A monolysocardiolipin-cytochrome c peroxidase causes defects in Barth syndrome

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.