Molecular mechanisms of DNA replication in human mitochondria
Cells contain thousands of mitochondria that are required to convert food into usable energy forms via the respiratory chain. Mitochondria have their own genomes (mtDNA) that in mammals encode 13 of the 90 respiratory chain proteins. All other mitochondrial proteins, including those necessary for mtDNA replication, are encoded in the nucleus. Mutations in the nuclear-encoded mtDNA maintenance proteins can cause neurodegeneration and muscle diseases. Mitochondrial dysfunction is also associated with common age-associated diseases (e.g. diabetes and Parkinson's). Our laboratory investigates the basic mechanisms of human mtDNA replication in health and disease. Much of the work involves in vitro biochemistry and reconstituted systems for mammalian mtDNA replication, using different types of artificial templates and recombinant proteins.