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The cause of neuropathochemistry of inherited Neurodegeneration

Research Project

Project/Area Number 08457232
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionThe Jikei University School of Medicine

Principal Investigator

ETO Yoshikatsu  Jikei University School of Medicine prof., 医学部・小児科, 教授 (50056909)

Co-Investigator(Kenkyū-buntansha) HASEGAWA Yoriyasu  Jikei University School of Medicine, Senior investigator, 医学部・小児科, 助手 (60256435)
TSUDA Takashi  Jikei University School of Medicine, Senior investigator, 医学部・小児科, 助手 (50188554)
OHASHI Toya  Jikei University School of Medicine assi prof., 医学部・小児科, 講師 (60160595)
IDA Hiroyuki  Jikei university School of Medicine, assi prof., 医学部・小児科, 講師 (90167255)
Project Period (FY) 1996 – 1997
Project Status Completed (Fiscal Year 1997)
Budget Amount *help
¥7,500,000 (Direct Cost: ¥7,500,000)
Fiscal Year 1997: ¥3,600,000 (Direct Cost: ¥3,600,000)
Fiscal Year 1996: ¥3,900,000 (Direct Cost: ¥3,900,000)
KeywordsGaucher disease / glucocerebroside / Conduritol-B-epoxedo (CBE) / サイコシン / 遺伝性ロイコヂストロフィー症 / ゴ-シェ病 / glicosylsphingosine / スフィンゴリピド / プロティンキナーゼC活性 / glucosylsphingosine / Human Oligodendroglioma(HOG)細胞 / Conduritol-B-epoxide(CBE) / 細胞内カルシウム濃度
Research Abstract

The cause of neurological findings in inherited neurodegernerative disorders is unknown. In this studies, we attempt to elucidate the possible cause of neurological disorder, particularly in Gaucher disease.
Gaucher disease is characterized by the accumulation of glucocerebroside in reticuloendotherial cells caused by a deficiency of lysosomal glucocerebrosidase. In central nervous tissues with Gaucher disease, there is few accumulation of glucocerebroside. The difference of the degree of accumulation of glucocerebroside seems to be cell type specific phenomenon. Therefore, we tested these finding using different tumor cells such as human oligodendroglioma cells, human neuroblastoma cells, Conduritol-B-epoxide (CBE) is a potent inhibitor for lysosomal betaglucosidase. Administration of CBE,less than 100ug/ml per bottle into cultured human oligodendroglioma cells produced significant accumulation of glucocerebroside. Simultaneously, enzyme activity was completely inhibited by administration of CBE at the same concentration. Morphological pictures shows abnormal membranous cytoplasmic body in HOG cells, whereas in human neuroblastoma cells. These data suggest the morphological picture in Gaucher disease is different from cells to cells.

Report

(3 results)
  • 1997 Annual Research Report   Final Research Report Summary
  • 1996 Annual Research Report
  • Research Products

    (26 results)

All Other

All Publications (26 results)

  • [Publications] Ohashi T., Eto Y., et al.: "Efficient and persistent expression of b-glucuronidase gene in CD34+ cells from・・・" J.Haematology. (in press). (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T., Eto Y., et al.: "Adenoviral-mediated gene transfer and expression of human beta-glucuronidase gene・・・" Proc.Natl.Acad.Sci.USA. 94. 1287-1292 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ida H., Eto Y., et al.: "Mutation prevalence among 47 unrelated Japanese patients with Gaucher disease・・・" J.Inher.Metabo.Dis.20. 67-73 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Iwasawa K., Eto Y., et al.: "Differences in origin of the 1448C mutation in patients・・・" Acta Pediatr.Jap.39. 451-453 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T., Eto Y., et al.: "Gene therapy for metachromatic leukodysprophy." Acta Paediatr.Jap.38. 193-201 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ida H., Eto Y., et al.: "Identification of three novel mutations in the acid sphingomyelinase gene of・・・" Hum Mutat. 7. 65-68 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 衛藤 義勝: "Gaucher病;小児科学" 医学書院, 308-309 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T., Eto Y., et al.: "Efficient and persistent expression of b-glucuronidase gene in・・・" J.Haematology. (in press). (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T., Eto Y., et al.: "Adenoviral-mediated gene transfer and expression of human・・・" Proc.Natl.Acad.Sci.USA. 94. 1287-1292 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ida H., Eto Y., et al.: "Mutation prevalence among 47 unrelated Japanese patients with・・・" J.Inner.Metabo.Dis.20. 67-73 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Iwasawa K., Eto Y., et al.: "Differences in origin of the 1448C mutation in patients・・・" Acta Pediatr.Jap. 39. 451-453 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T., Eto Y., et al.: "Gene therapy for metachromatic leukodysprophy." Acta Paediatr.Jap.38. 193-201 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ida H., Eto Y., et al.: "Identification of three novel mutations in the acid・・・" Hum Mutat. 7. 65-68 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ida H., Eto Y.et al.: "Clinical and genetic studies of five fatal cases of・・・" Acta Paediatr Jpn. 38. 233-236 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Ohashi T.,Eto Y.,et al.: "Efficient and persistent expression of b-glucuronidase gene in CD34_+ cells from・・・" J.Haematology. (in press). (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] Ohashi T.,Eto Y.,et al.: "Adenoviral-mediated gene transfer and expression of human beta-glucuronidase gene・・・" Proc.Natl.Acad.Sci.USA. 94. 1287-1292 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Ida H.,Eto Y.,et al.: "Mutation prevalence among 47 unrelated Japanese patients with Gaucher disease・・・" J.Inher.Metabo.Dis.20. 67-73 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Iwasawa K.,Eto Y.,et al.: "Differences in origin of the 1448C mutation in patients・・・" Acta Pediatr.Jap.39. 451-453 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Ida H.,Eto Y.et al.: "Identification of three novel mutations in the acid sphingomyelinasegene・・・" Hum Mutat. 7. 65-68 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Ida H.,Eto Y.et al.: "Clinical and genetic studies of five fatal cases of Japanese Gaucher・・・" Acta Paediatr Jpn. 38. 233-236 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Ida H.,Eto Y.et al.: "Mutation screening of 17 Japanese patients with neuropathic Gaucher・・・" Hum Genet. 98. 4294-4296 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Ohashi T.,Eto Y.et al.: "Adenoviral-mediated gene transfer and expression of human・・・" Proc.Natl.Acad.Sci.USA. 94. 1287-1292 (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] Ohashi T.,Eto Y.et al.: "Gene therapy for metachromatic leukodysprophy." Acta Paediatr.Jap.38. 193-201 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Ohashi T.,Eto Y.et al.: "Overexpression of Arylsulfatase A gene in fibroblasts・・・" Gene Therapy.2. 1-6 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] 衛藤義勝: "Niemann-Pick病.神経・筋疾患.5代謝性・中毒神経疾患." 中山書店., 11 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 衛藤義勝: "Zellwege症候群.神経・筋疾患.5代謝性・中毒性神経疾患." 中山書店., 3 (1996)

    • Related Report
      1996 Annual Research Report

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Published: 1996-04-01   Modified: 2016-04-21  

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