DNA transcription and translation in mitochondria

Abstract Mitochondria have their own circular DNA that contains 37 genes, which are essential for the oxidative phosphorylation system. Cellular bioenergetics relies on oxidative phosphorylation functioning, and thus is highly dependent on an accurate transcription and expression of the mitochondrial DNA (mtDNA) genes. The mitochondrial protein synthesis is complex, and it involves several players with origin in both nuclear genome and mtDNA. Furthermore, the process is under a tight regulation with the underlying mechanism remaining to be fully characterized. This chapter provides a detailed overview of the mitochondrial synthesis protein, including molecular mechanisms for mitochondrial transcription and translation, including description of the main known posttranscriptional RNA modifications. Our focus is on humans. Knowledge in mitochondria synthesis protein emerges as an important topic for basic and clinical research areas.