Abstract
Human diseases caused by inherited mutations in the mitochondrial genome demonstrate the importance of maintaining mitochondrial DNA with high fidelity. Although the mitochondrial genome encodes few genes, relative to the nuclear genome, functional copies of these genes are essential to the viability of humans. Many repair proteins are shared between nuclei and mitochondria, but the mechanisms of their action and the regulation of their subcellular localization are not well understood. While some pathways of mitochondrial DNA repair have been studied for decades, a comprehensive picture of the suite of proteins that ensure maintenance of the mitochondrial genome is still developing. The results of recent studies suggest a robust and complex network of repair pathways that maintain the integrity of this organellar genome.
Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
