Researchers at the University Medical Center Utrecht and collaborators report successful mitochondrial base editing to correct pathogenic mutations in mitochondrial DNA using a CRISPR/Cas-independent cytosine base editor known as DdCBE. Published in PLOS Biology, the study highlights effective gene editing in patient-derived cells, restoring mitochondrial function—a critical development for mitochondrial disease therapy. Concurrently, related work from the Netherlands details the application of mitochondrial base editors in liver cell organoids modeling mitochondrial dysfunction, a historically challenging area due to the mitochondrial membrane’s impermeability to traditional gene editing tools like CRISPR-Cas9.