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Glial participation in neuronal disturbance of Niemann-Pick disease type C

Research Project

Project/Area Number 12670788
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionSt. Marianna University School of Medicine

Principal Investigator

SAKIYAMA Takeshi  St. Marianna University, School of Medicine Pathology, Associate Professor, 医学部, 助教授 (20130510)

Co-Investigator(Kenkyū-buntansha) SUZUKI Hiroyoshi  Sendai National Hospital, 臨床検査科, 科長
ABE Mitsubumi  St. Marianna University, School of Medicine Pathology, Assistant Professor, 医学部, 講師 (30231971)
TADOKORO Mamoru  St. Marianna University, School of Medicine Pathology, Professor, 医学部, 教授 (80081644)
Project Period (FY) 2000 – 2001
Project Status Completed (Fiscal Year 2001)
Budget Amount *help
¥3,400,000 (Direct Cost: ¥3,400,000)
Fiscal Year 2001: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 2000: ¥2,200,000 (Direct Cost: ¥2,200,000)
KeywordsNiemann-Pick disease type C / Glia / neural disturbance / Niemann-Pick mouse / BMT / animal model / ニューマンピック病C型 / ニューマンピックマウス
Research Abstract

Some neurodegenerative disorders have possible disturbance in not only neuron but also glia including astrocytes. In order to establish an effective therapy for neurodegenerative disorders in the future, pathophysiology and chronological changes occurring in several species including human-being were studied. For this purpose, we used Niemann-.Pick type C (NPC) disease. The interrelationship between neuron and glia was.studied among different species such as human, mice and cat. The difference of phenotype was ascertained in that atrophy of cerebellum cortex and deletion of Purkinje cells occurred in human and mice, but not in cat, which showed lipid accumulation in Purkinje cells. The cerebral cortex of mice showed ubiquitin positive substance in neuron and its surrounding macrophages. This positive substance existed also in the white matter and astrocytes. It may disturb the protective function on neuron, and may result in nenronal disturbance. These findings progressed and showed a more diffuse distribution as mice grew older. However, BMT treated NPC mice at 8 weeks of age showed almost no remntfnt of ubiquitinized accumulation, and macrophages were contiguous to neurons. Neuronal change was minimum by light microscopy, although EM findings showed improvement. After BMT, the number of astrocytes decreased and the accumulation was diminished. After BMT, there was no clinical improvement in this disease. However, the fact that accumulation was processed in the brain may suggest future hope in obtaining an effective therapy for slowly progressive neurodegenerative disorders.

Report

(3 results)
  • 2001 Annual Research Report   Final Research Report Summary
  • 2000 Annual Research Report
  • Research Products

    (29 results)

All Other

All Publications (29 results)

  • [Publications] Sakiyama T, Suzuki H et al.: "The effect of BMT for the clearance of degenerated material in the CNS of Niemann-Pick disease type C"NEUROPATHOLOGY. 20・2. A56 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] 川名 裕, 崎山 武志他: "ムコ多糖症VI型のモデルラットに対する骨髄移植の効果"聖マリアンナ医大誌. 29・5. 459-468 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Orimo H, Sakiyama T et al.: "Importance of deletion of T at nucleotide 1559 alkaline phosphatase gene with hypophosphatasia"J Bone Miner Metab. 20. 28-33 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] 河村 英博, 田所 衛 他: "明らかな心筋症状,知能遅滞をみないLysosomal glycogen storage disease with normal acid maltaseの一例"臨床神経学. 40・3. 259-262 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Hisanaga K, Suzuki H et al.: "Fluoro-DOPA and FDG positron emission tomography in a case of pathologically verified pure diffuse Lewy body disease"J.Neurol. 248. 905-906 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Suzuki H, et al.: "A case of anaplastic ganglioglioma with sarcomatous component"NEUROPATHOLOGY. (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Sakiyama,T. et al.: "The correlative disturbance of glia in neuronal dysfunction"Neuropathlogy. 19 (2). A37 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Sakiyama,T. et al.: "The effect of BMT for the clea-Rance in the CNS ofNiemann-Pick disease type C"Neuropathlogy. 20 (2). A56 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Kawamura,H. et al.: "A case of lysosomal storage disease with normal acid maltase without MR"Clinical Neurology (in Japanese). 40 (3). 259-262 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Tajima,C. et al.: "A case of hypertrophic pachymeningitis improved with CPA pulse therapy"Neurological Therapeutics (in Japanese). 17 (2). 167-171 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Jinn,I. et al.: "Diffuse Lewy body disease from 114 cases with Parkinson disease"Clinical Neurology (in Japanese). 40 (4). 329-333 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Kushita,J. et al.: "An adult case of malignant rhabdoid tumor origined from upper saddle"Neurosurgery (in Japanese). 28. 351-358 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Kawana,Y. et al.: "The effect of BMT on bone in MPS VI model Rat"St. Marianna Med. J.. 29 (5). 459-468 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Hisanaga,K. et al.: "Fluoro-DOPA and FDG PET in a case of pure diffuse Lewy body disease"J. Neurol. 248. 905-906 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Sugihara,H. et al.: "An autopsy case of hemi-laterally dominant border zone infarction"Neuropathology. 21 (4). 278-287 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Orimo,H. et al.: "Importance of deletion of T at nucleotide 1559 Alp gene with hypophosphatasia"J Bone Miner Metab. 20. 28-33 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Suzuki,H. et al.: "A case of anaplastic ganglio-glioma with sarcomatous component"Neuropathology. (in press). (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] 川名 裕, 崎山 武志他: "ムコ多糖症VI型のモデルラットに対する骨髄移植の効果"聖マリアンナ医大誌. 29・5. 459-468 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Orimo H, Sakiyama T et al.: "Importance of deletion of T at nucleotide 1559 alkaline phosphatase gene with hypophosphatasia"J Bone Miner Metab. 20. 28-33 (2002)

    • Related Report
      2001 Annual Research Report
  • [Publications] 河村 英博, 田所 衛他: "明らかな心筋症状,知能発達遅滞をみないLysosomal glycogen storage disease with normal acid maltaseの一例"臨床神経学. 40・3. 259-262 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] 田島 千秋, 田所 衛他: "Cyclophosphamide静注パルス療法が著効した肥厚性脳硬膜炎の1症例"神経治療. 17・2. 167-171 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] Hisanaga K, Suzuki H et al.: "Fluoro-DOPA and FDG positron emission tomography in a case of pathologically verified pure diffuse Lewy body disease"J. Neurol.. 248. 905-906 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Suzuki H, Tadokoro M et al.: "A case of anaplastic ganglioglioma with sarcomatous component"Neuropathology. (in press). (2002)

    • Related Report
      2001 Annual Research Report
  • [Publications] T Sakiyama,H Suzuki et al: "The correlative disturbance of glia in neuronal dysfunction"NEUROPATHOLOGY. 19・2. A37 (1999)

    • Related Report
      2000 Annual Research Report
  • [Publications] T Sakiyama,H Suzuki et al: "The effect of BMT for the clearance of degenerated material in the CNS of Niemann-Pick disease type C"NEUROPATHOLOGY. 20・2. A56 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 河村英博,田所衛 他: "明らかな心筋症状,知能遅滞をみないLysosomal glycogen storage disease with normal acid maltaseの一例"臨床神経学. 40・3. 259-262 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 田島千秋,田所衛 他: "Cyclophosphamide静注パルス療法が著効した肥厚性脳硬膜炎の1症例"神経治療. 17・2. 167-171 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 神一敬,鈴木博義 他: "パーキンソン病114例から検討したdiffuse Lewy body disease"臨床神経学. 40. 329-333 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 久下淳史,鈴木博義 他: "鞍上部原発Malignant Rhabdoid Tumorの1成人例"脳外. 28. 351-358 (2000)

    • Related Report
      2000 Annual Research Report

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Published: 2000-04-01   Modified: 2016-04-21  

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