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Mitochondrial oxidants have been implicated in the cardiomyopathy in BTHS. Eleven mitochondrial sites produce superoxide\/hydrogen peroxide (H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub>) 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<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub> production from each site and the <jats:italic>ex\u00a0vivo<\/jats:italic> rate of superoxide\/H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub> production in the heart and skeletal muscle mitochondria of the tafazzin knockdown mice (tazkd) from 3 to 12\u00a0months of age. Despite reduced oxidative capacity, superoxide\/H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub> production was indistinguishable between tazkd mice and wild\u2010type littermates. These observations raise questions about the involvement of mitochondrial oxidants in BTHS pathology.<\/jats:p>","DOI":"10.1002\/1873-3468.13973","type":"journal-article","created":{"date-parts":[[2020,10,28]],"date-time":"2020-10-28T16:04:31Z","timestamp":1603901071000},"page":"415-432","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Cardiolipin deficiency in Barth syndrome is not associated with increased\u00a0superoxide\/H<sub>2<\/sub>O<sub>2<\/sub> production in heart and skeletal muscle mitochondria"],"prefix":"10.1002","volume":"595","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3044-9031","authenticated-orcid":false,"given":"Renata L. S.","family":"Goncalves","sequence":"first","affiliation":[{"name":"Sabri \u00dclker Center for Metabolic Research and Department of Molecular Metabolism Harvard T.H. 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