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The human mitochondrial ribosome, or mitoribosome, is a ribonucleoprotein complex located within mitochondria. It synthesizes proteins encoded by mtDNA, which are essential for oxidative phosphorylation and ATP production. It is composed of a 28S small subunit and a 39S large subunit, forming a 55S complex. The mitoribosome contains three rRNA molecules and approximately 80 proteins, with about 36 being mitochondria-specific. Mutations affecting mitoribosomal RNA genes or nuclear genes encoding its protein components can lead to severe mitochondrial diseases.
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