[Taurine for Mitochondrial Diseases].

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  • Author(s): Sunada Y;Sunada Y
  • Source:
    Brain and nerve = Shinkei kenkyu no shinpo [Brain Nerve] 2024 Oct; Vol. 76 (10), pp. 1127-1135.
  • Publication Type:
    English Abstract; Journal Article
  • Language:
    Japanese
  • Additional Information
    • Source:
      Publisher: igaku Shoin Country of Publication: Japan NLM ID: 101299709 Publication Model: Print Cited Medium: Print ISSN: 1881-6096 (Print) Linking ISSN: 18816096 NLM ISO Abbreviation: Brain Nerve Subsets: MEDLINE
    • Publication Information:
      Original Publication: Tōkyō : igaku Shoin
    • Subject Terms:
      Mitochondrial Diseases*/drug therapy ; Taurine*/administration & dosage; Animals ; Humans ; DNA, Mitochondrial/genetics ; MELAS Syndrome/drug therapy ; Mitochondria/drug effects ; Mitochondria/metabolism
    • Abstract:
      Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is characterized by a mitochondrial DNA mutation that leads to defective taurine modification of the leucine tRNA anticodon, with consequent impaired translation of the UUG codon. This defect reduces synthesis of respiratory chain complexes, which causes energy failure. Taurine supplementation improved mitochondrial function in MELAS model cells. A physician-initiated clinical trial reported that high-dose taurine supplementation therapy suppressed stroke-like episodes and improved taurine modification rates in leukocytes.
    • Accession Number:
      0 (DNA, Mitochondrial)
      1EQV5MLY3D (Taurine)
    • Publication Date:
      Date Created: 20241007 Date Completed: 20241007 Latest Revision: 20241028
    • Publication Date:
      20241028
    • Accession Number:
      10.11477/mf.1416202748
    • Accession Number:
      39370837