Abstract

The mammalian mitochondrial translation machinery is responsible for the synthesis of 13 mitochondrially encoded proteins that are essential for energy production. These proteins are subunits of the oxidative phosphorylation complexes embedded in the inner membrane of mitochondria. Mitochondrial protein synthesis is highly similar to that of bacterial systems; however, there are subtle differences between these systems in terms of their mechanisms and components. In this review, we will discuss the elements of mitochondrial translation, including the stages of protein synthesis and the factors involved in these processes. Although much still waits to be learned about the regulation of this system, a summary of what is currently known about the regulation of its protein components by post-translational modifications, specifically concerning energy metabolism, will also be included in this chapter.

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