Mutations in the NARS2 gene, downregulated in the FRDA cohort, are known to cause a spectrum of clinical phenotypes, including non-syndromic hearing loss, myopathy, and the mitochondrial energy deficiency disorders Leigh syndrome and Alpers syndrome (Mizuguchi et al., 2017; Simon et al., 2015; Sofou et al., 2015). The gene discussed is NARS2; the disease is Leigh syndrome.