription activity, cytoplasmic RNA and rough ER in the spinal anterior horn

    • Author(s)
      Anamizu Y, et al.
    • Journal Title

      Acta Neuropathol (In press)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] The nature of the parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam and magnesium deficiency

    • Author(s)
      Oyanagi K
    • Journal Title

      Parkinsonism Rel. Dis. (In press)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Amyotrophic lateral sclerosis : On the origin on the degenerated fibers in the white matter of the spinal cord

    • Author(s)
      Oyanagi K, Yamazaki M, Kawakami E, Morita T, Makifuchi T, Takahashi H.
    • Journal Title

      Amyotrophic lateral sclerosis : New Research, Nova Science Publishers (In press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Klotho insufficiency causes decrease of ribosomal RNA gene transcription activity, cytoplasmic RNA and rough ER in the spinal anterior horn

    • Author(s)
      Anamizu Y, Kawaguchi H, Seichi A, Yamaguchi S, Kawakami E, Kanda N, Matsubara S, Kuro-o M, Nabeshima Y, Nakamura K, Oyanagi K.
    • Journal Title

      Acta Neuropathol. (In press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Book] The mysterious diseases of Guam (ed.Kwang-Ming Chen)2004

    • Author(s)
      Oyanagi K
    • Total Pages
      292
    • Publisher
      Micronesia Research Center, Univ.Guam
    • Related Report
      2004 Annual Research Report
  • [Publications] Piao Y-S, Oyanagi K, et al.: "Neuropathology with clinical correlation of sporadic amyotrophic lateral sclerosis : 102 autopsy cases examined with between 1962 and 2000"Brain Pathol. 12. 10-22 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Sakamoto T, Oyanagi K, et al.: "Adenoviral gene transfer of GDNF, BDNF and TGFβ2, but not CNTF, cardiotrophin-1 or IGF1, protects injured adult motoneurons after facial nerve avulsion"J Neurosci Res. 72. 54-64 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Toyoshima Y, Oyanagi et al.: "Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia"Acta Neuropathol. 106. 50-56 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Aizu Y, Oyanagi K, et al.: "Topical instillation of ciliary neurotrophic factor inhibits retinal degeneration in streptozotocin-induced diabetic rats"Neuroreport. 14. 2067-2071 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Oyanagi K, et al.: "Amyotrophic lateral sclrerosis : on the origin of the degenerated fibers in the white matter of the spinal cord"Progress of the amyotrophic lateral sclerosis research. (印刷中). (2004)

    • Related Report
      2003 Annual Research Report
  • [Publications] Oyanagi K. et al.: "Alteration of rough endoplasmic reticulum in the anterior horn cells in amyotrophic lateral sclerosis"Brain Pathol. Suppl.. S120 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Oyanagi K: "The mysterious diseases of Guam(ed. Kwang-Ming Chen)"Micronesia Research Center, Univ. Guam(印刷中). (2004)

    • Related Report
      2003 Annual Research Report
  • [Publications] Toyoshima Y., et al.: "Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia"Acta Neuropathologica. (印刷中). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Sakarnoto T., et al.: "Adenoviral gene transfer of GDNF, BDNF arid TGFβ2, but not CNTF, cardiotrophin-1 or IGF1, protects injured adult motoneurons after facial nerve avulsion"Journal for Neuroscience Research. (印刷中). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Piao Y.-S., et al.: "Neuropathology with clinical correlation of sporadic amyotrophic lateral sclerosis : 102 autopsy cases examined between 1962-2000"Brain Pathology. 12(1). 10-22 (2003)

    • Related Report
      2002 Annual Research Report

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Published: 2002-04-01   Modified: 2016-04-21  

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