Cardiolipin deficiency in Barth syndrome is not associated with increased superoxide/H 2 O 2 production in heart and skeletal muscle mitochondria.

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  • Additional Information
    • Source:
      Publisher: John Wiley & Sons Ltd Country of Publication: England NLM ID: 0155157 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-3468 (Electronic) Linking ISSN: 00145793 NLM ISO Abbreviation: FEBS Lett Subsets: MEDLINE
    • Publication Information:
      Publication: Jan. 2016- : West Sussex : John Wiley & Sons Ltd.
      Original Publication: Amsterdam, North-Holland on behalf of the Federation of European Biochemical Societies.
    • Subject Terms:
      Acyltransferases/*genetics ; Barth Syndrome/*genetics ; Mitochondria, Heart/*enzymology ; Mitochondria, Muscle/*enzymology ; Muscle, Skeletal/*enzymology ; Myocardium/*enzymology; Acyltransferases/deficiency ; Animals ; Barth Syndrome/enzymology ; Barth Syndrome/pathology ; Cardiolipins/metabolism ; Disease Models, Animal ; Electron Transport Chain Complex Proteins ; Gene Expression ; Humans ; Hydrogen Peroxide/metabolism ; Mice ; Mice, Knockout ; Mitochondria, Heart/pathology ; Mitochondria, Muscle/pathology ; Muscle, Skeletal/pathology ; Myocardium/pathology ; NAD/metabolism ; Oxygen Consumption/genetics ; Superoxides/metabolism
    • Abstract:
      Barth syndrome (BTHS) is a rare X-linked genetic disorder caused by mutations in the gene encoding the transacylase tafazzin and characterized by loss of cardiolipin and severe cardiomyopathy. Mitochondrial oxidants have been implicated in the cardiomyopathy in BTHS. Eleven mitochondrial sites produce superoxide/hydrogen peroxide (H 2 O 2 ) at significant rates. Which of these sites generate oxidants at excessive rates in BTHS is unknown. Here, we measured the maximum capacity of superoxide/H 2 O 2 production from each site and the ex vivo rate of superoxide/H 2 O 2 production in the heart and skeletal muscle mitochondria of the tafazzin knockdown mice (tazkd) from 3 to 12 months of age. Despite reduced oxidative capacity, superoxide/H 2 O 2 production was indistinguishable between tazkd mice and wild-type littermates. These observations raise questions about the involvement of mitochondrial oxidants in BTHS pathology.
      (© 2020 The Authors. FEBS Letters published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies.)
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    • Grant Information:
      R01 GM115593 United States GM NIGMS NIH HHS; R01 GM115593 United States NH NIH HHS; R01 DK052539 United States NH NIH HHS
    • Contributed Indexing:
      Keywords: Barth syndrome; cardiomyopathy; mitochondria; mitochondrial reactive oxygen species; superoxide/H2O2; tafazzin; tazkd mice; ‘ex vivo’ rate of superoxide/H2O2 production
    • Accession Number:
      0 (Cardiolipins)
      0 (Electron Transport Chain Complex Proteins)
      0U46U6E8UK (NAD)
      11062-77-4 (Superoxides)
      BBX060AN9V (Hydrogen Peroxide)
      EC 2.3.- (Acyltransferases)
      EC 2.3.- (tafazzin protein, mouse)
    • Publication Date:
      Date Created: 20201028 Date Completed: 20210727 Latest Revision: 20210817
    • Publication Date:
      20231215
    • Accession Number:
      PMC7894513
    • Accession Number:
      10.1002/1873-3468.13973
    • Accession Number:
      33112430