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The role of reactive oxygen species and mitochondria in human neuromusclar diseases.

Research Project

Project/Area Number 07670738
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionUniversity of Nagoya

Principal Investigator

YONEDA Makoto  Nagoya University, Department of Biomedical Chemistry, Research Assistant, 医学部, 助手 (70270551)

Co-Investigator(Kenkyū-buntansha) OZAWA Takayuki  Nagoya University, Department of Biomedical Chemistry, Professor Emeritus, 医学部, 名誉教授 (80022771)
TANAKA Masashi  Nagoya University, Department of Biomedical Chemistry, Associate Professor, 医学部, 助教授 (60155166)
Project Period (FY) 1995 – 1996
Project Status Completed (Fiscal Year 1996)
Budget Amount *help
¥2,500,000 (Direct Cost: ¥2,500,000)
Fiscal Year 1996: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 1995: ¥1,300,000 (Direct Cost: ¥1,300,000)
Keywordsmitochondrial genome / mitochondrial diseases / nucleotide substitutions / cell fusion / oxidative damage / cell death / reactive oxygen species / signal peptide / ミトコンドリア / ρ^0細胞 / 酸素ストレス / 活性酸素ラジカル / 遺伝子欠失
Research Abstract

To gain an insight into the role of oxidative damages in the human diseases caused by mitochondrial DNA (mtDNA) mutations or during the physiological aging process, we have investigated the vulnerability to a high concentration of oxygen atmosphere (oxygen stress) and the oxidative damage to mtDNA in cultured cell lines carrying normal mtDNA (p^+), lacking mtDNA (p^0), or harboring mitochondrial tRNA gene mutations (syn^-) which are responsible for human neuromuscular disorders : mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) or myoclonus epilepsy associated with ragged-red fibers (MERRF) syndrome. These syn^- cell lines possessed higher vulnerability to the oxygen stress and exhibited higher production of hydroxyl radical than the parental p^+ cells. In the syn^- cells, extensive accumulation of deleted mtDNAs was demonstrated, compared with a smaller number of deleted mtDNAs in the parental p^+ cell. These in vitro culture studies demonstrate that the oxidative damage to mtDNA by the defective respiratory chain can be an underlying molecular mechanism in determining the cell death in human diseases caused by mtDNA mutations, or in aging.

Report

(3 results)
  • 1996 Annual Research Report   Final Research Report Summary
  • 1995 Annual Research Report
  • Research Products

    (22 results)

All Other

All Publications (22 results)

  • [Publications] Tanno Y: "Quantitation of heteroplasmy of mitochondrial tRNALeu (UUR) gene using PCR-SSCP." Muscle Nerve. 18. 1390-1397 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoneda M: "Oxygen stress induces an apoptotic cell death associated with fragmentation of mitochondrial genome." Biochem. Biophys. Res. Commun.209. 723-729 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoneda M: "Detection and quantitation of point mutations in mtDNA by PCR" Methods Enzymol.264. 432-441 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Sawano T: "Mitochondrial DNA mutations associated with the 11778 mutation in Leber's disease." Biochem. Int.38. 693-700 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Tanaka M: "Accumulation of deletions and point mutations in mitochondrial genome in degenerative diseases." Ann. NY Acad. Sci.786. 102-111 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kovalenko SA: "Accumulation of somatic nucleotide substitutions in mitochondrial tRNALeu (UUR) mutation encephalopathy and cardiomyopathy." Biochem. Biophys. Res. Commun.222. 201-207 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Tanno Y: "Quantitation of heteroplasmy of mitochondrial tRNALeu (UUR) gene using PCR-SSCP." Muscle Nerve. 18. 1390-1397 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoneda M: "Oxygen stress induces an apoptotic cell death associated with fragmentation of mitochondrial genome." Biochem.Biophys.Res.Commun.209. 723-729 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoneda M: "Detection and quantitation of point mutations in mtDNA by PCR." Methods Enzymol.264. 432-441 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Sawano T.: "Mitochondrial DNA mutations associated with the 11778 mutation in Leber's disease." Biochem.Int.38. 693-700 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Tanaka M.: "Accumulation of deletions and point mutations in mitochondrial genome in degenerative diseases." NY Acad.Sci.786. 102-111 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kovalenko SA.: "Accumulation of somatic nucleotide substitutions in mitochondrial tRNALeu (UUR) mutation encephalopathy and cardiomyopathy." Biochem.Biophys.Res.Commun.222. 201-207 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Tanno Y: "Quantitation of heteroplasmy of mitochondrial tRNA^<Leu>(UUR) gene using PCR-SSCP." Muscle Nerve. 18. 1390-1397 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yoneda M: "Oxygen stress induces an apoptotic cell death associated with fragmentation of mitochondrial genome." Biochem.Biophys.Res.Commun.209. 723-729 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yoneda M: "Detection and quantitation of point mutations in mtDNA by PCR" Methods Enzymol.264. 432-441 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Sawano T: "Mitochondrial DNA mutations associated with the 11778 mutation in Leber's disease." Biochem.Int.38. 693-700 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Tanaka M: "Accumulation of deletions and point mutations in mitochondrial genome in degenerative diseases." Ann.NY Acad.Sci.786. 102-111 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Kovalenko SA: "Accumulation of somatic nucleotide substitutions in mitochondrial tRNA^<Leu>(UUR) mutation encephalopathy and cardiomyopathy." Biochem.Biophys.Res.Commun.222. 201-207 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yoneda,M: "Oxygen stress induces an apoptotic cell death associated with fragmentation of mitochondrial genome." Biochem.Biophys.Res.Commun.209. 723-729 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Yoneda,M: "Detection and quantitation of point mutations in mtDNA by PCR." Methods Enzymol.264. 432-441 (1996)

    • Related Report
      1995 Annual Research Report
  • [Publications] Hayakawa,M: "Mitochondrial DNA minicircles,lacking replication origins,exist in the cardiac muscle of a young normal subject." Biochem.Biophys.Res.Commun.215. 952-960 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Sawano,T: "Mitochondrial DNA mutations associated with the 11778 mutation in Leber′s disease." Biochem.Int.(in press).

    • Related Report
      1995 Annual Research Report

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Published: 1995-04-01   Modified: 2016-04-21  

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