Products of Mitochondrial Genes Are Not Exported

As far as is known, all RNA transcripts of mtDNA and their translation products remain in the mitochondrion in which they are produced, and all mtDNA-encoded proteins are synthesized on mitochondrial ribosomes. Mitochondrial DNA encodes the rRNAs that form mitochondrial ribosomes, although most of the ribosomal proteins are imported from the cytosol. In animals and fungi, all the tRNAs used for protein synthesis in mitochondria are also encoded by mtDNAs. However, in plants and many protozoans, most mitochondrial tRNAs are encoded by the nuclear DNA and imported into the mitochondrion.

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Reflecting the bacterial ancestry of mitochondria, mitochondrial ribosomes resemble bacterial ribosomes and differ from eukaryotic cytosolic ribosomes in their RNA and protein compositions, their size, and their sensitivity to certain antibiotics (see Table 5-3). For instance, chloramphenicol blocks protein synthesis by bacterial and mitochondrial ribosomes from most organisms, but cycloheximide, which inhibits protein synthesis on eukaryotic cytosolic ribosomes, does not affect mitochondrial ribosomes. This sensitivity of mitochondrial ribosomes to the important aminoglycoside class of antibiotics, which includes chloramphenicol, is the main cause of the toxicity in patients that these antibiotics can cause.