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Study of the pathogenesis of adrenoleukodystrophy

Research Project

Project/Area Number 60480220
Research Category

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

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionNiigata University

Principal Investigator

MIYATAKE Tadashi  Brain Research Institute, Niigata University ・ Professor, 脳研究所, 教授 (50048998)

Co-Investigator(Kenkyū-buntansha) SATOU Syuzou  University Hospital, Niigata University ・ Assistant, 医学部付属病院, 講師 (30115034)
ATUMI Tetushi  Brain Research Institute, Niigata University ・ Assistant professor, 脳研究所, 助教授 (50049061)
Project Period (FY) 1985 – 1986
Project Status Completed (Fiscal Year 1986)
Budget Amount *help
¥7,000,000 (Direct Cost: ¥7,000,000)
Fiscal Year 1986: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1985: ¥6,500,000 (Direct Cost: ¥6,500,000)
KeywordsAdrenoleukodystrophy / Zellwerger Syndrome / Zellweger症候群
Research Abstract

We have studied the metabolism of long chain fatty acids in cultured skin fibroblasts from patients with adrenoleukodystrophy(ALD). The composition of radioactive fatty acids in total lipids of fibroblasts after culture in the presence of <^3H_2> -hexacosanoic acid on <^3H> -lignoreric acid was analyzed by radio-gaschromatography. Most of the fatty acids added to the culture media were degraded to shorter-chain fatty acids both by control and ALD fibroblasts. However, the content of the remaining radioactive <C_(24:0)> or <C_(26:0)> was higher in ALD fibroblast. This result indicated that the degradation of very long chain fatty acids (VLCFA) is decreased in ALD.
Next, we examined the VLCFA metabolism in fibroblasts from Zellweger syndrome. The increased synthesis of <C_(26:0)> from <C_(18:0)> was found in the fibroblasts of Zellweger syndrome as well as ALD.

Report

(1 results)
  • 1986 Final Research Report Summary
  • Research Products

    (7 results)

All Other

All Publications (7 results)

  • [Publications] K.Tanaka;A.Koyama;R.Koike;T.Ohno;T.Atsumi;T.Miyatake: J.Neurol. 232. 73-78 (1985)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] S.Tsuji;T.Sano-Kawamura;T.Ariga;T.Miatake: J.Neurol.Sci. 71. 359-367 (1985)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] 宮武正: Annual Review 神経. 200-204 (1986)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] 宮武正: 臨床医. 12. 110-112 (1986)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] K. Tanaka: "Adrenomyeloneuropathy: report of a family and electron microscopical findings in peripheral nerve." J. Neurol.232. 73-78 (1985)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] S. Tsuji: "Metabolism of [17, 18- <^3H_2> ]Hexacosanoic Acid and [15, 16- <^3H_2> ]Lignoceric Acid in Cultured Skin Fibroblasts from Patients with Adrenoleukodystrophy(ALD) and Adrenomyeloneuropathy(AMN)" J. Neurol. Sci.71. 359-367 (1985)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1986 Final Research Report Summary
  • [Publications] T. Miyatake: "Adrenoleukodystrophy(ALD)" Medical Clinics of Japan. 12. 110-112 (1986)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1986 Final Research Report Summary

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Published: 1987-03-31   Modified: 2016-04-21  

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