MT-ATP6 and myelodysplastic syndrome: In the 1990s, there were reports of the association of renal manifestations in MDs with genetic mutations in TI (tRNAIle (AUU/C)), TL1 (tRNALeu (UUR)), TF (tRNAPhe (UUU/C)), TY (tRNATyr (UAU/C)), and TN (tRNAAsn (AAU/C)) in the mtDNA.12, 13, 14, 15, 16, 17 Since 2006, studies have reported that mitochondrial nephropathy is associated with nDNA mutations related to coenzyme Q10 synthesis.35, 36, 37, 38, 39 In addition, mtDNA mutations in MT-ATP6,40MT-CO1,41 and MT-ND5,42 which encode the subunits of complexes I, IV, and V, respectively, have recently been reported.