Sophia Hsin-Jung Li et al.
Nature protocols, 16(6), 2802-2825 (2021-05-07)
Several essential components of the electron transport chain, the major producer of ATP in mammalian cells, are encoded in the mitochondrial genome. These 13 proteins are translated within mitochondria by 'mitoribosomes'. Defective mitochondrial translation underlies multiple inborn errors of metabolism