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Studies on pathogenesis of Zellweger syndrome

Research Project

Project/Area Number 61304046
Research Category

Grant-in-Aid for Co-operative Research (A)

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionTOHOKU UNIVERSITY

Principal Investigator

TADA Keiya  Department of Pediatrics, Tohoku University School of Medicine, 医学部, 教授 (20046907)

Co-Investigator(Kenkyū-buntansha) AIKAWA Junitiro  Department of Pediatrics, Tohoku University School of Medicine, 助手 (30191844)
YOKOTA Sadaki  Department of Anatomy, Yamanashi Medical College, 助教授 (40020755)
ORII Tadao  Department of Pediatrics, Gifu University School of Medicine, 医学部, 教授 (20045339)
HASHIMOTO Takashi  Department of Biochemistry, Shinshu University Medical School, 医学部, 教授 (80009935)
野呂 知世  東北大学, 医学部附属病院, 医員
Project Period (FY) 1986 – 1988
Project Status Completed (Fiscal Year 1988)
Budget Amount *help
¥13,000,000 (Direct Cost: ¥13,000,000)
Fiscal Year 1988: ¥3,000,000 (Direct Cost: ¥3,000,000)
Fiscal Year 1987: ¥5,000,000 (Direct Cost: ¥5,000,000)
Fiscal Year 1986: ¥5,000,000 (Direct Cost: ¥5,000,000)
KeywordsZellweger syndrome / Peroxisomes / beta-oxidation / β酸化系酵素 / β-酸化系酵素 / 脂肪酸βー酸化 / ペルオキシソーム / 脂肪酸Β-酸化
Research Abstract

This study was undertaken in order to elucidate pathogenesis of Zellweger syndrome (ZS), in which peroxisomes do not exist in cells. The following results were obtained 1) The defect of enzymes involved in peroxisomal -oxidation was recognized in cells from the patients with ZS. 2) The defect of peroxisomal membrane proteins of 70 KD, 26 KD and 22 KD was found in cells from the patient with ZD. 3) The enzymes involved in mitochondrial -oxidation were all intact in ZS cells. 4) It was suggested that the peroxisomal enzymes can be synthesized in ZS cells, but they are rapidly decomposed in cytoplasma because of the absence of peroxisomes.

Report

(4 results)
  • 1988 Annual Research Report   Final Research Report Summary
  • 1987 Annual Research Report
  • 1986 Annual Research Report
  • Research Products

    (25 results)

All Other

All Publications (25 results)

  • [Publications] Aikawa,J; Tada,K.et al.: J.Inher.Metab.Dis.10. 211-213 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Aikawa,J; Tada,K.et al.: J.Inher.Metab.Dis.in press. (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Miyazawa,S; Hashimoto,T.et al.: J.Biol.Chem.262. 8131-8137 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Osumi,T; Hashimoto,T.et al.: J.Bial.Chem.262. 8138ー8143 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Shimozawa,N; Orii,T.et al.: Prenatal Diagrosis. 8. 287-290 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Suzuki,Y; Orii,T.et al.: Clin.Chim.Acta. 172. 65-76 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Hashimoto,T.: "Peroxisomes in Biology and Medicine" Springer-Verlag,Heidelberg, 97-104、105-114、 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Tada, K.,et al.: "The abnormality of peroxisomal membrane proteins in Zellweger syndrome" J. Inher. Metab. Dis.10. 211-213 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Hashimoto, T.,et al.: "Complete nucleotide sequence of c-DNA and predicted amino acid sequence of rat acyl-CoA oxidase" J. Biol. Chem.262. 8131-8137 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Hashimoto, T.,et al.: "Isolation and structual characterization of the rat acyl-CoA oxidase gene" J. Biol. Chem.262. 8138-8143 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Orii, T.,et al.: "Immunoblot detection of enzyme proteins of peroxisomal beta-oxidation in fibroblasts, amniocytes and chorionic villi" Prenatal Diagnosis. 8. 287-190 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Orii, T.,et al.: "Molecular analysis of peroxisomal beta-oxidation enzymes in infants with Zellweger syndrome" Clin. Chim. Acta. 172. 65-76 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Hashimoto, T.: Peroxisomes in Biology and Medicine. Spring-Verlag, 97-104 105-114 341-352 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Aikawa,J.;Tada,K.;et al.: J.Inher.Metab Dis.(1989)

    • Related Report
      1988 Annual Research Report
  • [Publications] Tada,K.,et al.: Asian Med.J.31. 642-648 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Shimazawa,N.;Crii,T,et al.: Prenatal Diagnosis. 8. 287-290 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Suzuki,Y.;Orii,T.,et al.: Cln.Chim.Acta. 172. 65-76 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Shimazawa,N.;Orii,T.,et al.: Clin.Chim Acta.175. 345-348 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Naoitomi,K.;Orii,T.,et al.: Human Genet.80. 201-202 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 多田啓也 他: J.Inher.Metab,Dis.10. 211-213 (1987)

    • Related Report
      1987 Annual Research Report
  • [Publications] 横田貞記 他: 病理と臨床. 6. 198895-100

    • Related Report
      1987 Annual Research Report
  • [Publications] 清水直容,岡博 他編,多田啓也 他: "Annual Review 内分泌代謝1987" 中外医学社, 325 (1987)

    • Related Report
      1987 Annual Research Report
  • [Publications] 多田啓也 他: J.Inher,Metab.Dis.10. (1987)

    • Related Report
      1986 Annual Research Report
  • [Publications] 多田啓也 他: 医学のあゆみ. 139. 891-896 (1986)

    • Related Report
      1986 Annual Research Report
  • [Publications] 多田啓也著 他 清水直容,岡博 他編: "Arrnal Review内分泌,代謝" 中外医学社, 38 (1987)

    • Related Report
      1986 Annual Research Report

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

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