abstract |
Methods and compositions to deplete deleterious mitochondrial genomes (ΔmtDNAs), resulting in a compensatory increase in WT mtDNAs, by inhibition of LONP1, e.g., by inhibitory nucleic acids (including RNAi); inducing mutations that prevent the protease from bindng mtDNA; or administering an inhibitor, e.g., the clinically relevant compound CDDO-Me (Bardoxolone), all of which result in the preferential loss of ΔmtDNAs. |