Presence of interaction but not complementation between human mtDNAs carrying different mutations within a tRNA(Leu(UUR)) gene

This study provided answers to fundamental questions on mammalian mitochondrial genetics: Could respiratory function in mitochondria be maintained by their exchange of genetic contents even when mutations were created within the same genes in different mitochondrial DNA (mtDNA) molecules? Using cell fusion techniques, we created a chance to coexist two types of respiration-deficient syn(-) mitochondria carrying different mtDNA mutations within the same tRNA(Leu(UUR)) gene obtained from patients with mitochondrial diseases. The results showed that two syn(-) mitochondria exchanged their genetic contents, but did not restore respiration defects, suggesting that mitochondrial interaction could not complement the mutations created within the same gene in different mtDNA molecules.