AAC2 gene, encoding the major isoform of the translocator [23], is considered the yeast ortholog of human ANT1. Due to the dispensability of respiratory metabolism and mtDNA, yeast is an excellent model organism to study mitochondrial functions and biogenesis [25,26] and is largely used for the validation of pathological mutations leading to mitochondrial diseases, including mutations in ANT1 [18,19,21,27,28]. This evidence concerns the gene SLC25A4 and inborn mitochondrial metabolism disorder.