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Tuberous sclerosis; genetic, cellural and molecular investigation.

Research Project

Project/Area Number 03454265
Research Category

Grant-in-Aid for General Scientific Research (B)

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionTottori University

Principal Investigator

TAKESHITA Kenzo  Tottori Univ Fac Med. Professor, 医学部, 教授 (90037375)

Co-Investigator(Kenkyū-buntansha) INAGAKI Masumi  Tottori Univ Fac Med. Assistant, 医学部, 助手 (70203198)
KOEDA Tatsuya  Tottori Univ Fac Med. Assistant, 医学部, 助手 (70225390)
河原 仁志  鳥取大学, 医学部附属病院, 助手 (00186123)
Project Period (FY) 1991 – 1992
Project Status Completed (Fiscal Year 1992)
Budget Amount *help
¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1992: ¥700,000 (Direct Cost: ¥700,000)
Keywordstuberous sclerosis / GFAP / mitogen / 細菌内毒素 / 酸性グリア特異蛋白 / マイト-ゲン
Research Abstract

We investigated tuberous sclerosis(TS) epidemiologically, clinically and pathophysiologically.
The prevalence among the general population of the San-in district in 1979 was estimated to be 3.2 x 10^<-5>. The frequency of the sporadic case in live-births was 3.2 x 10^<-5>. The mutation rate was directly estimated to be 1.6 x 10^<-5> per gene per generation.
Sister chromatid exchange, chromosome aberration and lymphocyte proliferative kinetics in MNNG treated lymphocytes did not show the high cancer propensity. Skin fibroblasts from TS patients also showed normal reaction for growth characteristics and sensitivities to DNA alkylating agents.
The cells cultured from facial angiofibromas of TS patients showed apparently abnormal pattern morphologically. The cells were examined immunocytochemically for expression of selected cytoskeletal and non-structural proteins and were compared with normal skin fibroblasts and normal glial cells. In similarity to nromal glia, the angiofibroma stroma cells expressed glial fibrillary acidic protein (GFAP) and produced fibronectin. It was established that GFAP and vimentin were co-localized in intermediate filaments of the angiofibroma cells.

Report

(3 results)
  • 1992 Annual Research Report   Final Research Report Summary
  • 1991 Annual Research Report
  • Research Products

    (9 results)

All Other

All Publications (9 results)

  • [Publications] Kato M.: "Stimulation of human fetal astrocyte proliferation by bacterial lipopolysaccharides and lipids A." Acta Neuropathol. 45. 455-457 (1991)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1992 Final Research Report Summary
  • [Publications] Kato M.: "Expression of glial fibrillary acidic protein (GFAP) by cultured angiofibroma stroma cells from patients with tuberous sclerosis." Neuropath and Applied Neurobiol. 18. 559-565 (1992)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1992 Final Research Report Summary
  • [Publications] Kato M: "Stimulation of human fetal astrocyte proliferation by bacterial lipopolysaccharides and lipids A." Acta Neuropathol. 45. 455-7 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1992 Final Research Report Summary
  • [Publications] Kato M: "Expression of glial fibrillary acidic protein (GFAP) by cultured angiofibroma stroma cells from patients with tuberous sclerosis." Neuropath and Applied Neurobiol. 18. 559-65 (1992)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1992 Final Research Report Summary
  • [Publications] Kato M: "Stimulation of huaman fetal astrocyte proliferation by bacterial lipopolysaccharides and lipids A." Acta Neuropathol. 45. 455-457 (1991)

    • Related Report
      1992 Annual Research Report
  • [Publications] Kato M: "Expression of glial fibrillary acidic protein (GFAP) by cultured angiofibroma stroma cells from patients with tuberous sclerosis." Neuropath and Applied Neurobiol. 18. 559-565 (1992)

    • Related Report
      1992 Annual Research Report
  • [Publications] Kato M.,Ohno K.,Takeshita K.,et al.: "Stimulation of human fetal astrocyte proliferation by bacterial lipopolysaccharides and lipid A" Acta Neuropathol. 82. 384-388 (1991)

    • Related Report
      1991 Annual Research Report
  • [Publications] Kato M.,Katsumoto T.,Ohno K.,et al: "Expression of glial fibrillary acidic protein(GFAP)、by cultured angiofibroma stroma lls from patients with tuberous sclerosis" Applied Neuropathol and Neurobiol.

    • Related Report
      1991 Annual Research Report
  • [Publications] 大野 耕策,竹下 研三,有馬 正高: "結節性硬化症皮膚病変部細胞の増殖に与える細菌内毒素の影響" 脳と発達(総会号). 24. (1992)

    • Related Report
      1991 Annual Research Report

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Published: 1992-04-01   Modified: 2016-04-21  

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