| ATP | adenosine triphosphate |
| CI | complex I (NADH:ubiquinone oxidoreductase) |
| CII | complex II (succinate dehydrogenase) |
| CIII | complex III (cytochrome c oxidoreductase) |
| CIV | complex IV (cytochrome c oxidase) |
| CV | complex V (ATP synthase) |
| CPEO | chronic progressive external ophthalmoplegia |
| DddAtox | deaminase-like toxin |
| DdCBE | DddA-derived cytosine base editor |
| DNA | deoxyribonucleic acid |
| DSB | double-strand break |
| dsDNA | double-stranded DNA |
| ETC | electron transport chain |
| FusXTBE | FusX TALE Base Editor |
| IBM | inner boundary membrane |
| IMM | inner mitochondrial membrane |
| IMS | intermembrane space |
| iPSC | induced pluripotent stem cell |
| LHON | Leber Hereditary Optic Neuropathy |
| MD | mitochondrial disease |
| MELAS | mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes |
| MERRF | myoclonus epilepsy associated with ragged-red fibers |
| MIDD | maternally inherited diabetes and deafness |
| mito-CRISPR/Cas9 | mitochondria-targeted clustered regularly interspaced short palindromic repeat (CRISPR)-associated protein 9 |
| MitoRE | mitochondria-targeted restriction endonuclease |
| mitoTALEN | mitochondria-targeted transcription activator-like effector nuclease |
| mitoZFN | mitochondria-targeted zinc finger nuclease |
| mRNA | messenger RNA |
| mtDNA | mitochondrial DNA |
| MTS | mitochondrial targeting sequence |
| NARP | neuropathy, ataxia and pigmentary retinopathy |
| NES | nuclear export signal |
| OMM | outer mitochondrial membrane |
| OXPHOS | oxidative phosphorylation |
| PACE | phage-assisted continuous evolution |
| PANCE | phage-assisted non-continuous evolution |
| PBMC | peripheral blood mononuclear cell |
| RET | reverse electron transfer |
| RNA | ribonucleic acid |
| ROS | reactive oxygen species |
| rRNA | ribosomal RNA |
| TALE | transcription activator-like effector |
| TALED | TALE-linked deaminase |
| TCA | tricarboxylic acid |
| tRNA | transfer RNA |
| UGI | uracil glycosylase inhibitor |
| ΔΨm | mitochondrial transmembrane potential |