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A monolysocardiolipin–cytochrome c peroxidase causes defects in Barth syndrome

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We demonstrated increased phospholipid peroxidation due to the formation of monolysocardiolipin–cytochrome c complexes in tafazzin-deficient models of Barth syndrome. We found that a specific anti-peroxidase agent inhibited this complex and improved mitochondrial respiration. Thus, targeting the deleterious peroxidase activity offers a potential therapeutic approach to treat Barth syndrome.

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Fig. 1: Phospholipid oxidation and the effect of peroxidase inhibitors in BTHS models.

References

  1. Barth, P. G. et al. An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes. J. Neurol. Sci. 62, 327–355 (1983). This is the first report of the disorder now known as BTHS.

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This is a summary of: Kagan, V. E. et al. Anomalous peroxidase activity of cytochrome c is the primary pathogenic target in Barth syndrome. Nat. Metab. https://doi.org/10.1038/s42255-023-00926-4 (2023).

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A monolysocardiolipin–cytochrome c peroxidase causes defects in Barth syndrome. Nat Metab 5, 2043–2044 (2023). https://doi.org/10.1038/s42255-023-00925-5

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  • DOI: https://doi.org/10.1038/s42255-023-00925-5

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