Mitochondrial DNA (mtDNA) often exists in a state of heteroplasmy, in which mutant mtDNA
co-exists in cells with wild-type mtDNA. High frequencies of pathogenic mtDNA result in
maternally inherited diseases; maternally and somatically acquired mutations also
accumulate over time and contribute to diseases of ageing. Reducing heteroplasmy is
therefore a therapeutic goal and in vivo models in post-mitotic tissues are needed to
facilitate these studies. Here we describe a transgene-based model of a heteroplasmic …