Rapamycin, the most studied mTOR inhibitor, is reported to be beneficial in models of mitochondrial dysfunction, including the Ndufs4 knockout (similar to Leigh syndrome), a model for mitochondrial myopathy (the Deletor mouse), a muscle-specific conditional knockout for the complex IV assembly factor Cox15, a mouse model with mtDNA depletion syndrome, complex I-deficient C. elegans gas-1 mutants, and cells exposed to mitochondrial inhibitors [45–50]. This evidence concerns the gene MTOR and Leigh syndrome.