Researchers from University Medical Center Utrecht and collaborators have successfully applied mitochondrial base editing to correct pathogenic mitochondrial DNA mutations in patient-derived human cells. Using a double-stranded DNA cytosine base editor (DdCBE), they precisely converted specific cytosines to thymine without DNA breaks, restoring mitochondrial function in cell and organoid disease models. This pioneering technique overcomes prior barriers in mitochondrial genome manipulation and holds promise for treating inherited mitochondrial disorders and related conditions.