Figure 1.

Animal models of mitochondrial disease created through genome editing techniques. There are a number of animal models of mitochondrial diseases caused by defects in nuclear and mtDNA. Thus, to study defects in nuclear-encoded mitochondrial proteins, knockout mice and pigs were created using CRISPR/Cas9. Despite many limitations in their application, models of invertebrate animals, such as the fruit fly, created using mitochondria-targeted restriction endonucleases, are quite common. Nevertheless, the most promising models of mitochondrial diseases at the moment are mice, rats and zebrafish, with mutations in mtDNA introduced by DdCBEs.