A team led by the University Medical Center Utrecht successfully applied mitochondrial base editing (mtBE) using a DddA-derived cytosine base editor (DdCBE) to precisely correct deleterious mutations in mitochondrial DNA within human patient-derived cellular models. This CRISPR-independent approach overcomes previous limitations in mitochondrial genome editing, restoring mitochondrial function in vitro and opening new therapeutic avenues for mitochondrial disorders. The findings, published in PLOS Biology, represent a significant step toward clinical gene therapies targeting maternally inherited mitochondrial diseases and related conditions.