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1987 Fiscal Year Final Research Report Summary

MOLECULAR ANALYSIS OF THE DEFECT OF <beta>-OXIDATION ENZYMES IN PEROXISOMAL DISORDERS

Research Project

Project/Area Number 60480141
Research Category

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

Allocation TypeSingle-year Grants
Research Field Pathological medical chemistry
Research InstitutionShinshu University

Principal Investigator

HASHIMOTO Takashi  Department of Biochemistry, Shinshu University School of Medicine, 医学部, 教授 (80009935)

Co-Investigator(Kenkyū-buntansha) MIYAZAWA Shoko  Department of Biochemistry, Shinshu University School of Medicine, 医学部, 助手 (20020745)
Project Period (FY) 1985 – 1987
KeywordsPeroxisomal Disorders0Fatty Acid Oxidation Enzymes / 脂肪酢酸化系酵素 / 酵素欠損
Research Abstract

The presence of a new peroxisomal disorders has been recently recognized. The characteristic biochemical deterioration in these diseases in an accumulation of very long-chain fatty acids in body fluids and tissues in the patients. Peroxisomal -oxidation system has a mian role in the degradation of these fatty acids.
In the present project using patients' specimens, the contents of the individual enzymes of this system (acyl-CoA oxidase, enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenease bifunctional enzyme and 3-ketoacyl-CoA thiolase), their expression and the proteolytic processing were examined by immunoblot analysis and pulse-labeling and chase experiments. In the patients with Zellweger syndrome and infantile Refsum disease who lack peroxisome structure, all three enzymes were synthesized but disappeared rapidly. The absence of the specific enzyme was proved in some diseases. They are now called as acyl-CoA oxidase deficiency and 3-ketoacyl-CoA thiolase deficiency. In X-linked adrenoleukodystrophy, all of the enzymes were detected. The etiology of this disease is now suspected to the disfunction of the activation of very long-chain fatty acids. In neonatal adrenoleukodystrophy and rhizomelic chondrodysplasia punctata, the characteristic patterns in the enzyme defect including the absence of the proteolytic processing of 3-ketoacvl-CoA thiolase were found. This work was conducted in collaboration with many groups.
The cDNAs and genes for rat enzymes were isolated and characterized for the purpose of the gene analysis of the human disease.

  • Research Products

    (10 results)

All Other

All Publications (10 results)

  • [Publications] Suzuki, Y.;Hashimoto, T. 他: Clin. Chim. Acta. 156. 191-196 (1986)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Schram, A. W.;Hashimoto, T. 他: Proc. Natl. Acad. Sci. USA. 83. 6156-6158 (1986)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Suzuki, Y.;Hashimoto, T. 他: J. Inher. Metab. Dis.9. 292-296 (1986)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Chem, W. W.;Hashimoto, T. 他: Proc. Natl. Acad. Sci. USA. 84. 1425-1428 (1987)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Schram, A. W.;Hashimoto, T. 他: Proc. Natl. Acad. Sci. USA. 84. 2494-2496 (1987)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Suzuki, Y. Orii, T., Mori, M., Tatibana, M., and Hashimoto, T.: "Deficient activities and proteins of peroxisomal <beta>-oxidation enzymes in infants with Zellweger syndrome" Clin. Chim. Acta. 156. 191-196 (1686)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Schram, A.W., Srijland, A., Hashimoto, T., et al.: "Biosynthesis and maturation of peroxisomal <beta>-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum disease" Proc. Natl. Acad. Sci. USA. 83. 6156-6158 (1986)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Suzuki, Y., Orii, T., and Hashimoto, T.: "Biosynthesis of peroxisomal <beta>-oxidation enzymes in infants with Zellweger syndrome." J. Inher. Metab. Dis.9. 292-296 (1986)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Chen, W.W., Watkins, P.A., Osumi, T., Hashimito, T., and Moser, H.W.: "Peroxisomal <beta>-oxidation enzyme proteins in adrenoleukodystrophy: Distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy" Proc. Natl. Acad. Sci. USA. 84. 1425-1428 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Schram, A.W., Hashimoto, T., et al.: "Human peroxisomal 3-ketoacyl-CoA thiolase deficiency" Proc. Natl. Acad. Sci. USA. 84. 2494-2496 (1987)

    • Description
      「研究成果報告書概要(欧文)」より

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Published: 1989-03-30  

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