Evolutionary diversification of mitochondrial proteomes: implications for human disease

By combining comparative genomics and computational identification of protein targeting, we predicted the size and composition of the mitochondrial proteome for ten species. Functional mitochondria could harbour from a few hundred to more than 3000 gene products, and protein relocation from and to mitochondria occurred during evolution. Although each genome studied contains lineage-specific mitochondrial proteins, conserved mitochondrial proteins also exist. Their functions mostly relate to transport and metabolism, and in humans their mutated states are frequently associated with disease.