Mitochondrial protein translation

Mitochondrial protein translation is the process by which mitochondria synthesize 13 essential proteins from their own DNA, all of which are core subunits of the oxidative phosphorylation system required for ATP production. This process employs specialized mitochondrial ribosomes, unique tRNAs, and dedicated translation factors. Mitochondrial translation is distinct from cytoplasmic translation, sharing more mechanistic similarities with bacterial systems. Dysregulation or genetic defects in this process lead to diverse and often severe diseases, including many childhood metabolic and neuromuscular disorders as well as adult-onset conditions.

Other names

Mitochondrial translationMitochondrial protein synthesis

Inhibition of mitochondrial translation via ribosomal binding (e.g., by some antibiotics); Modulation of mitochondrial ribosome assembly or function