YbeY is required for ribosome small subunit assembly and tRNA processing in human mitochondria

Mitochondria contain their own translation apparatus which enables them to produce the polypeptides encoded in their genome. The mitochondrially-encoded RNA components of the mitochondrial ribosome require various post-transcriptional processing steps. Additional protein factors are required to facilitate the biogenesis of the functional mitoribosome. We have characterized a mitochondrially-localized protein, YbeY, which interacts with the assembling mitoribosome through the small subunit. Loss of YbeY leads to a severe reduction in mitochondrial translation and a loss of cell viability, associated with less accurate mitochondrial tRNA(Ser(AGY)) processing from the primary transcript and a defect in the maturation of the mitoribosomal small subunit. Our results suggest that YbeY performs a dual, likely independent, function in mitochondria being involved in precursor RNA processing and mitoribosome biogenesis. Issue Section: Nucleic Acid Enzymes. [GRAPHICS] .

Automated summary

Mitochondria have their own translation apparatus for producing polypeptides, with the protein YbeY playing a crucial role in mitoribosome biogenesis. Loss of YbeY leads to reduced mitochondrial translation and loss of cell viability, indicating its important functions in RNA processing and mitoribosome maturation.