Engineered zinc finger proteins for manipulation of the human mitochondrial genome

Currently there are no effective methods to manipulate or modify particular sequences in mammalian mitochondrial DNA (mtDNA) within cells. The availability of such methods would be of great value for basic mitochondrial research and would aid in development of therapies for diseases linked with mutations in mtDNA. Engineered zinc finger proteins have been used as powerful tools for intervening in nuclear gene expression and modifying nuclear DNA in a sequence-specific manner. Here, basic methods that are helpful in adapting the engineered zinc finger technology for targeting the DNA in mitochondria are presented with the main emphasis on mitochondrial import of zinc finger proteins.