Although quite different from the behavior of mammalian tissues, which have an extensive mitochondrial network, Saccharomyces cerevisiae lacking tafazzin function (Δtaz1 mutant) recapitulates many of the observed defects seen in cells from BTHS patients, including defective assembly of respiratory chain supercomplexes, decreased mitochondrial respiration, and increased ROS production [18,20,21,22]. This evidence concerns the gene TAFAZZIN and Barth syndrome.