The structure of the human mitochondrial ribosome

Abstract

The whole mitoribosome at high resolution Mitochondria are thought to be the descendents of a prokaryotic cell that took up residence in a protoeukaryotic cell. Mitochondria retain a few genes involved in oxidative phosphorylation. To translate these genes, mitochondria contain highly divergent mitochondrial ribosomes, or mitoribosomes. Amunts et al. determined the high-resolution structures of complete mammalian mitoribosomes using cryoelectron microscopy. Mitoribosomes include an unusual mRNA binding channel. The findings elucidate how aminoglycoside antibiotics can inadvertently inhibit mitoribosomes and how mutations in mitoribosomes can lead to disease. Science , this issue p. 95